PEDIA

PEDIA
PLATI
F
NUM
PLATINUM
IR S T E
FIRST D IT IO N
EDITION
2020
 PEDIA
PLATINUM
FIRST EDITION

2 0 2 0

RUBY ANN L. PUNONGBAYAN, MD

ANA MARIER. MORELOS, MD
CAROL STEPHANIE C. TAN-LIM, MD
ADRIAN SALVADOR M. DE VERA, MD
JAIME ALFONSO M. AH ERRERA, MD
MARC DENVER A. TIONGSON, MD
ENRICO PAOLO C. BANZUELA, MD

EDITOR
VICTORS. DOCTOR, MD
 The printing of Pedia Platinum is financed by Top Practice Medical Publishing
Corporation, Manila, Philippines. Proceeds from the purchase of this book will
fund the development and improvement of future editions of this book.

Please visit our official page, https://www.facebook.com/pediaplatinum,

for information on how to procure your Pedia Platinum book at the lowest
possible price. Copies can be delivered to your doorstep anywhere in the
Philippines within 1-7 days after bank payment. Unauthorized reproduction
of this book is illegal. Beware of counterfeits; an original Pedia Platinum copy
contains glossy pages, not matte or plain paper. You deserve to get the original.

First Edition, 2020

Philippine Copyright 2020 by Top Practice Medical Publishing Corporation

Book design and layout by Manuel S. Vidal, Jr. All rights reserved. No part of this publication may
be reproduced, stored in a retrieval system, or transmitted, in any form by any means without
prior permission from Top Practice Medical Publishing Corporation.

Published by:
Top Practice Medical Publishing Corporation
No. 27 Mercury Street, Fairview, Quezon City

Contact no: +63918-807-6700

ISBN 978-621-95388-4-8

ii
CONTRIBUTING
AUTHORS
Justine Iris C. Yap, MD, FPPS, FPCC, FEACVI
Jerry V. Pua, RMT,MD, DPPS
Romana Marie M. Vila, MD, FPCR, FCTMRISP
Diosemyl L. Leyson-Guzman, MD, DPPS, DPSN,DPNSP

ILLUSTRATOR
Joerelle V. Mojica, MD, FPCP, FPCC

LAYOUT
EDITOR
Manuel S. Vidal, Jr., RCh

V
 NOTICE
The information in this book has been reviewed and verified with
reliable sources, and the approaches to management have been utilized
in clinical practice. Howevet~ medicine is an ever-changing science. New
research, changes in guidelines, and human error occul'. The authors,
editor, and publisher are not responsible for errors or omissions or
for any untoward outcomes from application of data in this book. The
authors, edito1; publishe1; and other parties who have been involved in
the preparation of this book make no warranty, expressed or implied,
with respect to the completeness, accuracy, or being up-to-date of all
the information contained in this publication. The authors, edito1; and
publisher encourage the readers to confirm the information herein
with other sources, and to exercise critical thinking based on the clinical
presentation of the patient in making decisions for management.

vi
PREFACE
As a practicing pediatrician dealing with emergencies for the past 15 years, I find it highly
gratifying, and of utmost importance that I am able to share my clinical experiences with the
medical students and residents. Through those years as a teacher in Pediatrics, an inner desire
to provide the medical students a compendium for recapitulating what is necessary in this
subject stirred in me. This dream slowly took form as I became part of the faculty of Topnotch
Medical Board Prep since its inception. Topnotch Medical Board Prep has definitely gained a
renowned and prestigious reputation in the field and has taken essential steps in aiding the
medical students to realize their dream of becoming licensed physicians. The success of the first
two books, IM Platinum and Surgery Platinum with the publication of Medical Jurisprudence,
were testimonies of its quest for providing high quality review books.

There has been a dearth of review books in Pediatrics in the home front hence, we envisioned
to provide one for you. It is heartwarming to receive initial feedback that pediatric residents
in training as well as our other colleagues in the medical field who have been practicing for
some time have also expressed their anticipation of this book. At this digital era, one cannot
deny the instantaneous and expeditious access of a learner to journals, latest research, and
medical textbooks. But many of you may agree that all of this information can be overwhelming
as well. Thus, our team spearheaded by Dr. Enrico Paolo Banzuela sat down exactly two years
ago (January 2018) and discussed the birth of Pedia Platinum.

Our two-year trek has been spiced with trials both on a personal and professional level. The lulls in our
momentum have been due to conflicts in schedule, falling ill, fulfilling graduate school requirements,
welcoming babies in the family, dealing with loss of a loved one, and other professional commitments.
But we still forged to move forward as we were all fueled to come up with our dream review book

We came up with a total of21 chapters mirroring the subspecialties in Pediatrics, each carefully
scrutinized and dissected as if we were the ones who were going to read it. Will this topic
be useful for the board exam and for the usual rounds? Is this topic a "must know" category
versus something that is least likely to be discussed? All of these questions were disputed and
settled with our sense of humor intact. I believe my colleagues would agree with me that the
most challenging chapter was that of fluids and electrolytes as we argued on which guidelines
to put in the book. It is not a hidden truth that most of what we learn in medical school can be
challenged and unlearned during internship and residency training because of "hand me down"
information and local practices. To resolve this, we did an extensive research particularly on
the computation of fluids and electrolytes and cited references accordingly. You will also note
that after each presentation of a certain guideline, we included a statement explaining that such
guidelines may vary from one local institution to another.

In the end, we were highly satisfied that the product of our hard work gave rise to a very practical,
useful, and comprehensive review book in Pediatrics. I would like to give my eternal gratitude
to Dr. Enrico Paolo Banzuela, a visionary himself, who has painstakingly led us in accomplishing
this book; to my colleagues in Pediatrics Dr. Ana Marie Morelos, Dr. Adrian De Vera, and Dr. Carol
Tan-Lim whose dedication and tenacity are worth emulating; to Dr. Marc Denver Tiongson (for
his valuable contribution and leadership) and Dr. Jaime Aherrera (with his uncanny skill in
transforming texts into tables as well as his leadership) who have been highly instrumental in
the completion of this book; Manuel Vidal, an upcoming bright physician with his skills in laying
out the book; Dr. Mark Louis Mann for taking extra special care of handling the promotion of
the book; and to all the contributing authors. Special thanks to our beloved Dr. Victor S. Doctor,
past president the Philippine Pediatric Society and a pillar in pediatric nephrology, for being
our editor. Thank you to our Almighty God for His guidance and unending grace. This is a dream
team indeed!

This is our passion ...this is our legacy ...and we are sharing this with pride and joy to all of you ...

Ruby L. Punongbayan, MD, FPPS, MA

vii
FOREWORD
This book, PEDIA PLATINUM, symbolizes knowledge that does not rust, a source of light when
memory dims, sometimes, and a reminder that knowledge is indeed power. It is one of your
resources to strengthen your knowledge, skills, and confidence as a medical practitioner. It
is a handy reference to spark back your memory of Pediatrics.

The authors take pride in the production of a compact medical literature of the pediatric
specialties that should consolidate to a strong foundation. The men and women behind:

Ruby Ann L. Punongbayan, MD, FPPS, MA

Ana Marie R. Morelos, MD, DPPS
Carol Stephanie C. Tan-Lim, MD, DPPS
Adrian Salvador M. De Vera, MD, DPPS
Jaime Alfonso M. Aherrera, MD, FAMP, FPCP, FPCC
Marc Denver A. Tiongson, MD, FPCP, DPCC
Enrico Paolo C. Banzuela, MD, DHPEd, MSEd, FPSP

They put up their best efforts and interests to synthesize local and international literature
with proper acknowledgements. We dedicate this publication to all medical practitioners,
especially to our younger colleagues.

With the FIRST EDITION of PEDIA PLATINUM, we hope to accommodate the rush of
developments that impact pediatric practice. Ultimately, all of these will translate to
comprehensive patient care.

Find time to internalize what this book can offer for your professional growth. Love your
profession!

Victor S. Doctor, MD, FPPS, FPNSP, FPSN

viii
ACKNOWLEDGEMENT
We would like to offer our utmost gratitude:

To the Lord Almighty, who makes all things possible;

To our families, for their unfailing love, support and inspiration: The Punongbayan Family
especially to Miss Amelita Punongbayan; Mrs. Eladia Bautista Ramirez, Mr. Juan Paolo, Ms.
Maria Angelica Beatriz & Mr. Edilberto Lorenzo Morelos; Mr. Archie & Mstr. Chase Stephen
Lim; Mr. Manuel, Dr. Bonaleth & Atty. Jo Franz de Vera, & Mrs. Diana, Ms. Adriana & Mstr.
Nicolo de Vera; Capt. Jaime Julian & Mrs. Ma. Rosario Aherrera; Mrs. Maria Cristina Tiongson,
Dr. Lalaine & Mstr. Matthew Damian Tiongson, Atty. Virgilio, Mrs. Marilyn, Mr. Patrick Jeanne
& Ms. Patricia Meryl Tiongson, & Mr. Dennis, Mrs. Maita, Ms. Maiden Kristine & Mr. Andrei
Nikolai Tiongson; and Mrs. Arabella Chiong Banzuela, Dr. Nifia Banzuela-Cruz, Dr. Rocky Lim
& Ms. Arabella Aurora Lim Banzuela;

To all our mentors, Dr. Ramon L. Arcadia, Dr. Carmencita Padilla, Dr. Lulu Bravo, Dr. Salvacion
Gatchalian, Dr. Juliet Sia-Aguilar, Dr. Lorna Abad, Dr. Bernadette Madrid, Dr. Marysia Recto,
Dr. Mary Anne Castor, Dr. Agnes Mejia, Dr. Rody Sy, Dr. John Afionuevo, Dr. Madeleine Sosa,
Dr. Sandra Litao, Dr. Maria Concellene Laforteza, Dr. Jose Fernando Fontanilla, Dr. Maria
Luz Querubin, and Dr. Erlyn Sana, Dr. Rose Anna Banal, Dr. Robert Arias, Dr. Pacifico Eric
Calderon, Dr. Eric Calderon Jr., & Dr. Mark Louie Mann; Dr. Julius Lecciones, Dr. Michael
Resurreccion, Dr. Sonia Gonzales, Dr. Florentina Uy-Ty, Dr. Victoria Ribaya, Dr. Jonathan Cu,
Dr. Cristan Cabanilla, Dr. Amel Jiao, Dr. Maria Beatrice Gepte, Dr. Reynaldo de Castro Jr, Dr.
Sheila Ann Masangkay, and Dr. Cecile Gan for their invaluable guidance and wisdom;

To all our colleagues and friends from the Chinese General Hospital and Medical Center,
Pamantasan ng Lungsod ng Maynila College of Medicine, Ospital ng Maynila, Philippine
Children's Medical Center, St. Luke's Medical Center Quezon City, UP College of Medicine, UP-
Philippine General Hospital Department of Pediatrics, for helping us bring the best in us and
help us become the doctors we are today;

To our fellow teachers and bosses from San Beda University College of Medicine, Ateneo
School of Medicine and Public Health, De La Salle Medical and Health Science Institute, Bica!
University College of Medicine, New Era University College of Medicine, for enabling us to
undertake this endeavor; to our students, for inspiring us to be better educators every day;

To all main authors, contributing writers of the IM Platinum, Surgery Platinum and Philippine
Medical Jurisprudence, thank you very much for all your help in making the Platinum Series
Dream come true;

To PCMC lBandStrong and PGH Wakipedia;

To the Phi Kappa Mu Fraternity;

To the Mu Sigma Phi Sorority;

To Cohort 5 of the University of Pennsylvania Masters in Educational Entrepreneurship.

Classmates from Asian Institute of Management MDP Batch 97 and Maastricht University
MHPE;

To our patients especially the Filipino Children, our greatest teachers, to whom the essence
and soul of this book is rooted on. This book is for you first and foremost.

This book is lovingly dedicated to Mr. Eduardo Punongbayant, Mrs. Clarita Punongbayant,
Dr. Miguel L Noche, Jr.t, Justice Pedro Alaras Ramirezt, Dr. Edilberto Torres Morelost,
Atty. Virgilio Tiongsont, and Mrs. Paula Chuat.

Ruby, Menette, Carol, Ian, Jaime, Denver, and Broli

ix
DEDICATION
To my parents ...

Eduardo (tl 985)

and
Clarita (t2019)

....for instilling in me the importance of education ...

To all the teachers in my family who inspired me to become one ...

To the medical students for making me embrace the love for teaching ...

Ruby

X
THE
AUTHORS
Ruby L. Punongbayan, MD, FPPS, MA
Dr. Punongbayan graduated from Pamantasan ng Lungsod ng Maynila College of Medicine with full academic
scholarship. She took her fellowship in Ambulatory Pediatrics in the University of the Philippines ·Philippine
General Hospital and then proceeded to take Master of Arts in Clinical Psychology in Pamantasan ng Lungsod
ng Maynila Graduate School of Medicine. Dr Punongbayan also took the Basic Course in Health Professions
Education in the University of the Philippines •NTTC. To further help patients, she took Cognitive Behavioral
Therapy at Beck Institute, Pennsylvania, USA. lnspite being the Section Head of Pediatric Emergency
Medicine of St. Luke's Medical Center in Quezon City and Program Head of the Department of Pediatrics
of San Beda University College of Medicine, she is still an Active Consultant of the Institute of Pediatrics
and Child Health in St. Luke's Medical Center Quezon City, an Associate Professor in Pediatrics and a part
time clinical professor in the Department of Pediatrics of Pamantasan ng Lungsod ng Maynila and St. Luke's
College of Medicine. She also contributes her time to teach underboard physicians who will be taking the
physician licensure exam by reviewing them in Pediatrics at Topnotch Medical Board Prep. She is also an
Ambulatory Pediatrics consultant in Centre Medicale Internationale in Bonifacio Global City, Taguig City, a
Fellow of Philippine Pediatric Society and a member of the Philippine Ambulatory Pediatric Association. Dr
Ruby likes playing the piano, into abstract painting with acrylic as medium, singing, and is skilled at American
sign language. She also loves traveling, be it solo or by group, and explores the off-the-beaten paths.

Ana Marie R. Morelos, MD, DPPS

Dr Morelos is a BS Zoology graduate from the in University of the Philippines -Diliman, Quezon, City. She
then pursued to obtain her Medical degree at the University of the Philippines -Manila and her Residency in
Pediatrics in the same institution. Dr Morelos finished her Fellowship in Pediatric Infectious Diseases at the
University of the Philippines Philippine General Hospital and Research Institute for Tropical Medicine. After
fellowship training she practiced in Cagayan de Oro for 7 years as a consultant at the Northern Mindanao
Medical Cente1; Madonna and Child Hospital, and Maria Reyna Hospital. She was also a Faculty member
of the Departments of Pediatrics, Xavier University, College of Medicine. She then returned to Manila and
joined the Medical staff of the Chinese General Hospital and Medical Center. Since 2002 to present she is
an active consultant in the Department of Pediatrics of the said institution and is also a member of the
Hospital Infection Control Committee and Antimicrobial Stewardship Committee. Pursuing her passion for
teaching she has been a faculty of San Beda University College of Medicine from 2002 to present. In 2009,
was appointed Prefect of Student Affairs at San Beda University College of Medicine and at present continues
to hold this administrative position As a member of the San Beda Red Lions Coaching Staff for the past 10
seasons, she is 24/7 on call as Team Physician, nutritionist/food & beverage manager, tuto1: psychologist,
shoulder to cry on, cheerleader and is a very proud surrogate mom to this champion team. Dr. Moreles is also
affiliated with the Philippine Pediatric Society, Manila Medical Society, and Medical Staff Association CGHMC.
Three decades after graduation from medical school Dr Morelos feels like she is still a work in progress, but
it does not feel like work at all! She derives great pleasure and fulfillment in whatever she does may it be
reading a good book, cooking, sharing a meal, enjoying a cup of coffee, or watching a great movie (She is a
big Starwars fan]. She also spends time cheering for her favorite basketball team, shopping as a form of retail
therapy, and just connecting with people because everyone is family.

Carol Stephanie C. Tan-Lim, MD, DPPS

Dr. Tan-Lim obtained her B.S. Basic Medical Sciences degree (INTARMED) from UP Manila - College of
Medicine in 2009 and her medical degree from the same institution in 2012. She graduated magna cum
laude, class valedictorian, and was awarded the Faculty Gold Medal, Most Outstanding Medical Graduate, and
Gregorio T. Alvior Jr Award for Academic Excellence. She was also the Most Outstanding Intern in Pediatrics
and Internal Medicine, and Outstanding Intern in Surgery, Obstetrics and Gynecology, and Family and
Community Medicine. She ranked first in the Physician Licensure Examination in August 2012. She completed
her residency training and Chief Residency in Pediatrics at the Philippine General Hospital. She received the
Perla D. Santos-Ocampo Award for Most Outstanding Pediatric Resident and a National Publication Award.
She then obtained her Master of Science Degree in Clinical Epidemiology from UP Manila - College of Medicine.
She served as a faculty member at the Department of Pharmacology and Department of Research, Evidence-
Based Medicine and Medical Informatics at the Emilio Aguinaldo College of Medicine, and research instructor
at the Manila Doctors Hospital. She worked as a medical writer for the MIMS Docto1: and as a technical writer
for various projects of the Research Institute for Tropical Medicine, Infectious Diseases Prevention and
Control Division of the Department of Health, Child Protection Network Foundation, Philippine Society for
Microbiology and Infectious Diseases, and the Asia Pacific Center for Evidence-Based Healthcare Inc. She
has published in local and international scientific journals, such as the World Allergy Organization Journal.
She is currently serving as Chief Fellow of the Division of Allergy and Immunology at the Philippine General
Hospital. She is happiest when she is spending lazy days at home with her family.

xi
Adrian Salvador M. de Vera, MD, DPPS
Dr. De Vera graduated with a B.S. Biology degree from the Ateneo de Manila University in 2007. He then
attained his medical degree from the UP College of Medicine in 2012. He finished his Pediatrics Residency
program at the Philippine Children's Medical Center in 2016. At that same year, he was awarded the Dr. Joel
Elises Award for Excellence in Pediatrics in 2016, the highest award given to residents by the said institution.
His research on the use of peripheral veins to extract blood for monitoring dengue patients won several
awards locally and internationally. Today, he is a practicing pediatrician at De Vera Medical Center Inc. At the
same institution, he leads by serving as a member of the Board of Directors and as Training Officer of the
Nursing Professional Advancement and Development Program. His main advocacies include breastfeeding
and vaccination. He is also a professor at Topnotch Medical Board Prep since 2012, professional newborn
photographer at Nouveau Nee Photography, car racing and table tennis enthusiast. More than all these, he is
a loving husband to Diana Kibanoff-De Vera and father to Adriana and Nicolo.

Jaime Alfonso M. Aherrera, MD, FAMP, FPCP, FPCC

Dr. Aherrera graduated with a B.S. Human Biology degree from De La Salle University-Manila in 2004 and
obtained his medical degree from the De La Salle University-Health Sciences Institute in 2008, garnering
awards including the Dean's Special Award for Academic Performance and Special Citation for Academic
Excellence. He was also a board topnotcher during the Philippine Physician Licensure Examinations in August
2009. He completed his residency in Internal Medicine at the Philippine General Hospital in 2012 and was
one of the five national finalists for the Philippine College of Physicians Exemplar in Residency Training in
the same year. He completed his Cardiology and lnterventional Cardiology fellowship also at the Philippine
General Hospital where he was Chief Fellow and recipient of both the Dr. Ramon Abarquez Most Outstanding
Fellow Award and the Dr. Clemente Gatmaitan Most Outstanding Fellow in Research Award. At a young age,
he has already completed an impressive portfolio of researches that have been presented in numerous local
and international conferences, including three editions of IM Platinum, which was awarded as one of the
Outstanding Books of 2016 by the National Academy of Science and Technology (NAST). He was also winner
of the prestigious Dr. Francisco Tangco Young Investigator's Award from the Philippine Heart Association
for two consecutive years (2014 and 2015), recipient of the Most Outstanding Fellow in Cardiology from
the Philippine Heart Association in 2016, and topnotcher of the Cardiology Diplomate Examination that
same year. He is currently completing his Master of Science degree in Clinical Epidemiology at the UP
Manila - College of Medicine. He is a Fellow of both the Philippine College of Physicians and the Philippine
College of Cardiology, a member of the Philippine Society of Cardiac Catheterization and Interventions,
and is presently practicing as an lnterventional Cardiologist at the Asian Hospital and Medical Center, UP-
Philippine General Hospital, Manila Doctors Hospital, and De La Salle University Medical Center. Despite these
sterling achievements, Jaime remains grounded, down to earth, and a jolly friend to his peers and colleagues.
IG: @jaime_aherrera

Marc Denver A. Tiongson, MD, FPCP, DPCC

Dr. Tiongson graduated with a Bachelor in Science in Biology, major in Cell and Molecular Biology, cum laude,
from the University of the Philippines-Los Banos in 2006. He pursued on to obtain his medical degree from
the UP College of Medicine in 2011 and was awarded outstanding intern in several clinical departments.
Upon passing the medical board exams, he became a faculty member of the San Beda College of Medicine
and Topnotch Medical Board Prep teaching Physiology, Biochemistry, Anatomy, Pharmacology and Pathology.
Shortly after, he trained and completed his residency in Internal Medicine at the Philippine General Hospital
in 2015. In 2019 he finished his Cardiology Fellowship at the same hospital where he served as both the
Chief Fellow for the Division of Cardiovascular Medicine and Overall Chief Fellow for the Department. His
interest in research garnered him awards in local and international conferences. On top of this, he was able
to publish his works in highly regarded scientific journals. He likewise co-authored IM Platinum and Surgery
Platinum where the former was recognized as one of the Most Outstanding Books by the National Academy of
Science and Technology in 2016. Because of his extraordinary achievements he was awarded the Dr. Ramon
F. Abarquez Most Outstanding Fellow, Dr. Celemente Gatmaitan Most Outstanding Fellow in Research, and
Philippine Heart Association Most Outstanding Training Fellow in Cardiology for 2019. He is a Fellow of the
Philippine College of Physicians and Diplomate of the Philippine College of Cardiology. Presently, he is doing
his fellowship in lnterventional Cardiology at the Philippine General Hospital. He also holds the position as
the current national representative of the Philippines to the Heart Failure Association of the European Society
of Cardiology - Heart Failure Specialists of Tomorrow. Moreover, Denver remains to be a well-rounded guy
and a proud family man who values time with his family especially his wife, Lalaine and son, Matthew; and
friends. His interests span video games, watching movies and binging on Netflix.

xii
Enrico Paolo C. Banzuela, MD, DHPEd, MSEd, FPSP
Dr. Banzuela graduated with a B.S. Basic Medical Sciences degree (INTARMED) from UP Manila in 2002. He
obtained his medical degree from the UP College of Medicine in 2005. He finished his Masters in Educational
Entrepreneurship (MSEd) at the University of Pennsylvania in 2019. He obtained his Diploma in Health
Professions Education from the UP National Teacher Training Center for the Health Professions in 2018. He
also a graduate of the Management Development Program of the Asian Institute of Management in 2018.
He is currently taking a Masters in Health Professions Education at the University of Maastricht in the
Netherlands. At the same time he is also taking up a Postgraduate Certificate in Teaching Evidence-Based
Health Care at the University of Oxford in the United Kingdom. Upon passing the medical board exams in
2005, he served as a volunteer physician for the 2005 Manila Southeast Asian Games. He also worked as a
University Researcher for the UP National Institutes of Health under the Phil Health Research Study Group for
3 years. He co-authored a book entitled "Survival Guide for Doctors [and Non-Doctors Too) with Dr. Willie
Ong and Dr. Liza Ong. He is also a co-author of IM Platinum, Surgery Platinum and now Pedia Platinum.
Dr. Banzuela is the Chair of the Section of Medical Physiology (Associate Professor 11) at the San Beda
University College of Medicine and has been a faculty member since 2005. he has also served as lecturer for
Biochemistry, Neurology, Family and Community Medicine at the same institution. He is a guest lecturer for
Biochemistry at the Ateneo School of Medicine and Public Health. He currently serves as Vice-President of the
Philippine Society of Physiologists. He is the President of Topnotch Medical Board Prep where he also teaches
Physiology. Dr. Banzuela has always been involved with preparing doctors for the medical board exams since
2005, having written guides, given orientation talks, created school-specific board exam programs and
lectured subjects at various hospitals and institutions. He enjoys fishing, driving, playing basketball, listening
to audiophile setups, film photography, and going on food trips with his family.

THE
EDITOR
Victor S. Doctor, MD, FPPS, FPNSP, FPSN
Dr. Doctor as he is currently called by his colleagues pursued his medical career in the University of the East
-Ramon Magsaysay Medical Center, Inc. and his Residency in Pediatrics in the same institution. He went to
Georgetown University, Washington DC to pursue his subspecialty in Pediatric Nephrology. He is a fellow of
the following institutions: Philippine Pediatric Society, Inc. (PPS), Philippine Society of Nephrology (PSN),
and the Pediatric Nephrology Society of the Philippines (PNSP). He also has a passion for teaching and was
a professor at UERMMMCI (Retired November 2012), Professor Ill Pamantasan ng Lungsod ng Maynila
(Retired November 2012), Professorial Lecturer Ill Pamantasan ng Lungsod ng Maynila (December 1, 2012-
Present) Professorial Lecturer UERMMMCI. He also serves as a Nephrology Consultant at UERMMMCI -
Department of Pediatric, Cardinal Santos Medical Center and Amang Rodriguez Medical Center, Marikina. He
served as President in the prestigious Philippine Pediatric Society, Inc., Pediatric Nephrology Society of the
Philippines and Philippine Society of Nephrology [PSN) 2016-2017. In his spare time, he enjoys singing and
playing the guitar.

xiii
CONTRIBUTING
AUTHORS
Justine Iris C. Yap, MD, FPPS, FPCC, FEACVI
Dr. Yap earned her medical degree in 2005 and has completed her post graduate internship in 2006 in the University
of the Philippines• Philippine General Hospital. After passing the physician's licensure examination, she then pursued
her residency and fellowship training in General Pediatrics and Pediatric Cardiology in the University of the Philippines
- Philippine General Hospital, which commenced in 2010 and 2014 respectively. Majorly inspired by her professors
and mentors in the department, and with her enthusiasm to lead the trainees for more learning opportunities and
broaden the network amongst inter- national colleagues, she pioneered the international clinical attachment of pediatric
cardiology trainees as part of the curriculum in the Division of Pediatric Cardiology in UP-PGH which supported trainees
attached in different international training centres of their choice in their field of interest. Her inclination in the field
of advanced cardiac imaging was magnified when she went to lnstitut ]antung Negara in Kuala Lumpur Malaysia,
under the mentorship of Dr. Haifa Abdul Latiff in 2014 who was then already specialising in fetal echocardiography
and cardiac CT angiogram. Dr. Yap then subsequently joined the Division of Pediatric Cardiology in UP-PGH thereafter
performing non-invasive cardiac imaging which included transthoracic, transesophageal and fetal echocardiogram which
helped obtained more volume of congenital heart diseases carefully selected for heart surgeries and those amenable
with minimal interventional procedures to correct heart defects. Seeing hmv valuable multi modality imaging is in
congenital and structural heart diseases, she then further trained in advanced cardiac imaging which included advanced
echocardiography, cardiac CT and MRI in Birmingham Children's Hospital and was an honorary fellow in Evelina
London Children's Hospital from 2018to2019. Her main goal is to continue teaching and training our young cardiology
enthusiasts whilst strengthening the core of multi modality imaging in congenital and structural heart diseases both
pediatric and adult population. At present. she is a clinical associate professor and a consultant pediatric cardiologist in
the University of the Philippines• Philippine General Hospital. She is a Diplomate in Philippine Pediatric Society, Fellow
in Philippine College of Cardiology, Fellow of European Cardiovascular Imaging, a member of Society of Cardiovascular
Computed Tomography and Society of Cardiac Magnetic Resonance with Level Ill Accreditation.

Jerry V. Pua, RMT, MD, DPPS

Dr. Pua earned his Doctor of Medicine degree from UST Faculty of Medicine and Surgery on 2010. Took up Pediatric
Residency at PCMC (2012-2015) after passing the Physician Licensure Examination. Passed the PPS Diplomate
Examination on 2016 while under Fellowship Training in Pediatric Hematology and Oncology at PCMC. He became the
Chief Fellow of PCMC Cancer and Hematology Center from 2019·2020. Currently preparing for subspecialty diplomate
exam for Pediatric Hematology and Oncology. He enjoys a good food, book, music, movie and conversation

Romana Marie M. Vila, MD, FPCR, FCTMRISP

Dr. Vila graduated with a BS Biology degree in UP Diliman in 200 I. She obtained her medical degree from the College of
Medicine Pamantasan ng Lungsod ng Maynila in 2005. She finished her internship in the Jose Reyes Memorial Medical
Center last 2006 and passed the medical board exams in 2007. She had her Radiology Residency in University of the
Philippines - Philippine General Hospit.11, where she spend the I.1st ye.1r as Chief Resident. She further trained in CT/
MRI subspecialty in the same institution and was conferred a fellow in 2013. Currently, she is the Head of Radiology
Departments in De Vera Medical Center, Inc. and San Mateo Multicare Hospital.

Diosemil L. Leyson-Guzman, MD, DPPS, DPSN, DPNSP

Dr. Leyson-Guzman is a graduate of B.S. Biology degree from UP Cebu in 2005. She had her medical degree from the
West Visayas State University•College of Medicine and post graduate internship under the DOH-PCSHC. She had her
Pediatric Residency Training and her fellowship in Pediatric Nephrology at the Philippine Children's Medical Center.
She is a diplomate of the Philippine Pediatric Society, Philippine Society of Nephrology and the Pediatric Nephrologists
Society of the Philippines. She is currently practicing pediatric nephrology in several hospitals in lloilo City and a
lecturer at the West Visayas State University College of Medicine and Central Philippine University·College of Medicine.

ILLUSTRATOR
Joerelle V. Mojica, MD, FPCP, FPCC
Or. Joey Mojica completed her degree in B.S. Occupational Therapy at University of the Philippines · Manila in 2006,
Doctor of Medicine al the University of the East Ramon Magsaysay Memorial Medical Center in 2010, Internal Medicine
residency at Manila Doctors Hospital in 2014, Cardiology fellowship at Philippine General Hospital in 2018. and
Cardiac Electrophysiology and Pacing fellowship at St. Luke's Medical Center • Global City in 2019. She is currently
training for Cardiac EP in Universitair Ziekenhuis in Brussels, Belgium under Dr. Pedro Brugada. Joey enjoys watching
movies, eating out \.vith family and friends, and teaching Medicine to her younger sister, Chris. She's thrilled to be part
of something that cultivates the learning, this time, of many.

LAYOUT
EDITOR
Manuel S. Vidal, Jr., RCh
Mavi graduated with a BS Biochemistry degree, magna cum laude, valedictorian, from the De La Salle University- Manila
under the Star Scholarship Program, with several other awards: Excellence in Chemistry, Most Outstanding Thesis, and
Dr. Jose Rizal Honors Society inductee. He ranked second in the Chemistly Licensure Examinations in 2013. Currently,
he is an intern at the Philippine General Hospital under the MD-PhD Program, and aspires to be a future OBGYN
practitioner. On a personal note, his interests span current events, music, cookery, sports, and fragrances. He is very
grateful for the opportunity to be included in this ground-breaking work.
xiv
TABLE
OFCONTENTS
Preface vii
Foreword viii
Acknowledgment ix
Dedication X
Authors xi· xii
Editor xiii
Contributing Authors, Illustrator, Layout Editor xiv

CHAPTER 1:INTRODUCTION
TOPEDIATRICS
Section 1: History Taking and Physical Examination
1. History Taking in a Pediatric Patient 3
2. Sample History ofa Pediatric Patient 4
3. Vital Signs 5
4. Physical Examination of the Pediatric Patient 8
5. FRICHMOND Method of Endorsement 9
Section 2: Admitting Orders and Daily Rounds
1. Writing Admitting Orders 10
2. Tips on Doing Daily Rounds and Documenting Orders 10

CHAPTER
2:FLUIDSAND ELECTROLYTES
Section 1: Physiology ofBody Fluids 13
Section 2: Fluid Therapy 14
Section 3: Specific Electrolyte Disorders
1. Hypernatremia 21
2. Hyponatremia 23
3. Hyperkalemia 25
4. Hypokalemia 28
5. Hypocalcemia 30
6. Hypercalcemia 31
Section 4: Drips, Blood Transfusion, and Glucose Infusion 33

CHAPTER3:COMMON MEDICATIONS INPEDIATRICS

Section 1: Introduction to Prescription Writing
1. Overview of Prescription Writing 43
2. Parts ofa Prescription 44
3. Prescribing Medications in Pediatrics 44
Section 2: Common Drugs Used in Pediatrics
1. Antibiotics 46
2. Antituberculous Agents 51
3. Antibiotics for Topical/Ophthalmic/Otic Use 52
4. Antiparasitic Agents 53
5. Analgesics/Antipyretics 54
6. Anti fungal Agents 55
7. Antiviral Agents 55
8. Drugs Used for Allergy 56
9. Anticonvulsants 60
10. Anti hypertensives 62
11. Drugs Used for Gastrointestinal Complaints 63
11. Other Medications Used in Pediatrics 64

CHAPTER
4:INTERPRETATION
OFCOMMON
DIAGNOSTIC
TESTS
Section 1: Electrocardiogram
1. ECGWaves and Segments 69
2. Differences Between Pediatric and Adult ECG 70
3. Steps in Pediatric ECG Interpretation 72
4. Common ECGAbnormalities 80
Section 2: Arterial Blood Gas (ABG) Interpretation
1. Overview of Arterial Blood Gas (ABG) 83
2. Basic Steps in ABG Interpretation 83
Section 3: Reading Chest Radiographs
1. Overview ofRadiographs 91
2. Steps in Reading Radiographs 92
3. Common Pathologies and Findings 96
xv
CHAPTER
5:BASIC
PROCEDURES
Foley Catheter Insertion 101
Intravenous Line Insertion 103
Nasogastric/Orogastric Tube Insertion 104
Capillary Blood Sampling 106
Arterial Blood Sampling 107
lntraosseous Infusion 108
Umbilical Vein Catheterization 110
Lumbar Puncture 112
Needle Aspiration 113
Endotrachea\ Intubation via Direct Laryngoscope Technique 114
Mantoux Test and Interpretation (Tuberculin Skin Test) 116

CHAPTER
6:GROWTH
ANODEVELOPMENT
Section 1: Anthropometric Measurements
1. Overview of Anthropometric Measurements 119
2. Interpretation of Anthropometric Measurements 125
Section 2: Development
1. Introduction 130
2. Developmental Milestones 131
3. Second Year to Middle Childhood 133
4. Adolescence 133
Section 3: Common Behavioral Disorders
1. Autism Spectrum Disorder (ASD) 137
2. Attention-Deficit/Hyperactivity Disorder 138

CHAPTER
7:PREVENTIVE
PEDIATRICS
Section 1: Immunization
1. Overview of Immunization 141
2. Principles of Immunization 142
3. 2019 Childhood Immunization Schedule 143
4. Vaccines for Special Groups 146
Section 2: Preventive Pediatrics and Anticipatory Care
1. Screening Tests 149
2. Dental Care 150
3. Red Flags for Child Maltreatment 150
4. Infant and Child Nutrition 152
5. Deworming 153

CHAPTER
8:NEONATOLOGV
Section 1: Evaluating the Newborn 157
Section 2:Essential lntrapartum and Newborn Care 170
Section 3: Neonatal Resuscitation 173
Section 4: Breastfeeding 175
Section 5: Approach to Problems of the Newborn
1. Approach to Apnea 179
2. Approach to Bloody Stool 180
3. Approach to Abnormal Stool Patterns 181
4. Approach to Respiratory Distress 182
5. Approach to Oliguria and Anuria 183
6. Approach to Neonatal Seizures 184
7. Approach to Cyanosis 184
8. Approach to Jaundice 186
9. Approach to Eye Discharge 191
Section 6: Other Issues of the Newborn
1. Retinopathy of Prematurity 193
2. Multiple Gestation 193
3. Intrauterine Growth Restriction 194

CHAPTER9:INFECTIOUS
DISEASES
Section 1: Approach to Fever 199
Section 2: Infections in the Neonate
I. Neonatal Sepsis 202
2. Necrotizing Enterocolitis 204
3. Transplacental Infections 206

xvi
 Section 3: Viral Infections
1. Influenza 209
2. Herpes Simplex Virus [HSY) 210
3. Mumps 211
4. Viral Exanthems 212
5. Poliomyelitis 214
6. Dengue Fever 215
7. Rabies 221
8. Human Immunodeficiency Virus (HIV) 222
Section 4: Bacterial Infections
1. Streptococcal Infections 224
2. Staphylococcal Infections 226
3. Escherichia coli 227
4. Typhoid Fever 228
5. Meningococcemia 229
6. Pertussis 231
7. Tetanus 232
8. Tuberculosis 233
9. Leptospirosis 234
10. Bacterial Skin Infections 236
11. Other Bacterial Infections 237
Section 5: Fungal and Parasitic Infections 239
Section 6: Clinical Syndromes 240

CHAPTER
10:GASTROENTEROLOGY
Section 1: Approach to Gastrointestinal Complaints 247
Section 2: Gastrointestinal Disorders of the Neonate
1. Esophageal Atresia (EA)and Tracheoesophageal Fistula (TEF) 249
2. Hypertrophic Pyloric Stenosis 250
3. Hirschsprung Disease (HD) 251
4. Other Disorders Encountered in the Neonate 252
Section 3: Diarrhea and Gastroenteritis 254
Section 4: Disorders of the Gastrointestinal Tract
1. Gastroesophageal Reflux Disease (GERO) 259
2. Peptic Ulcer Disease (PUD) 260
Section 5: Obstruction in the Gastrointestinal Tract
1. Intestinal Obstruction 261
2. Some Causes of Intestinal Obstruction 262
Section 5: Disorders of the Liver and Pancreas
1. Overview of Viral Hepatitis (Hepatitis A-E) 264
2. Hepatitis B 265
3. Acute Pancreatitis (AP) 267
Section 6: Nutrition and Related Disorders
1. Severe Childhood Undernutrition (SCU) 268
2. Micronutrient Deficiency 269

CHAPTER
11:HEMATOLOGY
ANO
ONCOLOGY
Section 1: The Anemia
1. General Approach to Anemia 273
2. Anemias of Inadequate Production 273
3. Hemolytic Anemias 276
Section 2: Bleeding Disorders
l. GeneralApproachto Bleeding 279
2. Overview of Bleeding Disorders 279
3. Immune Thrombocytopenia (ITP) 280
4. Thrombotic Thrombocyt.openic Purpura 280
5. Hemophilia 281
6. Von Willebrand Disease 282
Section 3: Oncology
1.Leukemias 283
2. Lymphoma 285
3. Brain Tumor 286
4. Musculoskeletal Tumors 286
5. Abdominal Tumors 287

xvii
 12:PULMONOLOGY
CHAPTER
Section 1: Approach to Common Complaints 291
Section 2: Pulmonary Disorders of the Neonate
I. Respiratory Distress Syndrome (Hyaline Membrane Disease) 293
2. TransientTachypnea of the Newbron (TTN) 294
3. Persistent Pulmonary Hypertension of the Newborn (PPHN) 295
4. Meconium Aspiration Syndrome (MAS) 296
S. Bronchopulmonary Dysplasia (Neonatal Chronic Lung Disease) 298
6. Congenital Diaphragmatic Hernia (CDH) 299
7. Choanal Atresia 300
Section 3: Disorders of the Upper Respiratory Tract
1. Rhinitis (The Common Cold) 301
2. Sinusitis 302
3. Acute Pharyngitis 303
4. Peritonsillar Abscess 304
S. Retropharyngeal Abscess 305
6. Laryngotracheobronchitis (Viral Croup) 306
7. Acute Epiglotittis (supraglotittis) 307
8. Summary Table for Epiglotittis vs Croup 308
9. Bacterial Tracheitis 308
Section 4: Disorders of the Lower Respiratory Tract
1. Bronchiolitis 309
2. Acute Bronchitis 310
3. Pneumonia 310
4. Pulmonary Tuberculosis 313
S. Bronchial Asthma 318
6. Exacerbation of Bronchial Asthma 324
Section S: Disorders of the Pleura
1. Pleural Effusion 326
2. Pneurnothorax 329

CHAPTER 13:ALLERGYAND IMMUNOLOGY

Section 1: Allergy
1. Overview of Allergy 333
2. Allergic Rhinitis 334
3. Atopic Dermatitis (AD)/Atopic Eczema 336
4. Food Allergy 339
5. Urticaria (Hives) and Angioedema 341
6. Adverse Reaction to Drugs 342
7. Anaphylaxis 344
Section 2: Immunology 346

CHAPTER
14:RHEUMATDLDGY
Section 1: Approach to Rheumatologic Diseases
1. Overview of Rheumatologic Diseases 351
2. Approach to Musculoskeletal Pain/Swelling 352
Section 2: Disorders in Rheumatology
1. Juvenile Idiopathic Arthritis (JIA) 353
2. Systemic Lupus Erythematosus (SLE) 355
3. Reactive and Post-infectious Arthritis 357
4. juvenile Derrnatomyositis 358
5. Vasculitis Syndromes 360
6. Other Rheumatologic Disorders 361

CHAPTER 15:CARDIOLOGY
Section 1: Overview of the Circulatory System
1. Fetal Circulation 365
2. Transition at Delivery 366
Section 2: Approach to Common Cardiac Complaints
1. Approach to Chest Pain 367
2. Approach to Cyanosis 367
3. Approach to Common Pediatric Cardiac Murmurs 368
Section 3: Congenital Heart Diseases
1. Overview of Congenital Heart Diseases 369
2. Congenital Acyanotic Heart Diseases 370
3. Congenital Cyanotic Heart Diseases 375
xviii
 4. Obstructive Lesions 378
Section 4: Acquired Heart Diseases
1. Rheumatic Fever 382
2. Valvular Heart Disease 385
3. Infective Endocarditis 386
4. Kawasaki Disease 389

CHAPTER
16:NEPHROLOGY
Section 1: Approach to Hematuria
1. Overview of Hematuria 393
2. Glomerulonephritis 394
3. Evaluating a Patient with Gross Hematuria 397
Section 2: Approach to Proteinuria
1. Overview of Proteinuria 398
2. Nephrotic Syndrome 399
Section 3: Approach to Azotemia
1. Glomerular Filtration Rate (GFR) 402
2. Renal Failure 402
Section 4: Specific Disorders in Nephrology
1. Acute Poststreptococcal Glomerulonephritis (PSGN) 404
2. lgA Nephropathy or Berger Disease 405
3. Hemolytic-Uremic Syndrome (HUS) 405
4. Autosomal Recessive Polycystic Kidney Disease (ARPKD) 407
5. Urinary Tract Infection 407
6. Renal Tubular Acidosis 409

CHAPTER 17:ENDOCRINOLOGY
Section 1: Disorders of the Hypothalamus and Pituitary Gland
1. Overview of the Pituitary Hormones 413
2. Hypopituitarism 413
3. Hyperpituitarism 415
4 .. Diabetes lnsipidus (DI) 415
5. Precocious Puberty 417
Section 2: Disorders oft he Thyroid Gland
1. Overview of the Thyroid, Parathyroid.and Related Hormones 418
2. Hyperthyroidism 418
3. Hypothyroidism 420
Section 3: Disorders of the Adrenal Gland and Gonads
1. Congenital Adrenal Hyperplasia (CAH) 422
2. Addison Disease 423
3. Cushing Syndrome 424
4. Pheochromocytoma 425
5. Genetic Causes ofHypertrophic Hypogonadism 426
Section 4: Diabetes Mellitus
1. Diabetes Mellitus (DM) 427
2. Diabetic Ketoacidosis 428

CHAPTER 18:NEUROLOGY
Section 1: Approach to Neurologic Complaints
1. Neurologic Examination 433
2. Special Diagnostic Tests 436
Section 2: Congenital Anomalies in Neurology
1. Spinal Cord Malformation 438
2. Hydrocephalus 439
Section 3: Migraine and Headache
1. Approach to Headache in Children 441
2. Migraine 442
Section 4: Seizures and Epilepsy
1. Approach to Seizures 444
2. Seizures and Epilepsy 445
3. Febrile Seizures 448
4. Status Epilipticus 449
Section 5: Neurocutaneous Syndromes
1. Tuberous Sclerosis 450
2. Neurofibromatosis 451

xix
 Section 6: CNS Infections
1. Tuberculous (TB) Meningitis 452
2. Viral Meningitis 453
3. Bacterial Meningitis 453
4. Brain Abscess 455
Section 7: Neuromuscular Disorders
1. Guillain-Barre Syndrome 456
2. Cerebral Palsy (CP) 457

CHAPTER 19:ACUTECARE
Section 1: Pediatric Basic Life Support 461
Section 2: Pediatric Advanced Life Support and Arrhythmias
1. Bradyarrhythmias 463
2. Tachyarrhythmias 464
Section 3: Shock 467
Section 4: Foreign Bodies
1. Foreign Bodies in the External Auditory Canal [EAC) 471
2. Foreign Bodies in the Nose 471
3. Foreign Bodies in the Gastrointestinal Tract 472
4. Caustic Ingestions 473
Section 5: Burns and Electrical Injuries
1. Burns 475
2. Assessment of Extent ofBurns by Body Surface Area [BSA) 476
3. Electrical Injury 477
Section 6: Head Trauma 479
Section 7: Toxicology 481

CHAPTER20:ESSENTIALS INPEDIATRICS
Section 1: Examination of the Pediatric Patient
1. Vital Signs 485
2. Glasgow Coma Scale 485
3. Growth and Development 485
Section 2: Immunization Schedule 488
Section 3: Neonatology 489
Section 4: Fluids and Electrolytes 491
Section 5: Measurement of Commonly Used Equipment
1. Nasogastric Tube 495
2. Foley Catheter 495
3. Endotracheal Tube 495
Section 6: Laboratory Reference Values
1. Complete Blood Count 496
2. Blood Chemistry 497

CHAPTER21:BOARD
CORRELATES
Fluids, ECG,ABC, Chest Radiographs sos
Basic Bedside Procedures in Pediatrics sos
Preventive Pediatrics 506
Neonatology 507
Infectious Diseases 508
Gastroenterology 512
Hematology and Oncology 514
Pulmonology 516
Immunology 517
Rheumatology 518
Cardiology 519
Nephrology 519
Endocrinology 520
Neurology 522
Acute Care 523

xx
INTRODUCTION
TOPEDIATRICS

~ HISTORY TAKING AND PHYSICAL EXAMINATION

I. History Taking in a Pediatric Patient
II. Sample History of a Pediatric Patient
Ill. Vital Signs
IV. Physical Examination of the Pediatric Patient
V. FRICHMOND Method of Endorsement

0 ADMITTING ORDERS AND DAILY ROUNDS

I. Writing Admitting Orders
II. Tips on Doing Daily Rounds and Documenting Orders
 SECTION ONE I
.
HISTORY TAKING AND PHYSICAL EX~MINATION
HISTORY TAKING IN A PEDIATRIC PATIENT
Information during history taking in the pediatric patient almost completely
depends upon the caregivers. Key elements include establishing an appropriate
atmosphere and asking questions in a nonconfrontational manner. The following are the
components of history taking:
COMPONENT REMARKS
• Full name, date & place of birth, nickname, occupation of parents &
General data contact information
• Source of information (patient, parent, caregiver) & reliability
Chief complaint • The most important symptom which prompted the admission
• Must contain essential information like onset, duration, character,
severity, aggravating/relieving factors
History of • Must contain remedies given to relieve the symptom
present illness
(HPI) • If medications were given, note the type, dose, frequency, & response
• Include information regarding consultations done
• History must build up until the reason for confinement/consult
Review of systems • Other symptoms related to each organ system not included in HPl
Past medical • Childhood illnesses, accidents and injuries, operations,
history hospitalizations, and allergies
• lllnesses, both communicable and non-communicable, present in the
Family history
immediate family members and household members
• Elicit any concern in child's ability to associate with others, living
conditions, influences of community /school
Personal and • The ethnic and cultural milieu in which the family lives
social history • Family socioeconomic circumstances (e.g.,income, type of dwelling,
and neighborhood), parental work schedules, family interdependence,
support from relatives/friends/neighbors
• Home: inquire about all individuals living with the adolescent and
their relationships with these individuals
• Education/employment: name of school, year level, whether they
have recently changed schools, grades, favorite/least favorite
subject, relationship with teachers/classmates, experience with
bullying; for adolescents who are working, inquire about nature of
work, past employers, and relationships
• Activities: hobbies, recreational activities, sports or exercise, what
HEADSSS activities they do with friends, hours per day using the computer /TV
assessment • Drugs: inquire first whether their friends drink alcohol, smoke, or
(for adolescents) have used illicit drugs, before inquiring whether the adolescent has
tried or is currently drinking alcohol, smoking, or using illicit drugs
• Sexuality: dating history or relationships with same/opposite sex,
sexual activity, contraceptive use, pregnancy, sex education from
parents, secondary sexual characteristics, STDs; for females: ask
about menarche, last menstrual period, problems with menses
• Suicidality: depression, suicidal ideation, attempts to hurt self
• Safety: inquire if they feel safe at home/in neighborhood, presence
of guns at home, previous or current experience of abuse
Birth and • See Chapter 8 (Neonatology)
maternal history • For neonates, the HP! should start with the birth & maternal history

3
 • Important during infancy and childhood and in dealing with
problems of delayed development & behavioral disturbances
Growth and • Physical growth: actual ( or approximate) weight and height at birth
developmental
history and at 1, 2, 5 and 10 years, history of any slow or rapid gains or
losses, tooth eruption and loss pattern
• Developmental milestones
• Concerns in nutritional status contributing to the present illness
• Important during first 2 years of life & in dealing with problems of
Nutritional
under- and overnutrition
(feeding) history
• Breastfeeding, artificial feeding, preparation of milk, micronutrient
supplements, complementary feeding, and childhood eating habits
Immunization • Specific dates of administration of each vaccine
Source:BickleyLS,el al. Bales'guidelo physicalexamination andhistorylaking.6th ed.Philadelphia;
2007
Katzenellenbogen R. HEADSS:the reviewof systemsfor adolescents. VirtualMentor;2005
AthreyaB, et al. Pediatricphysicaldiagnosis.2nded.AnshanPublishers;2010

SAMPLE HISTORY OF A PEDIATRIC PATIENT

COMPONENT REMARKS
Juana De Leon, 3 year-old female, Filipino, Roman Catholic, born in Laguna
General data and presently residing in Tonda, Manila, was admitted for the first time at
(name of hospital) on July 1, 2019 at Sam.
Chief complaint Cough
The patient was apparently well until 2 days prior to admission (PTA), when
she developed productive cough and fever, with a maximum temperature of
39°C, relieved by intake of paracetamol at 10 mg/kg/dose every 4-6 hours.
History of 12 hours PTA,while aforementioned symptoms persisted, the patient experienced
present illness difficulty of breathing described by the parents as fast breathing with chest
indrawing. There was no cyanosis, convulsions, irritability or lethargy.
Due to persistence of symptoms, the parents decided to consult at the
emergency room where the child was subsequently advised admission
The patient has no changes in sensorium, rashes, jaundice, eye redness or
Review of discharge, naso-aural discharge, epistaxis, vomiting, diarrhea, constipation,
systems changes in urination, easy bruising, limitation of motion, edema, weakness,
or loss of consciousness
Past medical Patient has had no similar illness in the past, no previous hospitalizations and
history no previous surgery. She has no history of allergy.
The father has bronchial asthma and her maternal grandmother has
Family history hypertension. There are no other diseases in the family. No heredofamilial
diseases were reported. There is no family history of TB, hepatitis A or B,
nor are there any other chronic infectious conditions existing in their family.
Juana is the first child of Roberto and Sally. Her father works as a security
guard while the mother is a housewife. She has a 3-month old sister and
Personal and they live in a one-storey 2-bedroom house with adequate ventilation. Water
social history supply comes from Maynilad & distilled water is used for drinking. The
mother cooks the food eaten by Juana. Juana is able to feed herself. Their
toilet is flush type and garbage is collected by a dump truck 3 times a week.
The patient was born full term to a then 28 year-old GlPO mother at a local
hospital. The mother had no history of illness, alcohol intake, bleeding nor
exposure to infections or radiation during pregnancy. She had regular prenatal
Birth and consults with an 08-GYN. The mother did not take any medications during
maternal pregnancy aside from multivitamins.
history She was delivered by spontaneous vaginal delivery. She had good cry and
activity at birth with no meconium staining. Birth weight was 3 kg, length
was 50 cm and pediatric aging was 39 weeks, thus she was appropriate for
gestational age. She was immediately roomed-in a few hours after birth.

4
 Gross Motor: Rolled Over ; 4 months, Sat alone ; 8 months, Ran well ; 2
years, at present able to ride tricycle
I
.

Fine Motor: Hands not fisted; 3 months, Reached and pulled objects; 6
months, Linear scr·ibbles; 8 months, At present, draws circles
Growth and
developmental Language: Turned to sound ; 4 months, Babbled ; 7 months, First word
history "dada"; 12 months, Spoke in 2-word phrases; 2 years, At present, states
full name, age and sex
Personal / Social: Smiled responsively ; 3 months, Held and bit cracker;
6 months, Interactive games; 15 months, At present, removes garments,
engages in pretend play
Patient was fully breastfed and then shifted to Enfalac at 1:1 dilution at
3 months old. Enfapro A+ at the same dilution was started as follow-on
formula at 12 months. At 24 months, she was weaned from the bottle and
Nutritional
shifted to Nido 1+ at 4 scoops per glass. Complementary feeding was started
(feeding)
at 6 months. At 12 months, she was started on regular table food. Presently,
history
she eats a variety of food with preference for chicken, soup, fruits. Feeding
schedule comprises 3 main meals of about a cup full of rice, fish or chicken,
and fruits for dessert. Morning and afternoon snacks are given.
Juana was given BCG vaccine at birth. She has received 3 doses each of
hepatitis B, hemophilus influenza B, DPT, inactivated polio vaccine (6-in-1
Immunization
vaccine) at 6, 10 and 14 weeks. She received measles vaccine at 9 months of
history
age. She has not received pneumococcal, flu, MMR, hepatitis A, and varicella
vaccines.

VITAL SIGNS
The following table serves as a guide to check if the obtained vital signs are within normal range.
Howeve,; the range of normal values may va,y depending on the reference you use. Always
correlate the obtained measurements cli11ical/y.

AGE

Premature
l"=
..~1-1~
{beats/min)

120-170
(bre::min)

40-70 55-75 35-45

TEMP**

0-3 months 100-150 35-55 65-85 45-55

3-6 months 90-120 30-45 70-90 50-65 Rectal
temperature
6-12months 80-120 25-40 80-100 55-65
1-3 years 70-110 20-30 90-105 55-70 36.6-37.9°(
or
3-6 years 65-110 20-25 95-110 60-75 9 7.9-10 0. 2 ° F

6-12 years 60-95 14-22 100-120 60-75

>12 years 55-85 12-18 110-135 60-85
"RoutineBP measurementfor ~3 years old
--sites to measure temperature:rectum(preferredin infants& youngchildren),mouth,axilla

Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Elsevier:2019

5
I. RESPIRATORYRATE
• Based on WHO, the following values serve as the cut-off values per age for tachypnea
• These values are more commonly used in the clinical setting
AGE GROUP DEFINITION
Newborn >60 breaths/min
2-12 months >SO breaths/min
1-5 years >40 breaths/min
>5 years >30 breaths/min
Source:Haz1nsk1
MF.Manualof PediatricCnl1cal Care(1sted.).Elsevier,1999
BloomfieldD, et al. Tachypnea.
PediatricsIn Review;2002

II. BLOODPRESSURE(BP)
Determine the Blood Pressure using the Following Steps:
Taking the Blood Pressure using the Upper Extremities
• Child should be seated 3-5 minutes before measurement
• Right arm is usually recommended for standardization (since there is a possibility of
having false low readings in the left arm if there is coarctation of the aorta)
• Use the correct cuff size:
, Bladder length ;,BO% of the upper arm circumference
'Bladder width ;,40% of the upper arm circumference
• The lower part of the cuff is placed 2-3 cm above the antecubital fossa
• The bell of the stethoscope is then placed over the brachia! artery
• The cuff is inflated 20-30 mm Hg above the point at which the radial pulse disappears and
is then deflated at a rate of 2-3 mmHg per second
• The first and last Korotkoff sounds are measured and recorded with measurements read
to the nearest 2 mm Hg
Taking the Blood Pressure using the Lower Extremities
• Patient should be placed in prone position
• The cuff is placed at the midthigh and the stethoscope is placed over the popliteal artery
• Systolic BP in the legs is 10-20% higher than the arms
• Check the BP of all extremities to rule out diseases like coarctation of aorta

A. Detection of Hypertension
The following steps describe the ideal method for interpretation of blood pressure to detect
hypertension (table in previous page is only an approximation)
After determining the blood pressure as described above, follow the steps below:
Step 1. Determine where the Systolic and Diastolic BP Percentile Falls
Using the BP levels for age and height percentile, determine where the BP percentile falls
If the height fallsexactly in between two percentiles, choose the lower percentile cut-off
The BP percentile table is freely downloadable online

Step 2. Interpret the BP using the following table (Definitions of BP Categories & Stages)

INTERPRETATION* ----------~
I AGE
I 1 to <13 Years Old , _ 13 Years Old
Normal • <90'h percentile • <120/80 mmHg
• ;,90"" to <95'" percentile, or • 120/80 to 129/80 mrnHg
Elevated BP or
• 120/80 mmHg to< 95"'
Pre hypertension
percentile (whichever is lower)
• ;,95,h percentile to <95'" • 130/80 to 139/89mrnHg
Stage 1 percentile+ 12 mmHg, or
hypertension • 130/80 to 139/89 mmHg
(whichever is lower)
Stage 2 • ;, 95'" percentile+ 12 mmHg, or • ;,140/90 mmHg
hypertension • ;,140/90 (whichever is lower)
'Hypertensionis presentifchildmeets abovecriteriaon 3 or moreseparate occasions(differentclinicvisits)
6
Sample Case 1: An 8 year old male with a height of 141 cm sought consult for a general check-
up. BP on three separate determinations was 115/74 mmHg.
I
.

STEP FINDINGS FROM THE CASE

ARelyl SPP,recn:de

5¾ Hm 2:;% ®"' r:;" 90% s~"

·i·- .. ~--rr.---is5--to----·-·:;TS~r7-~m-
t1c,gn1M1 J7 8
t'e,gn11crn1 lZIJ 12ss m m m1 13n +1~1
,.,
SO<h

95th
"
107
$
,os
~

'"
ffl
110
~
Ill
~
112
100
112

Use the BP percentile 95th • i:immHV,

Ill

"' "'
"'
112

"' '"
'"
115
127
116
128
117

"'
1 table from standard
BP P~n:cnhle 09PfrnmH51
charts or tables A)iC !~I

l"e15rithn!
Hc1&IIIf~ml
·~
-H!
l21J
""'
.?~6
ins
1te1gritPerun:11eorMeasurtdHe1~nt

'ZS%
So
127
,,.
s~
'"19
,s...
1.SSI
...
S.is
"" +u1
95~
555
.,
,.,
""" 57

"
57
" "n 60

""
70

""
70
,Slh
"" "" "" " "
9:Sth•lJrnmllli;
" 81 87 81

• Based on the table the patient's BP falls between 90th and

2 Interpret 95th percentile
• This is therefore interpreted as ELEVATEDBP

Sample Case 2: An 8 year old male with a height of 141 cm sought consult for a general check-
up. BP on three separate determinations was 120/80.

STEP FINDINGS FROM THE CASE

BPPercentile

,..
S%
'"' "~ ""' "~ 95%

Ht,ght!Cfftl
~O!h
so:11
121 ~
95
IOi
,.
-,---ikililifit-,1----,1s·--4g-;---!IO--sis--~n-···--S'l""r-~
12.s;

10,
"'
" " "''
100
Ut
!Ill
110
13'>1
99
112
+1~1
,oo

,,.
112 112 '"
"' "'
~5th Ill

Use the BP percentile s,1n. 12,r.mH~

"' '" '"
"' IN
"'
"'
"'
1 table from standard At:tlrl BPPcrcen!ile DIJPlmmtlt)
charts or tables He,gh1i'erc,n11reorMeas1.1redHeIc111

'" 10li
~H
25%
"" "~
532 '"" "~
"'"
,-----Ht~ihn) ti6 !U6

..,,
~. 8 :;5$
Hc1ghtCcml
50th
121 ~ 12J.5
57 '"., 1351
s,
1.!88 ♦ 141

90th
51

",, 70 "
70
"
60

95th
" 7J
a; " " "" 75 "
75
9Sth•12rnmll)1:
" " 87
" "
• Patient's BP falls between 95th percentile and 95th + 12 mmHg
2 Interpret
• This is therefore interpreted as Stage 1 Hypertension

Sample Case 3: An Byear old male with a height of 141 cm sought consult for a general check-
up. BP on three separate determinations was 140/90 mm Hg.

• Blood pressure of;;,140 /90 mm Hg is automatically interpreted

as Stage 2 Hypertension

Source:The4th Reportonthe Diagnosis, Evaluation,andTreatment of HighBPin Children&Adolescents.Pediatrics;

2004
FlynnJT et al. ClinicalPracticeGuideline
for ScreeningandManagement of HighBloodPressure
Pediatrics;
in ChildrenandAdolescents. 2017

7
B. Detection of Hypotension
To determine if patient is hypotensive, the following cut-offs are used in practice (the values in
the above table derived from Nelson is only a quick reference)
AGE SYSTOLIC BLOOD PRESSURE (SBP)*
Term neonates (0-28 days) <60 mm Hg
Infants (1-12 months) <70 mm Hg
Children 1 to 10 years <70 + (age in years x 2) mmHg
Children >10 years <90 mm Hg
Source:Hazinski
MF.Manualof Pediatric
CriticalCare(1sted.).Elsevier:
1999

Sample Case 1: Interpret a BP of70/40 mm Hg for a term neonate.

• For a neonate, the lower limit of normal systolic blood pressure is 60 mm Hg
• The neonate is normotensive

Sample Case 2: Interpret a BP of 70/40 mm Hg for a 5 year old boy.

• For a 5 year old, the lower limit of normal systolic blood pressure is calculated as:
0 SBP =70 + (Sx2) =80 mmHg
• A blood pressure of70/40 mmHg is considered hypotensive for the 5 year old patient
since the SBP is <80 mm Hg

Sample Case 3: Interpret a BP ofS0/50 mm Hg for an 8 year old boy.

• For an 8 year old, the lower limit of normal systolic blood pressure is calculated as:
0 SBP = 70 + (8x2) = 86 mmHg

• A blood pressure of80/50 mm Hg is considered hypotensive for the 8 year old patient
since the SBP is <86 mm Hg

PHYSICAL EXAMINATION OF THE PEDIATRIC PATIENT

Clinicians employ various techniques to gain a patient's cooperation. Use of objects (e.g.,
toys, pictures) help in the examination of infants/toddlers/children. For infants and young
children, perform nondisturbing maneuvers first and potentially distressing maneuvers last.
For those old enough to understand, the clinician should explain what is going to be done during
the examination. Examination should be performed in the presence of a parent or guardian
and patient privacy should be respected. The order in which the examination is conducted is
age-specific & depends on the clinician's preference. See neurology chapter/or neurologic exam.

COMPONENTSOF THE PHYSICALEXAMINATION

COMPONENT NORMAL FINDINGS
• Patient is well-developed, well-nourished, not in cardiorespiratory
General Survey distress and coherent
• Patient looked her chronological age of_ years old
• Neither warm nor cold to touch
Skin
• No hypo/ hyperpigmentation, lesions, masses, nor cyanosis
• Head: symmetrical facial features, no palpable mass, hair with
normal texture and equally distributed
• Eyes: normal relationship of eyelids to the eyeballs, eyes not sunken,
anicteric sclerae, pink palpebral conjunctivae
HEENT • Ears: mobile pinna without masses or tenderness, no discharge
• Nose: symmetrical nose, midline nasal septum, equal size and shape
of the nares, no discharge
• Oral cavity: moist, no lesions, masses, tonsillo-pharyngeal congestion
• Neck: no cervical lymphadenopathy, no palpable thyroid mass

8
 • Inspection: no masses or lesions, deformities or defects on chest
wall, no lagging of respiratory movement, with regular and
symmetrical breathing, no retractions
I
.

Chest and lungs

• Palpation: no tenderness, masses, lesions; with equal tactile fremitus
• Percussion: resonant on percussion
• Auscultation: equal lung sounds on both fields, no crackles/wheezing
• Inspection: no precordial bulging nor lesions on the chest
• Palpation: no heave nor thrills, apex beat on the 4th !CS LMCL(Note:
Cardiovascular from birth to age 3 years, the apex beat is located at the 4th !CS
system LMCL,and gradually moves into the 5th !CSas the child grows)
• Auscultation: Sl louder than S2 on the apex, regular rate and
rhythm, no murmurs, bruits on carotid arteries
• Inspection: flat, non-distended, no scars or lesions, discoloration,
visible veins, visible peristalsis
• Auscultation: normoactive bowel sounds
• Palpation: soft, no tenderness on light and deep palpation, no
Abdomen palpable masses, liver edge palpable 1 cm below right costal margin
(Note: normal liver edge is up to 3.5 cm below right costal margin in
newborns and up to 2 cm in children)
• Percussion: tympanitic all over, intact Traubes space, no
costovertebral angle tenderness
• No masses, atrophy noted; symmetrical muscles on extremities
Extremities • No joint tenderness, normal range of motion
• Full and equal pulses, no clubbing or edema
Genital • Normal external genitalia, no skin lesions, no vaginal discharge (for
and rectal females), bilaterally descended testes (for males), intact rectal vault
examination • For adolescents, perform Tanner staging
Source:BickleyLS,el al. Bates·guideto physicalexamination andhistorytaking.6thed. Philadelphia;
2007
AthreyaB, et al. Pediatricphysicaldiagnosis.2nded.AnshanPublishers; 2010
AntiaAU,et al. Positionof theapexbeat.ArchDisChild;1978

FRICHMOND METHOD OF ENDORSEMENT

Fluids • Indicate present fluids and duration of administration

• Indicate if with electrolyte incorporation

Respiratory • Oxygen support if present

• Mechanicalventilation settings if applicable; indicate if adjustments are made
• Latest blood gas results and schedule of next determination

Infectious • Antibiotics given, dose (in mg/kg/day or mg/kg/dose), and number of

doses received (e.g., vancomycin Dl+l)
• Pending culture results
• Last febrile episode

Cardiac • lnotropic support, dose

Hematologic • CBC,PT/PTT, other bleeding parameters

• Blood transfusions given/pending
• Schedule of repeat blood sampling

Metabolic • Electrolyte results

• Electrolyte corrections if applicable

0 utput • Urine output in cc/kg/hr

• Fluid balance & other losses (e.g., vomiting, diarrhea, drainage from tubes)

N eurologic • Sensorium, GCSscore if applicable

• Anticonvulsant drugs if applicable; include dose and duration

Diet • E.g., NPO,diet for age, other special instructions

9
 SECTION TWO
ADMITTING ORDERS AND DAILY ROUNDS

WRITING ADMITTING ORDERS

• Admit under the service of ____________ (state name of attending physician or if using
HMO, under the name of the HMO coordinator first then state the name of the attending
MD who will actually manage and do the rounds of the patient)
• Admit to _________(room of choice: regular private room, or pedia ICU, or neonatal ICU,
or intermediate medical care unit - IMCU, or ward and bed number)
• How the patient should feed (NPO, full diet for age, state restrictions/known allergies
if present)
• IV fluids needed at the moment, the need for the patient to be re-assessed after fluid
resuscitation, if applicable, so that follow-up fluids could be carefully decided on)
• Diagnostics: include all laboratory exams and imaging modalities that need to be done
for the patient
• Therapeutics: all medications with properly computed doses, frequency, route of
administration (use only standard and universally accepted abbreviations; better to
spell out millileters and milligrams to avoid confusion)
• Frequency of monitoring vital signs, fluid input and urine output/stool output (e.g.,
monitor vital signs every 4 hours; monitor neurologic vital signs every 4 hours;
monitor urine output every 8 hours)
• Oxygen support, if needed (e.g., nasal cannula at 2 liters per minute)
• Watch out for: indicate pertinent signs of deterioration or signs of persistent
problematic symptoms (this should be specific and appropriate to the current clinical
condition of the patient)
• Inform pediatrics resident or pediatrics hospitalist or junior consultant of admission
(whichever is appropriate)
• If applicable, state whether there are other services that will be on board for the care of
the patient (e.g., refer to Surgery [and state name of attending MD if known at this time]
for further evaluation and management of abdominal pain)
• Refer accordingly

TIPS ON DOING DAILY ROUNDS AND DOCUMENTING ORDERS

• Always document on the left side of the chart the vital signs and pertinent PE findings
after assessing the admitted patient. Include the date and time you have seen the
patient. This is to justify why a certain diagnostic exam, change in medication, or
adjustment in fluids was warranted for that patient.
• Make sure the results of the initially requested diacrnostic tests are available and
inserted in the chart for correlation with the current cflnical condition of the admitted
patient.
• Communicate with the attending doctor/s of other service/s regarding the progress
of the patient.
• Communicate with the patient and their parents/guardians regarding the current
clinical status in words that would be easy to understand.

REFERENCES
1. Antia AU,MaxwellSR,GoughA, and Ayeni0. (1978). Position of the apex beat. Arch DisChild,53(7), 585-589.
2. Athreya 8, Pearlman SA,Zitelli 8 (2010). Pediatric physical diagnosis. 2nd ed. Anshan Publishers
3. Bickley LS,Szilagyi PG,& Bates 8. (2007). Bates' guide to physical examination and histo1y
taking. 6th ed. Philadelphia: Lippincott Williams & Wilkins.
4. Katzenellenbogen R. (ZOOS).HEADSS:the review of systems for adolescents. Virtual Ment01;
7(3):231- 233.
5. WolfADand LavineJE.(2000). Hepatomegalyin neonates and children. Pediatrics in Review,21(9), 303-310.

10
FLUIDS
ANDELECTROLYTES

~ PHYSIOLOGY OF BODY FLUIDS

0 FLUID THERAPY

~ SPECIFIC ELECTROLYTE DISORDERS

I. Hypernatremia
II. Hyponatremia
Ill. Hyperkalemia
IV. Hypokalemia
V. Hypocalcemia
VI. Hypercalcemia

~ DRIPS, BLOOD TRANSFUSION, AND GLUCOSE

INFUSION
 SECTION ONE

BASIC PHYSIOLOGY
PHYSIOLOGY OF BODY FLUIDS I
I. COMPOSITION OF BODY FLUIDS
• Total body water (TBW) percentages varies with age
75% of birth weight for term infant (higher in preterm infants)
0 By the 1st year of life, TBW decreases to adult values of 60%
0 Newborn extracellular fluid (ECF) is higher than the intracellular (ICF)
Hydrostatic and oncotic pressure maintain equilibrium between intravascular fluid and
interstitial fluid
lntravascular volume is crucial for tissue perfusion

Remember 60-40-20 Rule:

60% of Body Weight: Water
• 40% of body weight: ICF
• 20% of body weight: ECF
15% of Body weight: interstitial fluid
0 5% of Body weight: plasma (intravascular fluid)

OSMOLALITY
• ICF and ECF compartments are in osmotic equilibrium
• Osmotic pressure differences cause water movement between the ICF and ECF to equalize
the osmolality

Estimating ECF Osmolality

ECF osmolality

• Na: in mmol/L
= 2(Na') +Glucose+ BUN • Glucose and BUN: in mg/dL
-- • Normal value= 285-295 mOsm/kg
18 2.8

Corrected Sodium (Na•)

Corrected Na• • RBS: in mg/dL
• For every increase of glucose by 100 mg/dL
= Actual Na•+ 0.016 (RBS - 100) above lOOmg/dL, Na· is expected to increase
by 1.6 mEq/L

Hyperglycemia causes increased osmolality which causes movement of water from ICF to
ECF and dilutes Na•
Source:KliegmanR, et al. Nelsontextbookof pediatrics(21sted.). Philadelphia:Elsevier:2020
PhilippinePediatricSociety,Inc.Consensusstatements.:2017

13
 SECTION TWO
FLUID THERAPY

OVERVIEW OF FLUID THERAPY

Calculations for maintenance and deficit therapy stated in this book are simply guidelines
and not absolute requirements. Clinical and/or laboratory assessment are more important
in determining the appropriate fluid therapy

I. INDICATIONSFORSTARTINGINTRAVENOUSFLUIDS (IVF):
• Acute volume expansion
• Correction of fluid and electrolyte imbalances
• Compensation for ongoing problems that may affect fluid and electrolyte levels
• Maintenance therapy for patients who cannot be fed enterally to prevent dehydration,
electrolyte disorders, ketoacidosis, protein degradation

II. CLASSIFICATIONOF FLUID THERAPY (discussed in detail below)

TYPE REMARKS
• Given to compensate for ongoing losses
• Sensible losses (e.g., urine output, fecal fluid) make up the majority of
Maintenance
ongoing losses
Therapy
• Other losses: insensible losses (e.g., respiration, perspiration)
• Requirements for children are higher than those for adults
Deficit • Deficit fluids: refers to fluids lost prior to medical care (e.g., GI losses from
Therapy vomiting/diarrhea, significant blood loss, and inadequate fluid intake)
• Defined as those given to meet ongoing losses while on medical
Replacement
treatment (e.g., losses from chest tubes, uncontrolled vomiting/
Therapy
diarrhea, externalized cerebrospinal fluid shunts)

1) MAINTENANCE THERAPY
• Requirement depends on the age and clinical status of the patient
Healthy adolescents can tolerate nothing per orem (NPO) for 12-18 hours
Infants & children must be started on maintenance therapy ifNPO duration exceeds 8 hours
0

I. COMPUTATIONOF MAINTENANCEFLUID BASED ON BODY WEIGHT

• Several methods are used to compute the daily maintenance fluid requirement,
depending on the clinical situation and clinician's preference
• The usual maximum maintenance fluid per day is 2400 mL, equivalent to 100 mL/hour

A. Holiday-Segar Method (Weight Based)

0Most commonly used worldwide
0May overestimate energy expenditure in hospitalized children
May cause overestimation of water requirement as body weight increases
0May be used in obese & overweight children by using the 50th percentile of body
weight based on child's height
0Not used in neonates

WEIGHT MAINTENANCE THERAPY (FOR 24 HOURS)

0-10 kg • 100 mL/kg
11-20 kg • 1000 mL + 50 mL/kg for each kg in excess of 10 kg
>20kg • 1500 mL + 20 mL/kg for each kg in excess of 20 kg

14
 B. Ludan Method (Basal Caloric Expenditure-based)
Widely used in the Philippines, but not internationally
° Closer to basal caloric expenditure

WEIGHT
0-10 kg • 100 mL/kg
MAINTENANCE THERAPY FOR 24 HOURS
accepted

I
10-20 kg • 75 mL/kg
20-30 kg • 50-60 mL/kg
30-60kg • 40-50 mL/kg

C. Crawford Formula (Body Surface Area or BSA-based)

Consider use in critically-ill obese patients, acute and chronic kidney disease, malignancy,
severe sepsis
More difficult to remember and compute
Underestimates requirements in children 6-15 kg (ideally used in children >10 kg)
Body surface area (m')
Maintenance Therapy= 1500 mL/BSA
( in 24 hours)
Weight in kg x length in cm
3600
Source:KliegmanR, et al. Nelsontextbookof pediatrics(21sted.). Philadelphia:Elsevier;2020
PhilippinePediatricSociety,Inc.Consensusstatements;2017
CrawfordJ, el al. Pediatrics;1950
HollidayM & Segar,W.Pediatrics;1957
Del Mundo,et al. Textbookof Pediatricsand ChildHealth.JMC PressInc; 1947

Sample Case for Computation of Fluids

A 4-year old female is admitted for an appendectomy and was placed on NPO. Weight is
16 kg and height is 103 cm. Patient has no signs of dehydration. Compute for maintenance
fluid using the various methods described above.

IVF RATE
COMPUTATION OF (Total amount/
METHOD
MAINTENANCE THERAPY number of hours
infused)
First 10 kg = 1,000 mL
Holliday-
6kgx50mL/kg = 300 mL 54 mL/hour
Segar Method
Total = 1,300 mL in 24 hours
Ludan
16 kg x 75 mL/kg = 1,200 mL in 24 hours SOmL/hour
Method
BSA= ✓[(16 kg x 103 cm)/3600]
Crawford = 0.68 m 2 43 mL/hour
Method
1,500 mL/m' x 0.68 1112 = 1020 mL in 24 hours
Note the differences in results depending on the method used. This highlights the importance of
individualizingfluid requirements

15
II. MAINTENANCE ELECTROLYTE REQUIREMENTS
• Depends on the patients' weight and clinical condition
• The water and electrolyte requirements of patients dictate the appropriate fluid to be used
Recommended Electrolyte Requirements
Na (Na•) • 2-3 mEq/kg/day
Potassium (K•J • 1-2 mEq/kg/day
Chloride (Cl") • 2-4 mEq/kg/day
• 0.5-1 mmol/kg/day (200-500 mg/kg/day Ca2·gluconate, maximum
Calcium (Ca2 •J
2 g/day in infants and 3-4 g/day in older children)
Magnesium (Mg'•) • 0.25 mEq/kg/day
Phosphate' • 2 mEq/kg/ day
Sources:Avner,E.,et al. PediatricNephrology (7thed.).London:Springer;2016
KliegmanR, et al. Nelsontextbookof pediatrics(21thed.).Philadelphia:
Elsevier;2020

III. COMPOSITION OF AVAILABLE IV FLUIDS & COMPARISON WITH PLASMA

Fluids

Plain Normal pNSS = 308

Saline Solution 154 0 154
(pNSS) or D5NSS
D5NSS = 560
Plain 0.45 NaCl Plain 1/2 NS= 154
(1/2 NS)' or D5 77 0 77
1/2 NS D5 1/2 NS= 410
D5 0.3 NaCl 51 0 51 355
3%NaCl 513 0 513 1026

Lactated Ringer's Plain LR (pLR) = 274

130 4 109
(LR) D5 LR= 525
D51MB 25 20 22 350
D5NM 40 13 40 368
Isotonic
Electrolyte 145 4 127 276
Solution (IES)
'½ NS is not readilyavailableand is usuallycustom-made.

IV. RECOMMENDATIONS FOR !VF SELECTION

• Studies recommend the use offluids with 77-140 mEq/L of Na+ (options include½ NS,
LR or !ES, with or without incorporation of dextrose and K+)
• Isotonic fluids are best for volume resuscitation (e.g., pNSS, pLR, !ES)
• Avoid hypo tonic fluids due to increased risk of hyponatremia
• Custom-made solutions (cocktail fluids) are used for patients with significant
pathophysiologic derangements
• The following choice of fluid is commonly used in local practice. However, this is not
supported by studies and the authors do NOT recommend its use:
0 D5 1MBfor patients <20 kg
0 D5 NM for patients >20 kg
Source:PhilippinePediatricSociety,Inc.Consensusstatements;
2017
16
2) DEFICIT THERAPY
I. COMPUTATION OF FLUID DEFICIT
• The best way to determine the level of fluid deficit is by comparing the patient's weight to
the pre-illness weight

Fluid Deficit(%) = Pre-illness weight -Admission weight x 100

I
pre-illness weight

Sample case: A 2 year-old boy was admitted for diarrhea of 2 days. His pre-illness weight
is 11 kg and on admission he weighed 10 kg. Calculate the fluid deficit:

Fluid deficit= [(11-10)/11] x 100 = 9%

Source:Ph1hpp1ne
Pediatric
Society,
Inc.Consensus 2017
statements:

II. APPROXIMATION OF FLUID DEFICIT

• Clinical signs of dehydration and the degree of dehydration should be determined for
estimation of fluid deficit
• When classifying a child for the degree of dehydration, the higher classification takes
precedence (e.g., a child who is hypotensive with decreased tears and a capillary refill of
2 seconds is classified as having severe dehydration)
• Some parameters may not be accurately interpreted in patients with severe malnutrition,
but the thirst, sensorium, extremities, pulses and urine output remain accurate
• Table below combines recommendations (from WHO, Nelson and NICEguidelines) to
classify severity of dehydration
NONE TO MILD MODERATE SEVERE
PARAMETERS
DEHYDRATION DEHYDRATION DEHYDRATION
Sensorium* Alert Irritable Lethargic
Thirst* Drinks normally Thirsty, drinks eagerly Not able to drink
Skin Turgor* Goes back quickly Goes back slowly Goes back very slowly
Eyeballs* Normal Sunken Sunken
Respiratory Pattern Normal Tachypneic Deep and labored

Capillary Refill Time Normal >1.5 seconds >3 seconds

Heart Rate Normal or tachycardic Tachycardic Tachycardic
Rapid and weak
Peripheral Pulses Normal or increased Rapid
or absent
Blood Pressure Normal Normal Low
Urine Output Decreased Little or absent No urine output
Extremities Warm to touch Cool and pale Cold and pale
Mucous Membranes Normal Dry Parched
Tears Normal Decreased No tears
'The firstfour parameters are used in the WHOGuidelineson Treatmentof Diarrhea (see chapter 11for the
WHOclassificationof dehydrationin diarrhea)

Fluid Deficit in% BW (equivalence in mL/kg):

Infants <5% (SO mL/kg) 5-10% (50-100 mL/kg) >10% (>100 mL/kg)
Older children <3% (30 mL/kg) 3-6% (30-60 mL/kg) >6% (>60 mL/kg)

Commonly Used Values for Fluid Computation:

Infants SOmL/kg 100 mL/kg 150 mL/kg

Older children 30 mL/kg 60 mL/kg 90 mL/kg

17
 Sample Case: 4 year-old male is admitted for 2-day history of diarrhea and vomiting. On
physical examination, patient is irritable, normotensive, with sunken eyeballs and skin
turgor goes back slowly. Weight: 10kg
Classify the patient's degree of dehydration.
Answer: Moderate dehydration

Compute for the estimated amount of fluid deficit.

Moderate Dehydration= 60ml/kg = 60 X 10 = 600ml
Source:WorldHealthOrganization. Thetreatmentof diarrhoea.Geneva;2005
NationalInstitutefor HealthandClinicalExcellence:2009
KliegmanR,et al. NelsonTextbook
of Pediatrics(21sted.).Philadelphia: Elsevier;2020
FriedmanJN,et al. J Pediatr;2004

III. PHASES OF FLUID ADMINISTRATION

• Awareness of the different phases will lead to the best possible outcome and prevent
complications
• Frequent monitoring and re-evaluation are important
• The following parameters should be monitored:
0 Vital signs
Intake and output
Physical examination (especially daily weight monitoring)
Electrolyte levels
PHASE DESCRIPTION
Resuscitation • Emergency or life-threatening condition
Phase • Fluids administered as bolus
Optimization • Patient is unstable but already pulled out of the life-threatening condition
Phase • Fluids given more slowly
• Stable patient
Stabilization • Focus on organ perfusion
Phase • Fluids are given for physiologic maintenance and replacement of
ongoing losses
Evacuation
• Further fluid administration becomes harmful
Phase
Source:Malbrain,M.. et al.Anaesthesiology Intensive
Therapy;2014
KliegmanR, et al. Nelsontextbookof pediatrics(21sted.).Philadelphia:
Elsevier;2020
PhilippinePediatricSociety,Inc.Consensus statements;2017

18
IV. STEPS IN INTRAVENOUS(IV) MANAGEMENTOF FLUID DEFICIT
• Oral rehydration is still the safest and preferred method to correct fluid deficit
• The table below summarizes the steps in IV management of fluid deficit

AMOUNT OF FLUID TYPE OF FLUID REMARKS

I
STEP 1: ASSESS THE DEGREEOF DEHYDRATION(see "Severity ofDehydration'J
.. If with severe dehydration: proceed to step 2
If with mild to moderate dehydration: proceed to step 3

STEP 2: INITIAL RESUSCITATION

• 20 mL/kg bolus in 5-20 • Dextrose-free crystalloid • Giving large volumes of

minutes via "push-pull"
method* with large bore
catheter
• If with myocardial
dysfunction: 5-10 mL/kg
given over 10-20 minutes
with Na· of 130-154 (e.g.,
pNSS, pLR, IES)
• LR is avoided in liver
injury & metabolic
alkalosis
chloride-rich fluids (e.g., NSS)
results in hyperchloremic
metabolic acidosis
• Maximum fluid: 3 boluses
( 60 mL/kg) in most cases
• Resuscitation is successful
once there is normalization of:
0 Perfusion
0 Mental status
0 BP and urine output

STEP 3: CALCULATE24 HOUR FLUID REQUIREMENTS

• It is computed as
(Maintenance+ Deficit)
minus the amount of
fluids given during initial
resuscitation
• The first half of the amount
is given in 8 hours
• The remaining half is given
in the next 16 hours
• Dextrose-containing
crystalloid with Na· of
77-140mEq/L (e.g., D5
0.45NS, D5 LR, D5 JES)
• When possible, oral
rehydration therapy
is recommended over
intravenous rehydration
• Constant re-assessment is
important

STEP 4: REPLACEONGOINGLOSSES

• Given to patients in addition to the maintenance therapy in cases of ongoing fluid losses
• Refer to "replacement therapy" on the next page (see below)

"'Push-pull" method: where fluidsare intermittentlydrawn into a syringe from an infusionbag and manually
administered to the patient

Sample Case:
A 4 year-old male, weighing 15 kg, was brought in for 5 days history of diarrhea and
vomiting. On assessment, BP is palpatory 80 mm Hg, lethargic, sunken eyeballs, dry lips,
with poor skin turgor.

Assess degree of Patient has severe dehydration

1
dehydration
Initial Resuscitation= 15 kg x 20 mL/kg = 300 mL
Initial
2 Sample order: give 300 mL pNSS IV bolus
resuscitation
("push-pull" technique over 5-20 minutes)

19
 Continuation of the Case: After the first bolus, the patient is out of shock. Proceed with
computing for the 24-hour fluid requirements
First step is to compute for the Maintenance and Deficit
• Maintenance fluid requirement (using Holliday-Segar)
= 1250 mL
• Fluid deficit (for severe dehydration)
= 90 mL/kg x 15 = 1350 mL
Next is to compute for the 24-hour fluid requirement:

24-hour Fluid Requirement

=(Maintenance+ Deficit) - Initial bolus given above
24-hour fluid = (1250 mL + 1350 mL) - 300 mL
3
requirement = 2300 mL

• Half of computed value to be given in 8 hours

= 1150 mL for 8 hours (or 144 mL/hr)
• Half of computed value to be given in 16 hours
= 1150 mL for the next 16 hours (or 72 mL/hr)

Sample order: Shift JV fluids to D5 LR 1 Lat 144 mL/hr for

8 hours, then decrease rate to 72 mL/hour for the next 16
hours
4 Replace ongoing losses See "Replacement Therapy" below

3)REPLACEMENTTHERAPY
• Given in addition to maintenance therapy in cases of ongoing fluid losses (e.g., from GIT)
• For previously healthy and well-nourished patients, 1·eplacement of ongoing losses with
reduced osmolarity ORS is preferred (glucose 75 mmol/L, Na+ 75 mmol/L, K+ 20 mmol/L,
Cl- 65 mmol/L, citrate 10 mmol/L)
• Replace losses volume per volume every 1-4 hours
IV replacements are usually infused for 30 minutes to 1 hour (given at a slower rate for
those with congestion, fluid overload, and in malnourished patients)
Presented below are the specific recommended fluids depending on the type of losses
I
TYPES OF FLUID APPROACH TO REPLACEMENT***
• Reduced osmolarity ORS is the replacement fluid of choice
Diarrhea* • Options for those who cannot tolerate ORS: pLR or D5LR
(advantage of preventing hypoglycemia)

Emesis or nasogastric
• NSS + 10 mEq/L KC!
losses**
Surgical drains and chest tube • NSS or LR
Third space losses (burns) • NSS or LR or 5% albumin

• D5 ½NS+/- KCIfor insensible fluid losses

Polyuria • Replace urine output volume per volume with solution
based on measured urine electrolytes

Blood Joss • Warmed crystalloid (NSS, LR, JES), colloid or blood

'Composition of diarrhea: Na· 55 mEq/L, K· 25 mEq/L, HC0 3 15mEq/L
'"Composition of emesis of nasogastric losses: Na· 60 mEq/L, K· 10 mEq/L, Cl· 90 mEq/L
... The common clinicalpractice the Philippines is to use LR for replacement since it is easier to remember
and readily available. Unless the patient has significantderangements in fluid/electrolytes,LR may suffice.
Source:Kliegrnan
R, et al. Nelsontextbookof pediatrics(21sted.).Philadelphia:
Elsevier:2020
PhilippinePediatricSociety,Inc.Consensus statements.:
2017
20
 SECTION THREE
SPECIFIC ELECTROLYTE DISORDERS I
HYPERNATREMIA
I. ETIOPATHOGENESIS
• Hypernatremia is defined as serum Na· level> 145 mEq/L
• Mechanisms:
Excessive Na·: improperly mixed milk formula, iatrogenic
Water Deficit: diabetes insipidus, increased insensible losses, inadequate intake
Water and Na· Deficits: diarrhea, emesis, osmotic diuretics, chronic kidney disease,
burns, acute tubular necrosis, osmotic cathartics (e.g., lactulose), OM

Source:KliegmanR. et al. Nelsontextbookof pediatrics(21sted.).Philadelphia:

Elsevier:2020

II. MANIFESTATIONS
• Dehydration
• Doughy feel of skin during abdominal skin pinch
CNS manifestations: irritability, restlessness, weakness, lethargy, seizures, coma
• Fever
Hyperglycemia and hypocalcemia
• Most dreaded complication is brain hemorrhage

III. MANAGEMENT
A. Address the Underlying Cause

ETIOLOGY MANAGEMENT
Mild/moderate dehydration in AGE • Oral rehydration
• Priority is restoration of intravascular volume
Hypernatremic dehydration by giving isotonic boluses 10-20 mL/kg
• Fluid of choice: pNSS
Central diabetes insipidus • Desmopressin
Nephrogenic diabetes insipidus • Thiazide diuretic
Iatrogenic hypernatremia • Decrease or stop sodium infusion

B. Other Aspects in Management

• Decrease Na· by less than 12 mEq/L in 24 hours or at a rate of0.5
Goal
mEq/L/hr to avoid cerebral edema
• IVF with Na•< 77 mEq/L (1/2 NS, 0.3NS, 1MB,NM) at a computed rate
Fluid of of maintenance therapy plus 30-50 mL/kg over 48 hours
choice (or more depending on Na+ levels), or drink ad libitum
• Boluses given are subtracted from the total fluid computation
• Indicated for severe hypernatremia not responsive to fluid
Dialysis
management

21
IV. SAMPLESTRATEGYFOR TREATMENT OF HYPERNATREMIA
Step 1: Restore intravascular volume in patients who are severely dehydrated
May give pNSS 20 mL/kg over 5-20 minutes via "push-pull" technique
May repeat as necessary, usually up to 3 boluses

145-157 24
158-170 48
171-183 72
184-196 96

Step 3: Compute for amount of fluid to be given and determine IVF rate
Maintenance fluid+ 30-50 mL/kg given over desired correction time
• If boluses are given in Step 1, subtract it from the total fluid to be given
• If sodium is to be corrected in 48 hours, maintenance fluid is computed for 48 hours
• Example: the rnaintenance fluid of a 15 kg child is 1250 rnL for 24 hours, 2500 mL for
48 hours, 3750 mL for 72 hours and so forth
Initial fluid: D5 1/2NS +/- 20mEq KCL
Do not give KCL unless patient has established adequate urine output

Step 4: Monitor and adjust

Monitoring is essential
0 Na+ is monitored daily or as frequent as every 6-8 hours depending on severity
RESPONSE ADJUSTMENT OF FLUID
If with signs of volume depletion • See Step 1
• Increase sodium content of !VF or decrease
Sodium decreases too rapidly
rate of !VF
Sodium decreases too slowly • Decrease sodium of !VF or increase rate of IVF

Step 5: Replace losses as they occur

0 Refer to section on replacement therapy

Sample Case: A 15 kg male carne in due to vorniting and diarrhea for 5 days. BP 90/60
rnrnHg. He is awake, responsive, has sunken eyeballs & dry lips. Sodium level is 160
mEq/L. How would you manage this case?

1 Restore volume Patient has mild dehydration, proceed to step 2

Determine Because initial Na+ concentration is within 158-170 mEq/L, the
2
correction time correction time is 48 hours (based on table above)

Amount of fluid= (maintenance+ 30 mL/kg) - bolus

Amount of fluid= 2500 mL (this is the maintenance cornputed

Amount of fluid using Holliday Segar computed for 48 hours or 2 days)+ 450
3
given & IVF rate ml (this is 30 ml/kg) - 0 mL (no bolus given)= 2950 mL to be
given over 48 hours
• !VF rate= 2950 mL / 48 hours= 61 mL/hr
• Order: !VF DS 1/2NS lL at 61 mL/hr
Monitor and • Monitor and re-adjust accordingly
4
adjust • Incorporate K· or other electrolytes as needed
Source:KliegrnanR. et al. Nelsontextbookof pediatrics(21sted.).Philadelphia:
Elsevier:2010
PhilippinePediatricSociety.Inc.Consensus statements onparenteralOuidtherapy:2017
22
HYPONATREMIA
I. ETIOPATHOGENESIS
•
•
Hyponatremia is defined as serum Na· level< 135 mEq/L
Infants are more prone to hyponatremia from excess water ingestion because they have
lower GFR (e.g., water being offered to infants <6 months old)
I
II. MANIFESTATIONS
• Increase in intracellular water causes cells to swell (brain swelling) which leads to
increased intracranial pressure and impairment of cerebral blood flow
Acute and severe hyponatremia leads to brainstem herniation and apnea
• Common symptoms: anorexia, nausea, emesis, malaise, lethargy, confusion, agitation,
headache, seizures, coma, decreased reflexes
Other symptoms: Cheyne-Stokes respiration, muscle cramps and weakness, rhabdomyolysis

III. APPROACHTO DIAGNOSISOF HYPONATREMIA

Sodium(Na·)<135mEq/L

Checkosmolality

NormalOsmolality tigh Osmolality• I LowOsmolality I

,--------------------
t

.~-------~.--------_
~ :
:_Pseudohyponatremia , Hyperglycemia , I TrueHyponatremiaj
: Mannitoladministration:
·--------------------· IAssessvolumestatus
Euvolemia Hypovolemia
6 Hypervolernia
1
Spot UrineNa· >20mEq/l

,~
__________
L_________SIAOH<
SpotUrineNa·< 10 mEq/l I ~tUrineNa'>20mEq/L I SpotUrincNa'<lOmEq/l

--- ---- +__---- ----, ---------- ------

: Acutekidneyinjury : : NephroticSyndrome
Hypothyroidism Vomiting : Chronickidneydisease 1 : Heartfailure
Glucocorticoid
deficiency Diarrhea :: Cirrhosis
Thirdspacing ,:
,:
'
'------------------'-----------------
'

a. Pseudohyponatremia: laboratoryartifactseenwith very highconcentrationof proteinor lipid

b. Hyperosmolality:causesNa· to moveout into extracellularspace,therebydilutingNa· concentration
c. SIADH:retentionof waterwhichdilutesthe serumNa·
d. Hypovolemichyponatremia:patienthas a deficitin totalbodywater& a largerdeficitin total bodyNa·
e. In extrarenalcauses,urineNa· is <10 mEq/Lbecausethe kidneyconservesNa· to maintainintravascularvolume
f. Hypervolemic
hyponatremia:dueto excesstotalbodywater& Na·,buttheincreasein wateris greaterthanincreasein Na·

Source:
Kliegman
R,etal.Nelson
textbook
of pediatrics
(21sted.).Philadelphia:
Elsevier;
2020
PhadkeK.etal.Manualof Pediatric
Nephrology.London:
Springer:
2014

23
IV. MANAGEMENT
A. Management based on Underlying Pathophysiology
PATHOPHYSlOLOGY MANAGEMENT
• Restore intravascular volume with isotonic saline
Hypovolemic hyponatremia
• Restoration of intravascular volume takes priority
Hypervolemic hyponatremia • Water and sodium restriction

• Eliminate excess water intake

Euvolemic hyponatremia • SIADH: fluid restriction
• Hypothyroidism and cortisol deficiency:hormone replacement

B. Specific Management

• Given hypertonic saline

• Goal is to increase serum sodium by 5-6 mEq/L in the first 1-2 hours
Symptomatic
• Hypertonic saline (3% NaCl) 4-6 mL/kg given over 1-2 hours
patients
0 3% NaCl is concocted from readily available NaCl 2.5 mEq/ml
(e.g., seizures,
0 1 ml of 2.5 mEq/ml NaCl+ 4 ml sterile water= 5 ml 3%NaCI
coma)
• Monitor serum Na+, volume status, urine output, acid base status
• Clinical improvement often noted after administration of 3% NaCl

• Goal is to increase sodium by not more than 12 mEq/L in 24 hours or 18

mEq/L in 48 hours to prevent central pontine myelinosis (presents with
confusion, agitation, and flaccid or spastic quadriparesis)
Asymptomatic
• For patients without fluid deficit, if Na• is <13Smmol/L, decrease drip
patients
rate to 50-80% of maintenance fluid requirement
• Sodium is corrected slowly usually over 48 hours
• Frequent monitoring of electrolytes and adjustment offluid is essential

Sample Case: A 3 year old, 15 kg patient came in due to 3 day history of vomiting and LBM
>10 episodes per day, in active seizures. BP= 90/60 mm Hg, HR= 110 bpm, RR =30/min. He
had dry lips & sunken eyeballs. Hgt= 90 mg/dL. Na= 120 mEq/L

• 3% NaCl 4 mL/kg x 15 kg= 60 mL

Give hypertonic • 3% NaCl concocted as 1 mL of 2.5 mEq/mL NaCl+ 4 mL sterile
1 saline (patient is water= 5 mL 3% NaCl
symptomatic) • Order: Prepare 12 mL of2.5 mEq/mL NaCl+ 48 mL sterile water
to make 60 mL 3% NaCl. Give 60 mL 3% NaCl over 1 hour.

Once patient is • If with fluid deficit: see section on deficit therapy

2 symptom-free, • If without deficit: decrease drip rate to 50-80% of maintenance
assess fluid deficit fluid requirement
Continuation of the case: After hypertonic saline administration, seizure resolved. Patient was
also hydrated using isotonic fluids. After hydration repeat Na level is 130. There are no signs of
dehydration on physical examination but patient is still unable to tolerate oral intake. How will
you correct the hyponatremia?

Maintenance fluid using Holliday-Segar for a 15 kg child:

• 1st 10 kg= 1000 mL
• 5 kg x 50 = 250 mL
• Total = 1250 mL in 24 hours= 52 mL/hr

!VF rate = 50% of maintenance fluid requirement

= 50% of52 mL/hr
= 26 mL/hr
Order: lVF pNSS or D5NSS at 26 mL/hr
Sources:Philippine
Pediatric
Society,Inc.Consensusstatementson parenteralfluidtherapy:2017
PhadkeK.et al. ManualofPediatricNephrology.
London: Springer:2014

24
HYPERKALEMIA
I. ETIOPATHOGENESIS
• Defined as serum K+ >6 mEq/L in newborn and >5.5 mEq/L in older children
• Refer to essentials chapter for normal range of potassium levels depending on age group
I
MECHANISM MANAGEMENT
Spurious laboratory values • Hemolysis, tissue ischemia, thrombocytosis, leukocytosis
Increased intake ofK+ • Iatrogenic, blood transfusions
• Acidosis, rhabdomyolysis, tumor lysis syndrome,
Transcellular shifts of K+ exercise, beta-adrenergic blockers, insulin deficiency,
hyperkalemic periodic paralysis
• Renal failure, Addison Disease, congenital adrenal
hyperplasia, Hyporeninemic Hypoaldosteronism, Renal
Decreased excretion of K+
tubular disease, medications (ACEi,ARBs, potassium-
sparing diuretics, NSAIDs,heparin, drospirenone)

Source:KliegmanR.el al. Nelsontextbookof pediatrics(21sted.).Philadelphia;

2020

II. MANIFESTATIONS
• Most importantly affects membrane potential and the cardiac conduction system
• Some patients manifest with paresthesia, fasciculation, weakness and ascending paralysis
However, cardiac toxicity usually precedes these above manifestations

Et· tdPt L d ti . , d. ECG Ch

ESTIMATED K+ LEVEL ECG CHANGES
(mEq/L)

Tall peaked
Twave

7.5 Peaked T waves

_jJ{_
uL
Tall peaked
Twavc

8.0
Loss of P waves, =·"'
P wave

widening of QRS
~
\

Widened Tan peaked

QRS Twave

9.0

10
ST-segment
depression, further
widening of QRS

JvC
Bradycardia, sine wave QRS-T, first degree AV block,
ventricular arrhythmia
Source:EngornB, et al. TheHamelLaneHandbcok(20thed.).Ph1ladelph1a:
Elsevier;2015

25
III. APPROACH TO DIAGNOSIS
• Cause is olten evident on good clinical history
• Laboratory tests:
Serum K+ level (request for plasma levels if with significant leukocytosis or thrombocytosis)
BUN, creatinine
0Blood gases
ECG to assess the urgency of the situation

Hyperkalemia

True Hyperkalemia : Pseudohyperkalemia (e.g., spurious laboratory values)

Transcellular shift
Measure eGFR
---------------------------------------

eGFR< lSmL/min/1.73 m' eGFR> lSmL/min/1. 73 m 2

,----------------------
Acute kidney Injury Check aldosterone & renin levels
,___ Chronic kidney disease ___ ,

Normal/high renin Normal/high renin Low renin

Normal/high aldosterone Low aldosterone Low aldosterone

----------------------- -- -- -- - - - - ---- - ----------- -

: K· --sparingdiuretics : Primary hypoaldosteronlsm ' Diabetic ncphropathy 1

: Pseudohypoaldosteronlsm , Congenital adrenal hyperplasia Interstitial nephritis

: Type IV renal tubular acidosis : Addison disease , Obstructive uropathy ,
Obstn.ictivc uropathy ACE-inhibitor/ ARB use : Drugs {e.g., cyclosporin, tauolimus, :
:____~?~~D_s: ~~~)- ____;
~:t'~~c_k~~s:
Source:PhadkeK. et al. Manualof PediatricNephrology.
London:Springer:2014

IV. MANAGEMENT
A. Management based on Potassium Level

POTASSIUM LEVEL MANAGEMENT

• Limit potassium intake
IfK• < 6 mEq/L • Optimize effective volume status (e.g., make sure patient is
well hydrated)
• May be treated more gradually with long term therapy and
removal of potassium from the body
• Eliminate potassium from diet and parenteral fluids
IfK• = 6-7 mEq/L and • Sodium polystyrene resin (kayexalate) lg/kg up to 40 g
with normal ECG every 4-6 hours given per orem (PO), per rectum (PR), per
gastric (PG) or as retention enema over 4-6 hours:
0 Exchange resin that substitutes sodium in exchange for K+
0 The K+ containing resin is then excreted from the body
• Abnormal ECG warrants rapid correction and antagonizing
IfK• >7 mEq/L or
the effect of potassium on membrane potentials
with abnormal ECG
• See medications on the next page

26
B. Options in Management
RATIONALE
1. Calcium Glucanate
HOW TO ADMINISTER MAXIMUM DOSE

• Most important step is to
reverse membrane effects of
hyperkalemia (Ca2 • stabilizes
the membrane of cardiac
cells to prevent arrhythmia)
• Does not lower serum K' levels
2. Sodium Bicarbonate (NaHCO,)
• Causes intracellular shift
ofK+
• Most efficacious in patients
with metabolic acidosis
• 10% Ca2 • gluconate
100 mg/kg/dose (1 mL/kg/
dose) over 3-5 minutes (may
repeat in 10 mins)
• Administration: dilute 1
mEq/mL NaHC03 with
sterile water or D5 water
(1:1 dilution)
• 1-2 mEq/kg IV given over
5-10 minutes, at a rate no
greater than 10 mEq/min
• Commonpractice is a max
I
dose of 10-20 mL (10%
calcium gluconate)
• Maximum IV rate
• Push: 100 mg/min
• Infusion:120-240mg/kg/hr
with max concentration of
50 mg/mL
• Max dose 50-100 mEq
• Common practice is to infuse
over 30 minutes to 1 hour
• May be repeated every
5-10 minutes as needed to
reverse ECGabnormalities

3. Regular Insulin + Glucose Solution

• Insulin causes intracellular
shift of K+, while the glucose
prevents hypoglycemia
• Onset of action within 30
minutes
• Bolus: 0.1 unit/kg plus
D25W 2 mL/kg over 30
• Max dose 10 units of regular
minutes, Or
insulin
• Infusion:D25W 1-2 mL/kg/hr
witl1 regular insulin 0.1 U/kg/hr

4. Salbutamol Nebu/ization
• Stimulation of beta-1
• 2.5 mg for children <25 kg • May be given every 1-2
receptors causes
• 5 mg for children >25 kg hours
intracellular shift of K·
S. Sodium polystyrene (Kayexelate)
• Potassium binders • 1 g/kg/dose every 4-6 hours mixed with 250 mL of water as
• Exchange resin retention enema

6. Loop Diuretic

• Increases renal excretion of K· • Furosemide 1-2 mg/kg/dose

• Max dose: 80 mg/dose
in patients who are not anuric IV every 6 to 12 hours
7.Dialysis
• If above measures fail or if with concomitant significant renal failure
• Often necessary in severe renal failure and in high rates of endogenous potassium release (e.g.,
tumor lysis syndrome, rhabdomyolysis)

Sample Case: A 1 year-old was admitted due to severe hyperkalemia. Weight 10 kg. K· = 8
mEq/L. How will you manage the patient's hyperkalemia?
• Calcium gluconate 10% 100 mg/kg or 1 mL/kg: 1 gram or 10 mL slow IV push over 10 minutes
• NaHC03 1 mEq/kg: 10 mEq + 10 mL D5W slow IV over 10 minutes
• Regular Insulin 0.1 u/kg + 2 mL/kg D5W: 1 unit+ 20 ml D5W to be given over 30 minutes
• Salbutamol 2.5 mg nebulization every 1-2 hours
• Sodium polystyrene 1 g/kg: 10 g every 4-6 hours mixed with 250 mL of water as retention enema
• Furosemide 1 mg/kg: 10 mg IV every 6 hours
• Dialysis
Depending on clinical situation and physician's judgement, some strategies may be utilized. Remember
that calcium gluconate is the most important management since it stabilizes cardiac cell membranes.
Source:EngornB, et al.TheHamelLaneHandbook Elsevier;2015
(20thed.l.Ph1ladelph1a:
Kliegman :t~~o~~ii~~d~~~~o\~~
R, et al;,_~r\'~~0£. Mfi ti~~~~::
.,intt~§~~ ~8ti
27
HYPOKALEMIA
I. ETIOPATHOGENESIS
• Defined as serum K· level <3.5 mEq/L
• Common etiologies include the following:
MECHANISM MANAGEMENT
Spurious • Increased WBC
Transcellular • Alkalosis, insulin, drugs/toxins, hypokalemic periodic paralysis,
shifts thyrotoxic periodic paralysis, refeeding syndrome
Decreased Intake • Anorexia
Extrarenal Loss • Diarrhea, laxative, kayexalate or clay ingestion
• RTAType 1 and 2, OKA,Gitelman Syndrome, Bartter Syndrome
Renal losses
• Loop/thiazide diuretics, hyperaldosteronism, licorice ingestion, emesis
Source:KllegmanR,et al. Nelsontextbookof ped,atncs(21sted). Ph1ladelph1a:
Elsevier:2020
II. MANIFESTATIONS
• Cardiac and skeletal muscles are vulnerable to hypokalemia
Muscle weakness and cramps, constipation, ileus, urinary retention
• Paralysis may occur at potassium levels <2.5 mEq/L and may cause respiratory compromise
• ECGchanges: flattened T wave, depressed ST segment, U waves, arrhythmias

III. DIAGNOSIS
• Etiology of hypokalemia is usually apparent from clinical history
• If etiology is uncertain, measure urinary K' to distinguish renal from extra-renal losses
If extra-renal loss: kidneys should conserve K· (i.e., low urinary K·)
0 Methods: 24 hour urine collection, spot K·:creatinine ratio, fractional excretion of K·

IV. MANAGEMENT
• There is no single strict formula for correcting potassium derangements
• Patients with impaired renal function should be treated cautiously to avoid hyperkalemia
ROUTE DOSE REMARKS
• Dose: 2-4 mEq/kg/day
• Preparations (varies depending on brand): • Max dose: 120-140 mEq/
Oral
° KC!syrup 5 mEq/5 rnL day in divided doses
° KC!tablets (1 tab= 10 mEq or 8 mEq K·)
• Maximum concentration of
IV • Dose: 2-4 mEq/kg/day K• incorporation:
Correction • Incorporate KCIinto the IV fluid 0 40 mEq/L in peripheral line

0 80 mEq/L in central line

• Dose 0.5-1 mEq/kg/dose, infuse over 1-2 hrs

• Common practice is 0.5-1 mEq/kg/dose
IV "Fast" + D5W to make 100 mL to run for 1 hour • Usually done under
Correction (max dose of 10 mEq) monitoring in the ICU
• Dilute accordingly and compute for
maximum IV K· infusion rate (KIR)

A Oral Correction:
0 Safest and is the preferred method (effective in 90% of cases)
Increase dietary intake ofK'-rich food (e.g. broccoli, tomato, squash, banana)
0

or give potassium preparations (see above)

8. Intravenous "Fast" Correction
0 Severe hypokalemia in symptomatic patients should be treated aggressively
Indicated for patients with ECGchanges and respiratory paralysis
0 Goal is to increase potassium to around >3 mEq/L
Administration guide:
Infusion should not contain bicarbonate
Hypokalemia needs to be corrected prior to correcting metabolic acidosis
Cardiac monitoring and central venous access are recommended
28
C. Correct Hypomagnesemia if present

0
Hypomagnesemia is considered if hypokalemia is persistent or unresponsive to correction
IV MgSO4 25- 50 mg/kg/dose (max: 2 g) over 2 hours x 3-4 doses, q4-6 hours
° Common method is to mix MgSO4 with D5W to make a total volume of 30 mL to be
I
: ,

given over 30 minutes

D. Potassium Infusion Rate (KIR)
The IV infusion of KCIshould not exceed 0.5-1 mEq/kg/hr (common practice is to use
0.2-0.3 mEq/kg/hr). This is computed via the potassium infusion rate (KIR} formula
(take note of fluids that already have K' in them (e.g., DSIMB contains 20 m£q/l of K'). This
should be added to the equation):

KIR = mEqs KC!x IVF rate

Weight x Total volume of solution

Sample Case 1: A 2 year-old female was rushed to the ER due to LBM of more than 10
episodes. Weight is 10 kg. On work-up, K' = 2 mEq/L and there are ECG changes. How will
you correct the hypokalemia?
IV "fast" correction
• Dose: 1 mEq/kg x 10 kg= 10 mEq
• Order: Give 10 mEq KCIplus D5W to make 100 mL to run for 1 hour

Check KIR = 10 mEq KCLx 100 mL/hr

10 kg x 100 mL

= 1 mEq/kg/hr
*KIR is the maximum recommended (0.5-1 mEq/kg/hr)

Reminder!
• IV "fast" correction should be done under close monitoring, ideally in an ICU setting
• Take note of the maximum KIR and concentration of potassium incorporated depending
on the site of infusion

Sample Case 2: A 1 year-old male was brought in due to diarrhea of3 days. Weight 10 kg.
Physical examination shows no signs of dehydration. Work-up shows hypokalemia K' = 3
mEq/L and patient cannot be fed enterally but is otherwise asymptomatic.
Strategy: Incorporate KCI in the maintenance IVF taking note of the maximum
concentration of KCI incorporation and maximum KIR.

Maintenance fluid requirement for 10 kg patient is 1000 mL using Holliday-Segar method.

For example, our choice offluid is DS ½ NS at a rate of 1000 mL/24h = 40 mL/hr

IV incorporation
• Dose: 3 mEq/kg/day = 3 mEq x 10 kg= 30 mEq/day
• Compute for KCIto be incorporated: 30 mEq KCIin 1 liter offluid
• Order: incorporate 30 mEq KCIto 1 liter D5 ½ NS to run for 40 mL/hr
(delivering 3 mEq KCl/kg/day)

Check KIR = 30 mEq x 40 mL/hr = 0.12 mEq/kg/hr

10 kg x 1000 mL

Reminder! Take note of fluids that already have potassium in them (LR, 1MB, NM, IES).
The amount of potassium in these fluids should be considered in the computation of the
amount of KC! incorporation and KIR.
Source:Engorn8, et al.TheHarrietlane Handbook
(20thed.).Ph1ladelph1a:
Elsevier;2015

29
Sample Case 3: A 1 year-old male was brought in due to diarrhea of3 days. Weight 10
kg. Physical examination shows no sign of dehydration. On work up K· = 3 mEq/L. He is
asymptomatic and can tolerate oral feeding. How will you correct patient's hypokalemia?
Oral correction
• Dose: 3 mEq/kg/day x 10 kg= 30 mEq/day
• Frequency: In three divided doses= 10 mEq/dose
• Order: Give KC)syrup 5 mEq/5 mL, 10 mL qB per orem. Re-check K· levels as needed

HYPOCALCEMIA
I. ETIOPATHOGENESlS
A. Review of Physiology
Calcium exists in three forms in plasma:
Calcium bound to protein, predominantly albumin (40%)
Ionized calcium (48%)
Calcium complexed with anions like phosphate, citrate and bicarbonate (12%)
B D f fH
SERUM CALCIUM IONIZED CALCIUM
Newborn • Refer to essentials section

Children • <8.4 mg/dL

• <2.1 mmol/L I• <4.3 mg/dL
• <1.12 mmol/L

C. Etiology of Hypocalcemia
ETIOLOGY EXAMPLE
Vitamin D deficiency • Nutritional, Vitamin D dependent rickets, CKD
• DiGeorge Syndrome
• CHARGE(Coloboma, heart anomaly, choanal atresia, mental
Hypoparathyroidism retardation, genital hypoplasia, ear anomalies) association
• Neck surgery, Thalassemia, Wilson disease, Iodine 131 therapy

Redistribution of • Tumor lysis syndrome, hyperphosphatemia, acute

plasma calcium pancreatitis
Poor calcium intake • Nutritional, dietary calcium chelators, malabsorption
• Septic shock, drugs (e.g., furosemide, steroids, phenytoin,
Others
rifampicin), massive blood transfusion
Source:KliegmanR.et al. NelsonTextbookof Pediatrics(21st ed.).Philadelphia:
Elsevier:2020

II. MANIFESTATIONS
Often asymptomatic
• Most common presentation: tetany and seizures
Acute manifestations: neuromuscular irritability, prolonged QT interval, heart block
• Chronic manifestations: cataracts, basal ganglia calcifications, extrapyramidal symptoms,
enamel hypoplasia, papilledema, rickets
SIGN PROCEDURE RESULT

• Sphygmomanometer • Carpopedal spasm: ~-"""'.'

flex ion of wrist &
inflated above
Trousseau metacarpophalangeal
systolic pressure
sign joints, extension of the
and maintained for 3
interphalangeal joints
minutes
& adduction of finger

• Repeatedly tap
lateral cheek over • Twitching of the
the course of the corner of the mouth
Chvostek
facial nerve (-1 cm on the ipsilateral side
sign
over the zygomatic due to contractions of
process & 2 cm circumoral muscle
anteriorto earlobe)
30
III. DIAGNOSIS
• Serum calcium, phosphate, magnesium
• Serum albumin

Corrected Calcium
Change in serum albumin by 1 g/dL causes proportional change in serum Ca2 • by
I
0.8 mg/dL (0.2 mmol/L)

Corrected Ca2 • (mg/dL) = Serum Ca'' (mg/dL) + [0.8 x (4 -serum albumin (g/dL)))

Conversion calcium 1 mg/dL = 0.25 mmol/L

Conversion albumin 1 g/dL = 10 g/L

Sample Case: A child sought consult. serum Ca 2' was 1.8 mg/dL and albumin 3.5 g/dL.
Compute for the corrected Ca''

Solution:
• 1.8 mg/dL + (0.8 x (4 - 3.5)] = 2.2 mg/dL

IV. MANAGEMENT
A. Symptomatic Hypocalcemia
Calcium gluconate 10% at 100-200 mg/kg/dose (1-2 mL/kg/dose) + equal diluent
given IV at a rate not more than 100 mg/min. Maximum adult dose is 3 g
Common practice is to give calcium gluconate infusion over 1 hour, at a maximum dose
of 1-2 g/dose (equivalent to 10-20 mL of calcium gluconate 10%)
0 Same dose is repeated every 6-8 hours until asymptomatic
, Hook patient to cardiac monitor while ongoing calcium infusion to monitor for arrhythmia

8. Asymptomatic Hypocalcemia
, Oral supplements at 50-100 mg/kg/day of elemental calcium in 4 divided doses
Source:PhadkeK, et al. Manualof PediatricNephrology. London:Springer:2014
Engorn8, et al. TheHarrietLaneHandbook(20thed.).Philadelphia: Elsevier:2015
KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia:
Elsevier:2020

HYPERCALCEMIA
I. ETIOPATHOGENESIS
A. Definition of Hypercalcemia
0 Serum Ca2 '>12 mg/dL (>3 mmol/L)
0 Severe hypercalcemia = serum Ca'' >15 mg/dL (>3.75 mmol/L)
B. Etiology
ETIOLOGY EXAMPLE
• Adenoma, Multiple endocrine neoplasia, secondary and
Hyperparathyroidism
tertiary hyperparathyroidism from CKD
Excess vitamin D • Hypervitaminosis D, Sarcoidosis, Cat-scratch disease, TB
Ca'' release from bone • Thyrotoxicosis, immobilization, malignancy, SLE
• Idiopathic infantile hypercalcemia, Phosphate depletion in
Others
preterm newborns, Addison disease, Cushing disease

31
II. MANIFESTATIONS
EARLY SIGNS OF HYPERCALCEMIA PROLONGED HYPERCALCEMIA

• Nephrocalcinosis, progressively diminished

• Muscular weakness renal function, renal calculi
• Fatigue, headache • Osseous changes may produce pain in the
• Anorexia, nausea back or extremities, gait disturbances,
• Vomiting, abdominal pain, constipation fractures, genu valgum, tumors, decreased
• Polydipsia, polyuria height from vertebral compression
• Weight loss • Cognitive impairment, convulsions, blindness,
• Fever psychiatric manifestations (depression,
confusion, psychosis), stupor, coma
Mnemonic for symptoms of hypercalcemia:
"Bones,Stones, Groans, Thrones,Psychic Overtones"
• Painfulbones: bone-relatedcomplications
• Renalstones
• Abdominalgroans:constipation,nausea, vomiting
• Thrones:polyuria
• Psychicovertones:fatigue,depression

Ill. DIAGNOSIS(depends on etiology)

• Serum calcium, ionized calcium, serum phosphate
• Serum PTH
• Plasma 2Shydroxy vitamin 03
• Urine Ca/Crea ratio
• Serum creatinine
• Thyroid function
• Ultrasound of neck for parathyroid

IV. MANAGEMENT
A.Goals:
0Decrease intestinal calcium absorption
0Increase urinary calcium excretion
Decrease bone resorption
0Remove excess calcium through dialysis
B. Specific Management
TREATMENT EXAMPLE
• 3000 mL/m 2 /day of isotonic saline
Hydration
• Renal function must be normal
Loop diuretics • Furosemide 1 mg/kg/dose every 6 hours
• 1 mg/kg/dose every 6 hours
Hydrocortisone • F'or vitamin D intoxication, granulomatous disease,
paraneoplastic syndrome
• Pamidronate
Bisphosphonates • Mild hypercalcemia: 0.5-1 mg/kg/dose IV
• Severe hypercalcemia: 1.5-2 mg/kg/dose
• 4 IU/kg every 12-24 hours IM or SC
Calcitonin
• May increase up to 8 JU/kg every 6-12 hours
Dialysis • Done if with renal failure or if above measures fail
Surgical subtotal • For primary or tertiary hyperparathyroidism
parathyroidectomy

32
 SECTION FOUR
COMMON DRIPS, BLOOD TRANSFUSION,
GLUCOSE INFUSION
I
INTRAVENOUS DRIPS

There are many methods in compt1ting for the rate and concentration ofintravenot1s drips.
Familiarizeyot1rselfwith the method that is easiest for yot1 to remember.

I. METHOD 1:

STEP 1. Determine the concentration of the drug based on the preparation of the drug and
amount of diluent used.

Concentration _ Volumeof drug (mL)x Preparation of drug (mg/mL) x 1000 (if desired dose is in mcg)
(mcg/m L) - Total volume of preparation (mL)

STEP 2. Determine the drip rate

Rate weight (kg) x desired dose (mcg/kg/min) x 60 (min/hour)

(mL/hour) Concentration (mcg/mL)

Sample Case: For a 10 kg patient, administer dopamine via drip at a dose of 5 mcg/kg/
min. The available dopamine ampule has a preparation of 200mg/5mL, 5 mL per ampule.
A user defined desired total volume of 50 mL (5 mL of dopamine+ 45mL D5W diluent).
Desired total volume is set by user. 50 mL is a commonly used volume since most syringe
pumps for inotropes have a maximum of 50 mL volume.

1. Concentration= [5 mL x (200 mg/5mL) x 1000 mcg/mg) / 50mL = 4000 mcg/mL

2. Rate mL/hour = [10kg x 5mcg/kg/min x 60min/hour)/ 4000 mcg/mL = 0.75 mL/hour

Order: Start dopamine drip as follows: Dopamine (200mg/5mL) 5mL + 45mL D5water to
run at 0.75mL/hour (delivering 5 mcg/kg/min)

II. METHOD 2
Determine the total volume of the preparation based on the preparation of the drug and
desired drip rate

Rate weight(kg) xdesired dose (mcg/kg/min) x 60 (min/hour) x total volume (mL)

(mL/hour)
Amount of drug per 1 ampule or 1 vial (mcg)

33
 Sample Case: A 10 kg patient, compute how to give dopamine drip at a dose of 5 mcg/kg/
min. The available dopamine ampule has a preparation of200mg/5mL, 5 mL per ampule.
Your desired drip rate is 1 mL/hour
1 mL/hour = 10kg x 5 mcg/kg/min x 60min/hour x total volume
200,000 mcg
(this is 200 mg converted to mcg)

Total volume= 67 mL

Order: Start dopamine drip as follows: Dopamine 200mg/5mL ampule, 1 ampule+ enough
D5W to make a total volume of 67 mL, to run at 1 mL hour (delivering 5 mcg/kg/min)

III. METHOD 3:

Quick/Easy Preparation Commonly used in Practice

EASY PREP
CONCENTRATION USUAL DOSE
DRUG (amount of drug + (in mcg/ml)
diluent)
5 mL of dopamine
Dopamine
+ 45 mL of diluent= 4000
200mg/5mL
50 mL solution • 5-20 mcg/kg/min
Dopamine pre-mixed
No need for diluent 800
200mg/250ml

20 mL of • 2.5-15 mcg/kg/min
Dobutamine
dobutamine 5000
250mg/20mL • Max:40 mcg/kg/min
+ 30 mL of diluent

Epinephrine 5 mL of epinephrine
250 • 0.1-1 mcg/kg/min
lmg/mL + 15 mL of diluent
3 mL ofmidazolam
300
Midazolam + 47 mL of diluent
• 1-2 mcg/kg/min
Smg/mL 6 mL of midazolam
600
+ 44 mL of diluent

4 mL of • Start at 0.05 - 0.1

Norepinephrine
norepinephrine 160 mcg/kg/min
4mg/4mL
+ 21 mL of diluent • Max: 2 mcg/kg/min
Milrinone 10 mL of milrinone
200 • 0.25-0.75mcg/kg/min
10mg/10mL + 40 mL of diluent
• Start at
Nicardipine
10mg/10mL 10 mL ofnicardipine 0.5-1 mcg/kg/min
500
+ 10 mL of diluent (titrate by 0.25)
• Max:4-5 mcg/kg/min

Guide on how to use this quick/easy preparation.

• Use the concentration provided in the table and go directly to calculation of drip rate
Sample Case: Compute for dobutamine drip at 5mcg/kg/min for a 10kg child.
Rate (mL/hour) = weight (kg) x desired dose (mcg/kg/min) x 60 (min/hour)
Concentration (mcg/mL)
Rate= (10 x 5 x 60)/ 5000 (<--from quick prep table above)= 0.6 mL/hour
Sample order: Dobutamine (250mg/20mL) 20 mL + 30 mL DSW to run at 0.6 mL/hour

34
BLOOD TRANSFUSION
I. PACKED RED BLOOD CELLS (pRBC)
• 1 unit= approximately 250 mL
• Transfuse properly typed and cross-matched blood product whenever possible
• Blood type 0- may be used if transfusion is emergent
I
.,

A. Indications
CHILDREN > 4 MONTHS OLD
1. Hb < 7.0 g/dL and patient is in the
perioperative period, with symptomatic
chronic anemia, or with marrow failure
2. Hb < 12.0 g/dL and patient has severe
cardiopulmonary disease or is on
extracorporeal membrane oxygenation
(ECMO)
3. Acute loss of >25% of circulating volume
INFANTS S4 MONTHS OLD
l. Hb < 7.0 g/dL and patient has
symptomatic anemia or is in the
postoperative period
2. Hb < 10.0 g/dL and patient has moderate
pulmonary disease, preoperative period, or
undergoing major surgery
3. Hb < 12.0 g/dL and patient has severe
cardiopulmonary disease or is on ECMO

B.Volume: There are 3 common methods to com ute the needed volume for transfusion
Method 1 (Empiric)
Volume of PRBC= 10-15 ml/kg
'This increases Hb by about 2-3 g/dL
Method 2 (Harriet Lane)
Volume of PRBC (mL) = EBVx (desired Hct- actual Hct) x Hctof PRBC

Where
• Desired Hct is usually 35%-45%
• Hct of PRBC is 55%-70%
• EBV is estimated blood volume

AGE TOTAL BLOOD VOLUME (ml/kg)

• Preterm • 90-105
• Term • 78-86
• 1-12 months • 73-78
• 1-3 years • 74-82
• 4-6 years • 80-86
• 7-18 years • 83-90
• Adults • 68-88

In clinical practice, 80 mL/kg EBV is used for children.

Method 3 (Pediatric Transfusion Medicine)
Volume of PRBC (mL) = (desired Hct - actual Hct) x weight (kg)
*where Hct values are expressed in whole number (ex. Hct 25%, use 25)

C.Administration
Transfuse at a rate no faster than 2-3 mL/kg/hour (generally equivalent to 10-15 mL/kg
over 4 hours)
° For severe compensated anemia, transfuse slowly to avoid congestion. The general rule
is to transfuse a maximum amount in mL/kg equivalent to patients Hb level in g/dL
(e.g., if patient's hemoglobin is 5.0 g/dL, transfuse 5 mL/kg PRBC over 4 hours)

35
 Sample Case: Compute for PRBC transfusion for a symptomatic 1 year old child diagnosed with
acute leukemia, weighing 10 kg with Hct 25% and Hb of 6.8 g/dL

Compute for desired volume of PRBC using the any of the formulas mentioned above.

Method 1 10 mL/kg PRBC = 10 x 10 = 100 mL PRBC

MethodZ Volume of PRBC (ml)= EBV x (desired Hct - actual Hct) x Hct of PRBC
EBV = 80 m L/kg x 10kg = 800111L
Desired HCT = 35%
Hct of PRBC = 70%
Volume of PRBC = 800 mL x (0.35 - 0.25) x 0.7 = 56 mL PRBC
Method3 Volume of PRBC = (desired Hct - actual Hct) x weight in kg
= (35-25) X 10
= 100 mL PRBC

Reminder
• Take note of the maximum amount to be transfused for this patient (based on the Hb level:
approximately 6.8 mL/kg). Since our target volume to transfuse is around 100 mL based
on method #s 1 and 3:
0 Order transfusion as follows: Transfuse PRBC properly typed and cross-matched, 2
aliquots at SO mL/aliquot each to run for 4 hours, given 6 hours apart
0 After transfusion of 2 aliquots, we have given a total volume of 100ml PRBC which is our
computed target for this patient
• Common clinical practice is to transfuse the amount of PRBC for 2-4 hours, 6 hours apart
(this is acceptable as long as we take note of the maximum infusion rate of 2-3 mL/kg/hour)
• CBC is then repeated after 6 hours to check for increase in Hb and HCT

II. PLATELET CONCENTRATE

• 1 unit= approximately SO mL
• Transfuse type-specific blood product
• 1 unit/m2 raises platelet count by 10,000 to 15,000/uL
• 10 mL/kg usually raises platelet count by 50,000/uL

A. Indications

CHILDREN > 4 MONTHS OLD INFANTS S4 MONTHS OLD

l. Platelet <50,000/uL and patient has l. Platelet <100,000/uL and patient has
bleeding or will undergo major invasive bleeding or on ECMO
procedure (<25,000/uL if minor procedure) 2. Platelet <50,000/uL and patient is
2. Platelet <20,000/uL and the patient has clinically unstable or will undergo
marrow failure with hemorrhagic risk invasive procedure
factors
3. Platelet <20,000/uL and the patient is
3. Platelet <10,000/uL and the patient has clinically stable
marrow failure without hemorrhagic risk
4. To maintain platelet count at any level
factors
if patient has platelet dysfunction PLUS
4. To maintain platelet count at any level bleeding or invasive procedure
if patient has platelet dysfunction PLUS
bleeding or invasive procedure

36
 B. Dose (common clinical practice):
0 1 unit per 10 kg or 4 units per m2 (body surface area)
10 mL/kg (neonate)
° Common clinical practice for patients beyond neonatal period
mL of platelet concentrate= weight (in kg) x 7
• usually rounded based on unit volume of platelet concentrate
I
.

C. Administration
0 Given as fast drip because it adheres to tubings
0 Exception: given for 1 hour in neonates
Sample Case: A 4 year-old patient diagnosed with ALL was found to have a platelet count of
10 on routine CBC.Weight is 20 kg patient. Compute for platelet transfusion.
Amount to be transfused= lunit/lOkg = 2 units
OR mL of platelet concentrate= 20 x 7 = 140 m L = 2 or 3 units can be given

Order:
• Prepare type-specific platelet concentrate 50ml/unit
• Transfuse 2 units as fast drip one after the other
• Repeat CBC 6 hours post-transfusion

III. FRESH FROZENPLASMA (FFP)

• 1 unit= approximately 250 mL

A. Indications
Severe clotting factor deficiencies with active bleeding or with invasive procedure
0 Reversal of effects ofwarfarin
0 Disseminated intravascular coagulopathy (DIC)
, Liver disease
0 Dilutional coagulopathy and bleeding (seen in massive transfusion)

B. Dose
0 10-15 mL/kg infused as rapidly as tolerated by patient
Common clinical practice is to infuse FFP over 2 hours (sometimes over 4 hours if
patient is prone to volume overload)

IV. CRYOSUPERNATANT
• Contains unmeasurable amount of Factor VIII and Fibrinogen
• Standard dosing: 10-15 mL/kg
The volume transfused will depend on the clinical situation and patient size
• Indications: Thrombotic Thrombocytopenic Purpura (TTP) or Hemolytic Uremic
Syndrome (HUS)

V. CRYOPRECIPITATE
• Sometimes called Cryoprecipitated Antihemophilic Factor
• Each unit (around 10-15 rnL) provides Fibrinogen, Factor VIII, von Willebrand Factor, and
Factor XIII
• Each unit contain minimum of 150 mg Fibrinogen and 80 IU of Factor VIII
• Indications:
Hemophilia as emergency back up when factor concentrates are not available
0 Von Willebrand Disease as a reserve treatment
0 Hypofibrinogenernia secondary to massive transfusion
Afribrinogenemia
DIC and Uremic Bleeding Tendency
Sources:EngornB,et al. TheHarrietLaneHandbook (20thed.).Philadelphia:
Elsevier;2015
OrkinS, et al. NathanandOski'shematology of infancyandchildhood(7thed.).SaundersElsevier:2009
HillyerC, et al. Handbook of PediatricTransfusionMedicine(1sted.).SanDiego:Elsevier:2004
HillyerC. Bloodbankingandtransfusion medicine.Philadelphia,PA:ChurchillLivingstone/Elsevier:
2007
Ktiegman R, et al. Nelsontextbookor pediatrics(21sted.).Philadelphia:
Elsevier:2020
37
GLUCOSE INFUSION
I. FOR SYMPTOMATIC HYPOGLYCEMIA
• Give D10 water (Dl0W) 2 mL/kg for neonates OR 5 mL/kg for older infants and children
• Re-check glucose levels after 30 minutes
• May give another bolus of Dl0W and adjust glucose infusion accordingly

II. GLUCOSE INFUSION RATE (GIR)

• Usual GIR requirement for infants and children= 4-6 mg/kg/min
• Dextrose concentration is expressed in whole number
DSW = 5 g of glucose/dL, so dextrose concentration is 5
0DlOW = 10 g of glucose/dL, so dextrose concentration is 10

General Formula:
IV rate (mL/hr) x dextrose concentration (g/dL) x 1000 (mg/g)
Weight (kg) x 60 (min/hr) x 100 (mL/dL)

GIR (mg glucose/kg/min)

Formula simplified as:
IV rate x dextrose concentration x 0.167
Weight

Sample Case. A 10 kg child was placed on NPO due to vomiting. IV fluids started is DSLR
at maintenance rate using Holliday-Segar formula. Calculate for the G!R
= 1000 mL/day
!VF rate
= 42 mL/hour
= IV rate x dextrose concentration x 0.167
Weight

GIR = 42 X 5 X 0.167
10

= 3.5 mg/kg/min

38
FORMULAS FOR ADJUSTING DEXTROSITY/DEXTROSE CONCENTRATION
• Computations for increasing and decreasing dextrosity are important to be able to
concoct desired dextrose concentration using readily available stock preparations.
• In general, the maximum dextrose concentration for peripheral lines is 12.5% (e.g., D12.5) I
I. INCREASING DEXTROSITY

Sample Case. Concoct a final mixture of 1LD101MB using 051MB and D50 Water (D50W)

Step 1. Determine
stock preparations D5 1MB and DSOW
tobeused
Factor= Desired dextrosity - actual dextrosity
Highest dextrosity- lowest dextrosity

In our example:
Step 2. Compute • Desired dextrosity = 10 (we want to make D10 1MB)
for Factor • Actual dextrosity = 5 (we are making the solution from D5 1MB)
• Highest dextrosity = 50, lowest dextrosity = 5
(we are mixing 051MB and DSOW)
Factor = 10 - 5 = 0.11
--
50 - 5
Step 3. Compute
for amount of Amount of D50W = factor x total volume offinal product (i.e. 1000 mL)
D50Wto be = 0.11 x 1000 = llOmL
added
Step 4. Concoct Prepare D10 1MB as follows: OS 1MB 890 rnL + D50W 110 mL = lL
the fluid D10 1MB

II. DECREASING DEXTROSITY

Sample Case. Decrease dextrosity from a solution of 500 mL 0101MB to D7.5 1MB by
adding sterile water
Step 1.Determine
stock preparations 500ml D5 1MBand sterile water
to be used
Actual dextrosity x (volume of solution)
= Desired dextrosity x (Volume of solution+ Y)

*Y is the amount of sterile water to be added

Step 2. Compute
for the amount
of sterile water Solve for Y:
to be added
10 x 500 mL = 7.5 (500 mL + Y)
using the
equation 5000 = 3750 + 7.5Y
5000-3750 = 7.SY
1250 = 7.5Y
1250/7.5 = Y
167mL=Y
Step 3. Concoct
500 mL D10 1MB+ 167 mL Sterile Water= 667 mL ofD7.5 1MB
the fluid
Sources:ClohertyJ, et al. Manualof NeonatalCare(7thed.).LippincottWilliams& Wilkins:2012
KliegmanR, el al. Nelsontextbookof pediatrics(21sted.).Philadelphia:
Elsevier:2020
FeldL, et al. FluidandElectrolytes
in Pediatrics.NewYork:HumanaPress:2010
GlucoseInfusionRate.(2018).Retrievedfromhttp://www-users.med.cornell.edul-spon/picu/calc/glucinfr.hlm

39
REFERENCES
1. Avnet; E., Harmon, W.,Niaudet, P.,Yoshikawa, N.,Emma, F.,& Goldstein, S. Pediatric Nephrology
7th ed. 2016; London: Springer
2. Cloherty, )., Eichenwald, E., Hansen, A., & Stark, A. Manual of Neonatal Care 7th ed. 2012.
Philadelphia: Lippincott Williams & Wilkins.
3. Crawford,)., Terry, M., & Rourke, G. Simplification of drug dosage calculation by application of
the surface area principle. Pediatrics, 1950, 5(783)
4. Del Mundo, Fe. Textbook of Pediatrics and Child Health. )MC Press Inc; 1947
5. Engorn, B., and Flerlage, J. The Harriet Lane Handbook 20th ed. 2015, Philadelphia:
Elsevier.
6. Feld, L., and Kaskel, F.Fluid and Electrolytes in Pediatrics. 2010, New York: Humana Press.
7. Friedman JN, Goldman RD, Srivastava R, Parkin PC. Development of a clinical dehydration
scale for use in children between 1 and 36 months of age. J Pediat,: 2004;145(2):201-207.
8. Glucose Infusion Rate. Retrieved from
9. http://www-users.med.cornell.edu/~spon/picu/calc/glucinfrhtm. Accessed: November 10, 2018.
10. Gillis HC, et al. Rapid fluid administration: an evaluation of two techniques. Medical
Devices: evidence and Research; 2018
11. Hall, j. Guyton and Hall textbook of medical physiology 13th ed., 2006. Philadelphia: Elsevier·.
12. Hillyer, CD, Strauss Sand Luban, N. Handbook of Pediatric Transfusion Medicine. 2004. San
Diego: Elsevier
13. Hillyet; CD, Silbersfein LE, Ness PM et al., Blood banking and transfusion medicine. 2007.
Philadelphia, PA: Churchill Livingstone/Elsevier.
14. Holliday, M., and Segat; W. The maintenance need for water in parenteral fluid therapy.
Pediatrics, 1957; 19.
15. Kliegman, R., ST GEME Ill,)., Blum, N., Shah, S., Tasker, R., Wilson, K., & Behrman, R. Nelson
Textbook of Pediatrics 21st ed. 2020. Philadelphia: Elsevier.
16. Koeppen, B. M., Stanton, B. A. Berne & Levy Physiology. 2010, Philadelphia, PA: Mosby/
Elsevier.
17. Ludan AC. Pediatric fluid and electrolyte therapy. In Del Mundo, et al. Textbook of Pediatrics
and Child Health, 1st Ed, 1974. Quezon City: )MC Press Inc
18. Malbrain, M., Maria, P., Witters, I., Cordemans, C., Kirkpatrick, A., Roberts, D., & Van
Regenmortel, N. Fluid overload, de-resuscitation, and outcomes in critically ill or injured
patients: a systematic review with suggestions for clinical practice. Anaesthesiology
Intensive Therapy, 2014, 46(5), 361-380.
19. National Institute for Health and Clinical Excellence. Diarrhoea and vomiting caused by
gastroenteritis diagnosis, assessment and management in children younger than 5 years.
2019 London: RCOGPress.
20. National Institute for Health and Care Excellence. Intravenous fluid therapy in children
and young people. Available from: https://www.nice.org.uk/guidance/ng29. Published
2015. Accessed: November 15, 2018.
21. Phadke, K.,Goodye1; P.,& Bitzan, M. Manual of Pediatric Nephrology. 2014, London: Springe1:
22. Philippine Pediatric Society, Inc. Consensus statements on parenteral fluid therapy in
infants, children and adolescents, 2017; Philippine Pediatric Society, Inc.; Philippines
23. Orkin, SH and Nathan NG. Nathan and Oski's hematology of infancy and childhood (7th ed.)
2009. Saunders Elsevier
24. World Health Organization. The treatment of diarrhoea. Geneva: World Health Organization; 2005
Available from: https:/ /apps.who.int/iris/bitstream/handle/10665/ 43209 /9241593180.
pdf;jsessionid= 6 BF5 24 B8A D49C4Cl E439 D18C5 E3 D7717?seq uence= 1

40
COMMONMEDICATIONS
INPEDIATRICS
 SECTION ONE
INTRODUCTION TO PRESCRIPTION WRITING

OVERVIEW OF PRESCRIPTION WRITING

COMMON HOUSEHOLD MEASURES AND EQUIVALENTS
HOUSEHOLD MEASURES APPROPRIATE EQUIVALENT
I
METRIC APOTHECARY

1 drop () 1/20 mL
(or 0.05 mL)
1 minim

1 teaspoonful
~ 5 mL 1 dram

1 tablespoonful
1 15 mL 1/2 fluid ounce

1 wine glassful
2 60 mL 2 fluid ounces

□
1 glassful 250 mL 8 fluid ounces

COMMONABBREVIATIONS USED LEGENDSFOR THIS CHAPTER

ABBREVIATION DERIVATION MEANING LI Units
a.c. Ante Cibum Before meals mcg Microgram
b.i.d Bis in Die Two times a day g Gram
Gm.or gm. Gramme Gram PO Per orem
gr. Grain Grain PR Per rectum

gtt Gutta Drop IV Intravenous

h.s. Hora Somni Hour to sleep IM Intramuscular

(at bedtime)
SC Subcutaneous
o.d. Omni Die Everyday
SL Sublingual
p.c. Post Cibum After meals
syr Syrup
p.r.n. Pro Re Nata If necessary
MDI Metered dose inhaler
q.h. Quaque Hora Every hour
DPI Dry powder inhaler
q.i.d. Quarter in Die Four times a day
LD Loading dose
q.s. Quantum Sufficit As much as required
MD Maintenance dose
stat. Statim Immediately
Max Maximum dose
t.i.d Ter in Die Three times a day
ER Extended release
Neonatesmay have differentregimens,dependingon the day of life Supp Suppository
43
PARTS OF A PRESCRIPTION
Description of the Parts
PART DESCRIPTION
Superscription • Should include the name of the patient, address, age, and date
prescription was written
• Has the symbol "Rx"
Inscription (body) • Consists of the name of the drug, dosage form and the amount
per dose/strength of the preparation
Subscription • Contains the total quantity to be dispensed based on the
frequency of administration and the duration of treatment
Signa/Iabel • Direction to the patient/caregiver
Prescriber's data • Name & signature of physician, license numbe1; etc.

Example Prescription
PART DESCRIPTION
Superscription Patient X; 1/M March 5, 2020
Alabang, Muntinlupa
Rx
Inscription (body) Paracetamol Syrup 250 mg/5mL
Subscription Dispense 1 Bottle (60 mL)
Sign a/label Sig: Take 5 ml every 6 hours for 3 days
Prescriber's data Juan Aherrera MD
License No. 123456
PTR No. 345678

PRESCRIBING MEDICATIONS IN PEDIATRICS

I. LIQUID,ORAL PRESCRIPTION
• Inscription consists of the name of the drug, dosage form, and the strength or
concentration of preparation
• Subscription consists of the total volume to be dispensed

Example for drugs computed as milligram per kilogram per dose (mkdose):
Case: You want to prescribe a child (with a weight o/25 kg) paracetamol syrup ata dose of 10
mkdose for 3 days. The available paracetamol preparation is 250mg/5ml syrup.
Compute for the dose Dose = (Desired Dosage x Weight)/ Preparation
= (10 mkdose x 25 kg)/ (250 mg/ 5 mL)
= 5 mL per dose
Compute for the dispense Dispense Number= 5 mL X 4 doses per day x 3 days
number
=60mL
Dispense 1 bottle (60 mL)
Sample Signatura Take 5 mL every 6 hours for 3 days

44
Example for drugs computed as milligram per kilogram per day (mkday):
Case: You want to prescribe a child (with a weight of 25 kg) amoxici/lin syrup at a dose of 30
mkday qB hours for 7 days. The preparation of amoxicillin is 250mg/5ml syrup
Compute for the dose Dose = (Desired dose x weight)/ Preparation
= (30 mkday x 25 kg)/ (250 mg/ SmL)
= 15 mL per day
I
Convert to "per dose": 15 mLperday / 3 doses per day= 5 mL per dose
Compute for the dispense Dispense Number= 5 mL X 3 doses per day x 7 days
number
= 105 mL
Dispense 1 bottle (120 mL)
Sample Signatura Take 5 mL every 8 hours for 7 days

II. SOLID, ORAL PRESCRIPTION

• Inscription consists of the name of the drug, dosage strength, and dosage form
• Subscription consists of the total number of tablets/capsules to be dispensed

Example for drugs computed as milligram per kilogram per dose (mkdose)
Case: You want to prescribe a child (with a weightof50 kg) paracetamol tablet at a dose oflO
mkdose for 3 days. The available paracetamol preparation is 500 mg/tablet
Compute for the dose Dose = (Desired Dosage x Weight)/ Preparation
= (10 mkdose x 50 kg)/ 500 mg/tab
= 1 tablet per dose
Compute for the dispense Dispense Number= 1 tablet X 4 doses per day x 3 days
number
= 12 tablets
Sample Signatura Take 1 tablet every 6 hours for 3 days

Example for drugs computed as milligram per kilogram per day (mkday)
Case: You want to prescribe a child (with a weight of 50 kg) amoxicillin ata dose of30 mkday
qBfor 7 days. The preparation of amoxicil/in is 500 mg/capsule
Compute for the dose Dose = (Desired dose x weight)/ Preparation
= (30 mkday x 50 kg)/ 500 mg/cap
= 3 capsules per day

Convert to per dose: 3 capsules per day or 3 doses per day

(1 capsule per dose)
Compute for the dispense Dispense Number= 1 capsule X 3 doses per day x 7 days
number = 21 tablets
Sample Signatura Take 1 capsule every 8 hours for 7 days

45
 SECTION TWO
COMMON DRUGS USED IN PEDIATRICS

ANTIBIOTICS
MAXIMUM DOSE/
DRUG INDICATION/DOSE PREPARATIONS
REMARKS
• Max: initial 1.5 g/
day, then monitor
• 100 mg/amp
• 15-22.5 mkday IV/IM levels
• 200 mg/amp
Amikacin q8, or • WOF: ototoxicity,
• 250 mg/amp
• 15-20 mkday IV/IM OD nephrotoxicity,
• 500 mg/amp
neuromuscular
blockade, rash
• Standard dose: 25-50 • 250 mg cap
mkday PO q8/q12 • 500 mg cap • Max: 2-3 g/day
Amoxicillin • High dose {resistant S. • 100 mg/mL drops • WOF: rash,
pneumoniae): 80-90 • 125 mg/5mL syr diarrhea
mkday PO q12 • 250 mg/5mL syr
• 250/125 mg tab
• Q8 dosing: 20-40 • Max: 2 g PO q12
• 500/125 mg tab
mkday PO • 125/31.25mg/5mL syr • WOF: rash,
Amoxicillin- • 250/62.5 mg/5mL syr diarrhea
Clavulanic • Dosage based
Acid • 875/125 mg tab on amoxicillin
• Q12 dosing: 25-45 • 200/28.5 mg/SmL syr component
mkdayPO • 400/57 mg/5mL syr
• 600/42.9 mg/5mL syr
• Max: 12 g/day
• Mild-moderate infection • Usual meningitic
• 250 mg vial
100-200 mkday IV/IM q6 dose is 200mkday
• 500 mg vial
Ampicillin • Severe • WOF: rash, diarrhea,
• 1 gvial
200-400 mkday IV/IM interstitial nephritis,
q4/q6 pseudo membranous
enterocolitis

Infants ;:el month

• Mild-moderate infection
100-150 mkday IV/IM q6
• Meningitis/severe infection
200-300 mkday IV/IM q6
Ampicillin-
Sulbactam Child
• Mild-moderate infection
100-200 mkday IV/IM q6
• Meningitis/severe infection
200-400 mkday IV/IM
q4/q6
• Max:
8 g ampicillin/day
• WOF: rash, diarrhea,
• 250/125 mg vial
interstitial nephritis,
• 500/250 mg vial
pseudomembranous
• 1/0.5 g vial enterocolitis
• Dosage based on
ampicillin component

46
 , Maxfor 5 day regimen:
, 5 day regimen ( otitis
500 mg/day day 1,
media, PCAP,pertussis}
250 mg/day days 2-5
10 mkday PO OD day 1,
, Maxfor 3 day regimen:

Azithromycin
5 mkday PO OD days 2-5
, 3 day regimen (otitis
media, acute sinusitis)
10 mkday PO OD
• 1 day regimen {otitis
• 250 mg tab
• 500 mg tab
• 200 mg/5mL syr
500mg/day
• Maxfor 1 day regimen:
1500 mg/day
• Max for pharyngitis/
I
tonsillitis:500 mg/day
media): 30 mkday PO OD • WOF: transaminitis,
, Pharyngitis/tonsillitis:
cholestatic jaundice,
12 mkday PO ODx 5 days GI discomfort
• 50-100 mkday IV/IM , Max dose: 6 g/day,
• 250 mg vial
q6/q8 prophylaxis 1 g/dose
Cefazolin • 500 mg vial
, IEprophylaxis , WOF: leukopenia,
(1st gen) • 1 g vial
SOmkdose IV/IM 30 mins thrombocytopenia,
before procedure transaminitis
, 25-100 mkday PO q6
• 250 mg cap
(q8/q12 for , Max dose: 4 g/day;
• 500 mg cap
Cephalexin uncomplicated infections) prophylaxis 2 g/dose
• 100 mg/mL drops
(1" gen) • IE prophylaxis • WOF: GI discomfort
• 125 mg/5mL syr
SOmkdose PO 1 hour
• 250 mg/5mL syr
before procedure
• 250 mg cap • Max dose: 2 g/day
, 375 mg ER tab , WOF: increased
Cefaclor • 500 mg cap hepatic enzymes,
, 20-40 mkday PO q8
(2 nd gen) • 50 mg/mL drops bone marrow
, 125 mg/5mL syr suppression,
• 250 mg/5mL syr moniliasis
• 250 mg vial • Max dose: 9 g/day
• IV/IM:
• 750 mg vial • WOF: GI discomfort,
75-150 mkday qB
• 1.5 g vial thrombophlebitis
Cefuroxime
(2 nd gen) • 250 mg tab • Max dose: 1 g/day
• PO: • 500 mg tab
• WOF: GI discomfort
20-30 mkday q12 • 125 mg/SmL syr
• 250 mg/5mL syr

• Usual dose • Max dose: 12 g/day

• 250 mg vial
100-200 mkday IV/IM • WOF: allergy,
Cefotaxime • 500 mg vial
q6/q8 neutropenia,
(3'" gen) • 1 g vial
• Meningitic dose thrombocytopenia,
200 mkday IV/IM q6 eosinophilia

• Usual dose • 250 mg vial

• Max dose: 6 g/day
Ceftazidime 90-150 mkday IV/IM q8 • 500 mg vial
• WOF: rash,
(3'" gen) • Meningitic dose • 1 g vial
transaminitis
150 mkday IV/IM q8 • 2 g vial

• Usual dose • Max dose:

50-75 mkday IV/IM usual dose 2 g/day,
• 250 mg vial meningiticdose4g/day
q12/q24
Ceftriaxone • 500 mg vial , WOF:rash, pain over
, Meningitic dose/
(3'" gen) • 1 g vial injection site, diarrhea,
penicillin-resistant
transaminitis,
pneumococci jaundice cholelithiasis,
100 mkday IV/IM q12 gallbladder sludging

47

• Usual dose
100 mkday IV/IM q12
Cefepime
• Meningitis, neutropenia,
(4 th gen)
serious infection
150 mkday IV/IM q8
• Usual dose
50-75 mkday IVor PO q6
Chloramphenicol
• Meningitic dose
75-100 mkday IV q6
Clarithromycin • 15 mkday PO q12
• IV/IM:
25-40 mkday q6/q8
Clindamycin
• PO: 10-30 mkday q6/q8
• Bacterial endocarditis
prophylaxis:
20 mkdose PO 1 hour
before procedure
• Mild-moderate infection:
12.5-50 mkday PO q6
Cloxacillin
• Severe
50-100 mkday PO q6
• 30-50 mkday PO q6/q8
• RF prophylaxis
Erythromycin 500 mg/day PO q12
• Pertussis
SOmkday POq6 x 14 days
Gentamicin • 7.5 mkday IV/IM q8
48
• Max dose:
• 500 mg vial usual dose 4 g/day,
• 1 g vial 6 g/day
meningiticdose:
• 2 g vial • WOF: GI discomfort,
thrombophlebitis,
transaminitis
• Max dose: 4 g/day
• WOF: marrow
• 250 mg cap suppression, Gray
• 500 mg cap baby syndrome
• 125 mg/5mL syr • Use with caution
• 1 g/vial in G6PD deficiency,
renal or hepatic
dysfunction
• Max dose: 1 g/day
• 125 mg tab
• WOF: diarrhea,
• 250 mg tab
abnormal taste,
• 500 mg tab
abdominal pain,
• 125 mg/5mL syr
QT prolongation,
• 250 mg/5mL syr
arrhythmias
• Max dose: 4.8 g/ day
• WOF:diarrhea, rash,
pseudomembranous
• 150 mg/mL amp colitis, Steven-
Johnson syndrome,
• 150 mg/2 mL amp granulocytopenia,
thrombocytopenia,
sterile abscess at
injection site
• Max dose: 1.8 g/day,
prophylaxis
600 mg/dose
• 75 mg cap
• WOF:diarrhea, rash,
• 150 mg cap
pseudo membranous
• 300 mg cap
colitis, Steven-
• 75 mg/SmL syr
Johnson syndrome,
granulocytopenia,
thrombocytopenia
• 500 mg cap • Max dose: 2 g/day
• 125 mg/SmL syr • WOF: nausea,
• 250 mg/SmL syr vomiting, diarrhea
• 250 mg tab • Max dose: 2 g/day
• 500 mg tab • WOF:nausea,vomiting,
• 100 mg/mL drops abdominal cramps,
• 100 mg/2.5 mL drops CYP450drug
• 125 mg/5 mLsyr interactions,
• 200 mg/5 mL syr hypertrophic
• 250 mg/5 mL syr pyloric stenosis in
• 400 mg/5 mL syr neonates
• WOF: ototoxicity,
• 40 mg/mL vial
nephrotoxicity
 • Max for skin &
subcutaneous
tissue infection:
1.5 g/day

Meropenem
• Skin and subcutaneous
tissue infection
30 mkday IV q8
• Intra-abdominal,
mild-moderate infection
• 500 mg vial
• 1 g vial
• Max for intra-
abdominal, mild-
moderate infection:
3 g/day
I
• Max for meningitis,
60 mkday IV q8
severe infection:
• Meningitis, severe
6 g/day
infection
• WOF: diarrhea,
120 mkday IV q8
rash, nausea, oral
moniliasis,glossitis,
headache, pain at
injection site
• Anaerobic infection
30 mkday IV/PO q6
• Bacterial vaginosis • Max for anaerobic
(adolescent) infection: 4 g/day
500 mg PO q12 x 7 days, • Max for giardiasis
750 mg PO OD x 7 days 750 mg/day
(if extended release tab) • Max for C.difficile:
• Trichomoniasis 500 mg/dose
° Child • Max for H. pylori:
• 250 mg/tab
15 mkday PO q8 x 7 days 500 mg/dose
• 500 mg/tab
0 Adolescent • WOF: nausea,
• 125mg/5 mL
Metronidazole 2 g PO x 1 dose, or 250 diarrhea, urticaria,
suspension
mg PO q8 x 7 days or dry mouth,
• 500 mg/vial
375 mg PO q12 x 7 days leukopenia,
• Ciardiasis vertigo, metallic
15 mkday PO q8 x 5 days taste, peripheral
• C.difficile infection neuropathy,
30 mkday IV/PO q6 disulfiram-type
x 10 days (IV less reaction with
efficacious) alcohol ingestion
• H. pylori infection
15-20 mkday PO q12 x
4 weeks
• Max dose: 12 g/day
• 100-200 mkday IV/IM
Oxacillin • 500 mg vial • WOF: rash, GI
q4/q6
disturbances
• Max dose: 4 g IV q6
• WOF: rash, diarrhea,
• <6 months: headache, fever,
• 2/0.25 g vial
Piperacillin- 150-300 mkday IVq6/q8 thrombophlebitis,
• 4/0.S g vial
Tazobactam • 2:6 months: injection site pain,
300-400 mkday IVq6/q8 • Dosage based
on piperacillin
component

49

• 100,000-400,000
ukday IV/IM q4/q6
Penicillin G -
• Neurosyphilis
aqueous
200,000-300,000ukday IV
q4/q6 x 10-14 days
• Max dose: 24
million u/day
• WOF: anaphylaxis,
• 1 million u vial
urticaria, hemolytic
• 5 million u vial
anemia, interstitial
nephritis, Jarisch-
Herxheimer
reaction (syphilis)

Group A streptococci
• <27 kg:
600,000 u/dose IMx 1
• "?.27kg:
1.2 million u/dose IMxl
• Max dose for
RF prophylaxis syphilis: 2.4 million
• <27 kg: 600,000 u/dose
Penicillin G · u/dose IMq3 weeks • WOF: same as pen
• 1.2 million u vial
benzathine • "?.27kg: 1.2 million G-aqueous
u/dose IMq3 weeks • Do not administer
IV
Syphilis
• Early acquired
50,000 ukdose IM xl
• > 1 year duration
50,000 ukdose IM q7
days x 3 doses
Usual Dose
• Child:
25-50 mkday PO q6/q8
• Adolescent: 125-500
mg/dose PO q6/q8
Group A strep
pharyngitis
• <27 kg: 250 mg PO • 250 mg cap • Max dose: 3 g/day
Penicillin VK • 500 mg cap • WOF: same as pen
q8/q12 x 10 days
• "?.27kg: 500 mg PO G-aqueous
q8/q12 x 10 days
RF prophylaxis:
• 2 months-<3 years:
125 mg PO q12
• "?.3years:
250 mg PO q12

• Max dose: 160 mg/

• Mild-moderate infections
8-12 mkday IV/PO q12
• Severe infections
20 mkday IV/PO q6/q8
• Pneumocystic carinii
Sulfamethoxazole- pneumonia (PCP)
Trimethoprim
treatment
(TMP)
20 mkday IV/PO q6/q8
• PCPprophylaxis
5-10 mkday IV/PO q12
x 3 consecutivedays/week
• UT! prophylaxis
2-4 mkday PO OD
dose q12
• WOF: kernicterus
in newborns, blood
• 400 /80 mg tab
dyscrasia, giossitis,
• 800/160 mg tab
renal or hepatic
• 200/40 mg/5mL
injury, GI irritation,
suspension
rash, SJS,hemolysis
• 400/80 mg/5mL
in G6PD deficiency
suspension
• Contraindication:
megaloblastic
anemia from folate
deficiency
• Dose based on TMP

50
 • Mild-moderate
infection: 2 g/day
• Mild-moderate infection • Severe infection: 4
40-45 mkday IV q6/q8 • 500 mg vial g/day
Vaneomycin
• Severe infection
45-60 mkday IV q6/q8
• 1 g vial • WOF: Red man
syndrome,
ototoxicity,
nephrotoxicity
I
ANTITUBERCULOUS AGENTS
INDICATION/ MAXIMUM DOSE I
DRUG PREPARATION
DOSE REMARKS
• 300 mg tab
• 200 mg H/
10 mg pyridoxine/ • Max dose: 300 mg/day
5 mLsyr • WOF: peripheral neuropathy,
lsoniazid • 10 (10-15J • 200 mg H/ hepatic side effects
(HJ mkday PO OD 12 mg pyridoxine/
5 mL • Supplemental pyridoxine
• 200 mg H/ (1-2mkday) is recommended
20 mg pyridoxine/
5 mL

• 15 (10-20J • Max dose: 600 mg/day

mkdayPO OD • Prophylaxis: 1200 mg/day
• Prophylaxis for • WOF: GI irritation, hepatitis,
Rifampicin
• 200 mg/5mL syr allergy, ataxia, blood dyscrasia,
(RJ N. meningitidis
interstitialnephritis,hyperuricemia
20 mkdayPO • Causes red discoloration of
q12 x 2 days urine, saliva, tears
• Max dose: 2 g/day
Pyrazinamide • 30 (20-40J • 250 mg/5mL syr • WOF: hepatotoxicity, rash,
(ZJ mkday PO OD • 500 mg/5mL syr hyperuricemia, arthralgia,
fever, acne, porphyria
Ethambutol • 20 (15-25J • Max dose: 1.2 g/day
• 400 mg/tab
(E) mkday PO OD • WOF: optic neuritis
• Max dose: 1 g/day
Streptomycin • 30 (20-40J • WOF: ototoxicity,
• 1 g vial
(SJ mkdaylM OD nephrotoxicity, CNS
depression, serum sickness
Common preparations are also available
PREPARATION DOSE PER BODY WEIGHT
75mgH/150mgR • 30-37 kg • 2 tabs/day

75mgH/150mg R/275mgE • 38-54 kg • 3 tabs/day

75mgH/150 mgR/300mg E • 55-70 kg • 4 tabs/day

75 mgH / 150mg R/ 400 mgZ/ 275mg E • >70kg • 5 tabs/day

51
ANTIBIOTICS FOR TOPICAL/OPHTHALMIC/OTIC USE
INDICATION/ MAXIMUM DOSE/
DRUG PREPARATION
DOSE REMARKS

Ciprofloxacin +/ • 4dropsq12x7 • WOF: stinging, redness,

dexamethasone days, or
• Ear drops itching, ear pain, fungal and
• 2 drops q8/q12
(2'6 months) x 7-14 days secondary bacterial infection

• Apply q8/q12 x • WOF: hypersensitivity

Fusidic acid • Ointment, cream
7 days reaction
• WOF: minor local irritation,
Mupirocin • Apply q8 • Ointment, cream
dry skin
Neomycin, • WOF:stinging, photosensitivity,
polymyxin B, • Apply qS-24 • Topical ointment
bacitracin superinfection, delayed healing

Polymyxin, • 3-4 drops • WOF: burning, itchin~,

neomycin, • Ear drops irritation, dryness, fo liculitis,
fluocinolone q8/q12 secondary infections
• Otitis externa
3-4 drops
q8/q12 • WOF for ear drops: caution
• Ophthalmic in perforated tympanic
solution membrane
Polymyxin, • Ear drops,
1-2 drops q4-6 • WOF for eye drops/ointment:
neomycin, eye drops, eye
dexamethasone • Ophthalmic allergic sensitization, elevation
ointment
ointment of intraocular pressure,
apply 1-1.5 fungal & seconda1y bacterial
inch ribbon into infections
conjunctiva!sac
q6/q8
• WOF:pruritus, rash, bone
Silver • Cover affected
sulfadiazine • Cream 1% marrow suppression, hemolytic
areas q12/q24
anemia, interstitial nephritis
• Ophthalmic
ointment
apply 0.5 inch
ribbon to
Tobramycin conjunctiva! sac • WOF: itching, swelling,
• Eye ointment,
with or without
dexamethasone q8/q12 (q3-4 eye drops conjunctiva! erythema
hours if severe
infection)
• Eye drops
1-2 drops q4

52
ANTIPARASITIC AGENTS
MAXIMUM DOSE/
DRUG INDICATION/DOSE PREPARATION
REMARKS

• Uncomplicated Malaria
10 mg base/kg PO then
6, 24, & 48 hours: 5 mg
base/kg PO; total dose:
• Max dose for prophylaxis:
300 mg/dose
• WOF: nausea, vomiting,
ECGabnormalities, QT
prolongation, blurred
I
2 5 mg base /kg • 250 mg/tab
Chloroquine vision,headache,confusion,
• Malaria prophylaxis (150 mg base)
skeletal muscle
5 mkdose PO q7 days
weakness,
(start 1 week prior &
increased liver
continue for 4 weeks
enzymes, hair
after exposure)
depigmentation
• Pinworm
l00mgPOxl
• Hookworm, roundworm,
whipworm • 500 mg tab • WOF: rash, headache,
Mebendazole 100 mg PO q12 x 3 days • 20 mg/mL syr diarrhea, abdominal
• Capillariasis • 50 mg/mL syr cramping
200 mg PO q12 x 20 days
• Toxocariasis
100-200mg POq12 x 5 days
Pediculus capitis
• Saturate hair and scalp
with shampoo. Leave for
10 mins then wash off.
May repeat in 9-10 days.
• WOF: pruritus,
• Shampoo, hypersensitivity,
Permethrin Scabies lotion burning, stinging,
• Apply lotion from neck erythema, rash
to toe (head to toe for
infants and toddlers).
Wash off in 8-14 hrs. May
repeat in 7 days.

53
ANALGESICS/ANTIPYRETICS

DRUG INDICATION/DOSE MAXIMUM DOSE/

PREPARATION
REMARKS
• Max dose for analgesic/
antipyretic: 4 g/day
• Ana/gesic/antipyretic
• WOF: GI upset, allergic
10-15 mkdose PO/PR
reactions, liver
q4/q6
• 80 mg tab toxicity, decreased
• Anti-inflammatory
• 100 mg tab platelet aggregation,
Aspirin 60-100 mkday PO q6/q8
• 300 mg tab tinnitus
• Kawasaki disease
• Do not use in <16
80-100 mkday PO q6
years for varicella or
during febrile phase,
flu-like symptoms
then 3-5 mkday PO OD
due to risk for Reye
syndrome
• 200 mg tab/cap
• Max dose:
• 300 mg tab
40 mkday, 1.2 g/day
• 400 mg tab
• WOF: GI distress,
Ibuprofen • 5-10 mkdose PO q6/q8 • 100 mg/2.5mL
rashes, ocular problems,
drops
hypertension, anemia,
• 100 mg/5mL syr
granulocytopenia
• 200 mg/5mL syr
• Maxdose: 1000 mg/ day
• 220 mg tab • WOF: GI bleeding,
• 275 mg tab thrombocytopenia,
Naproxen • 5-7 mkdose PO q8/q12
• 500 mg tab heartburn, headache,
• 550 mg tab drowsiness, vertigo,
tinnitus
• 325 mg tab
• 500 mg tab • Max dose: 90 mkday,
• 650 mg tab 4 g/day
• 100 mg/mL drops • WOF: hepatotoxicity
• 120 mg/5mL syr when used with
• 10-15 mkdose PO/PR
• 125 mg/5mL syr barbiturates,
q4 to q6
• 250 mg/5mL syr carbamazepine,
• 80 mg supp phenytoin, or chronic
• 125 mg supp alcohol use
• 250 mg supp
• 500 mg supp

Paracetamol Max dose:

• <SO kg: 75 mkday,
• 2:50kg, adolescents,
and adults: 4 g/day
• <SO kg: 15 mkdose q6 • 150 mg/mL amp • WOF:nausea, vomiting,
or 12.5 mkdose IV q4 • 10 mg/mLamp/vial constipation, pruritus,
• 2:50kg adolescents, and • 300 mg vial agitation, atelectasis,
adults: 500-1000 mg q6 • 1 gvial hepatotoxicity
or 650 mg IV q4 when used with
barbiturates,
carbamazepine,
phenytoin, or chronic
alcohol use

54
ANTIFUNGAL AGENTS
DRUG INDICATION/DOSE
Amphotericin B
• Initial dose
0.5-1 mkday
• Maintenance dose
0.5-1 mkday IV OD or 1.5
mkdose every other day
• Apply to affected areas
Clotrimazole
q8-12 x 2-8 weeks
• Oropharyngea/
candidiasis
LO 6 mkdose IV/PO,
MD 3 mkday IV/PO OD
• Esophageal candidiasis
LO 12 mkdose IV/PO,
Fluconazole
MD 6 mkday IV/PO OD
• Cryptocaccal meningitis
or invasive systemic
candidiasis
LO 12 mkdose IV/PO,
MD 6-12 mkday IV/PO OD
• Topical cream: 1-2
applications/day
Ketoconazole • Shampoo: 2x weekly
with at least 3 days
between applications
Nystatin • 4-6 mL swish & swallow q6
ANTIVIRAL AGENTS
IVfor HSV encephalitis
• 3 months-<12 years:
60 mkday q8 x 14-21 days
• ~12 years:
30 mkday q8 x 14-21 days
IVfor Varicella
• 2:2years:
Acyclovir
30 mkday q8 or
1500 mg/m 2 /day q8
x 7-10 days
POfor Varicel/a
• 2:2years:
20 mkdose q6 x 5 days
Influenza treatment (S days)
• <3 mos: 12 mg PO q12
• 3-5 mos: 20 mg PO q12
• 6-11 mos: 25 mg PO q12
Oseltamivir • 2'1 year & ,;;15kg: 30mg
PO q12
• >15-23 kg: 45mg PO q12
• >23-40 kg: 60mg PO q12
• >40 kg: 75 mg PO q12
55
MAXIMUM DOSE/
PREPARATION
REMARKS
• 50 mg vial
• Max dose: 1.5 mkday
• WOF: feve1;chills,
headache, hypotension,
vomiting, hypercalciuria,
hypoK- hyp0Mg 2 ·, RTA,
I
renaljliver failure
• Cream, powder, • WOF: erythema,
topical solution blistering, urticaria
• Maxfororopharyngeal
& esophageal
candidiasis: 400 mg/day
• Max for systemic
• 50 mg cap candidiasis/cryptococcal
• 150 mg cap meningitis: 800 mg/day
• 200 mg cap • WOF: nausea, headache,
• 200 mg vial rash, vomiting, diarrhea
abdominal pain,
hepatitis, cholestasis
• Begin MD 24 hours
after LD
• WOF: local burning
• Cream,
sensation, itching,
shampoo
contact dermatitis
• 100,000 IU/ml
• WOF: GI side effects
suspension
• WOF: renal
impairment [adequate
hydration and slow
• 250 mg vial
IV administration to
prevent crystallization
in renal tubules)
• 200 mg tab • Max dose: 800 kg/dose
• 400 mg tab
or 3200 mg/day
• 800 mg tab
• WOF: renal impairment
• 200 mg/SmL syr
• 75mgcap • WOF: nausea, vomiting
DRUGS USED FOR ALLERGY AND ASTHMA
I. ANTIHISTAMINES
MAXIMUM DOSE/
DRUG INDICATION/DOSE !"REPARATION
REMARKS
• 6-12 months:
2.5 mg PO OD
• 12-23 months: • 10 mg tab • WOF:sedation,
2.5 mg PO OD or q12 • 2.5 mg/mL drops headache,
Cetirizine • 2-5 years: • 10 mg/mL drops sore throat, GI
2.5 mg PO OD or • 1 mg/mL syr symptoms,
5 mg/day PO OD or q12 • 5 mg/SmL syr dry mouth
• ;;,6years:
5-10mg/dayPOODorq12
• 6-11 months:
• WOF: fatigue, dry
1 mg PO OD
mouth,
• 12 mo-5 years: • 5 mg tab
headache, diarrhea,
Desloratadine 1.25 mg PO OD • 2.5 mg/SmL syr
fever,
• 6-11 years:
insomnia,
2.5 mg PO OD
hypersensitivity
• ;;,12years: 5 mg PO OD
• Max dose: SO mg/
• 25 mg tab
dose, 300 mg/day
• 1-2mkdoselV/lM/ • SO mg cap
Diphenhydramine • WOF: sedation,
POq6 • 12.5 mg/SmL syr
nausea, vomiting, dry
• SO mg amp
mouth, blurred vision
• 6 months-<2 years:
15 mg PO q12
• WOF: drowsiness,
• 2-llyears: • 60 mg tab
fatigue, headache,
Fexofenadine 30 mg PO q12 • 120 mg tab
dyspepsia, nausea,
• ;;,12 years: • 180 mg tab
dysmenorrhea
60 mg PO q12 or
180 mg PO OD
• WOF: somnolence,
• 6 months-5 years: • 5 mg tab
dry mouth, cough,
1.25mgPOOD • 10 mg tab
headache, fatigue,
Levocetirizine • 6-11 years: • 2.5 mg/SmL syr
nasopharyngitis,
2.5 mg PO OD • 500 mcg/mL syr
pharyngitis, pyrexia,
• ;;e12years: 5 mg PO OD • 5 mg/mL syr
epistaxis, rhinitis
• 2-5 years:
5 mg PO OD • 10 mg tab • WOF: drowsiness,
Loratadine • ;;,6 years: • 5 mg/SmL syr fatigue, dry mouth,
lOmgPOODor headache,nausea
SmgPOq12

56
II. DRUGSFORASTHMA
MAXIMUM DOSE/
DRUG INDICATION/DOSE PREPARATION REMARKS

Ipratropium
bromide
• Nebulization for <12 yr:
250 mcg/dose q6/q8
• Nebulization for 2:12 yr:
250-500 mcg/dose q6/q8
• 500mcg/2mL
neb
• Salbutamol +
ipratropium
500mcg/ 2.5mg/
• WOF: anxiety,
dizziness, headache,
GI discomfort, cough
I
2.5mL neb
• 6 months-5 years:
• WOF: headache,
4 mg PO OD at night • 4 mg granules
abdominal pain,
• 6-14 years: • 5 mg tab
Montelukast dyspepsia, fatigue,
5 mg PO OD at night • 10 mg tab
dizziness, cough,
• >14 years:
elevated liver enzymes
10 mg PO OD at night
Nebulization: • WOF: tachycardia,
• <l years: palpitations,
0.05-0.15 mkdose q4-6 tremo1~insomnia,
• l mg/mL neb
• 1-5 years: nervousness, nausea,
• 2 mg/mL neb
1.25-2.S mg/dose q4-6 headache
• 2.5 mg/2.SmL neb
• 5-12 years: • Oral dose is discouraged
Salbutamol 2.5 mg/dose q4-6 due to increased side
• >12 years: effects & decreased
2.5-5 mg/dose q4-8 efficacy
• MDI: 100 mcg/
Inhaler
actuation • WOF: same as in
• 2 puffs q4-6 prn
• Inhalation powder nebulization
cap: 200 mcg

Ill. STEROID-CONTAININGDRUGS
MAXIMUM DOSE/
DRUG INDICATION/DOSE PREPARATION
REMARKS
• Betamethasone
di propionate • Max dose: 14daysAND
• Topical
cream or ointment • Cream and ointment:
Betamethasone Apply to affected areas
• Betamethasone 45g/week
q12 or OD
valerate cream, • Lotion: 50mljweek
lotion or ointment
Nebulized
• No prior steroid use: • Maxif no p1iorsteroiduse:
0.5 mg/day q12 or OD 0.5 mg/day
• 250 mcg/mL neb
• Prior inhaled steroid use: • Max if with prior
• 500 mcg/mL neb
0.5 mg/day q12 or OD inhaled/ oral steroid use:
• Prior oral steroid use: 1 mg/day
Budesonide 1 mg/day q12 or OD
• Max dose: 4
Oral inhaler DP!: inhalations/day
• 2:6years: 1 inhalation q12, • 200 mcg/ • WOF:pha1yngitis,cough,
may increase as needed metered dose HPA-axis suppression
• Rinse mouth after use

57
 5-11 years:
• 2 inhalations q12 of
80/4.5 mcg inhaler • Maxdose for 5-11 years:
;,,12 years: 2 inhalations of
• No prior inhaledsteroid use 80/4.5 mcg q12
2 inhalations q12 of MDI: • Max dose for ;,,12 years:
80/4.5 mcg OR 160/4.5 • 80/4.5 mcg 2 inhalations of
mcg inhaler depending inhaler 160/4.5 mcg q12
Budesonide +
on severity • 160/4.5 mcg • WOF: pharyngitis,
Formoterol
• Prior low-medium doses inhaler cough, HPA-axis
of inhaled steroid use • 320/9 mcg suppression,
2 inhalations q12 of inhaler abdominal pain,
80/4.5 mcg inhaler nausea, dyspepsia,
• Prior medium-high doses tremor
of inhaled steroid use
2 inhalations q12 of
160/4.5 mcg inhaler
Intranasal
• 2-llyears
Nasal spray:
(fluticasonefuroate):
• 27.5 mcg/
1 spray each nostril OD
actuation • Maxdose: 2 sprays per
• 4-11 years
(fluticasone nostril/day
(fluticasone propionate):
furoate) • WOF: epistaxis, nasal
1 spray each nostril OD
• SO mcg/ irritation
• ;,,12 years:
actuation
2 sprays each nostril OD,
(fluticasone
may reduce to 1 spray
propionate)
Fluticasone each nostril OD once
controlled
Inhaler
• 1-4 years: 100 mcg q12 MDI:
• >4 years: 50-100 mcg • SO mcg/
q12 if well-controlled actuation
• WOF: oral candidiasis,
asthma, 200 mcg q12 if • 125 mcg/
hoarseness
insufficient control actuation
• > 16 years: 100-1000 (fluticasone
mcg q12 (depends on propionate)
severity of asthma)
• Max dose: 2
inhalations q12 of MDI
250/10 mcg
• WOF: insomnia,
MDI: headache, tremor,
Fluticasone + • ;,,12 years: 2 • 50/5 mcg dizziness, palpitations,
formoterol inhalations q12 • 125/5 mcg, ventricular
• 250/10 mcg extrasystoles,
dysphonia, throat
irritation, dry mouth,
rash, peripheral
edema

58
 MDI
• >4 years: 2 inhalations
MDI:
q12 of 50/25 mcg
• 50/25 mcg • Max dose: 1
• ;,12 years: 2

Fluticasone +
salmeterol
inhalations q12 of
50/25 mcg, 125/25
mcg, or 250/25 mcg
DP!
• 125/25 mcg
• 250/25 mcg
inhalation q12 of
DP! 500/50mcg, or 2
inhalations q12 of MDI
250/25 mcg
I
.

• WOF: dysphonia, oral

• ;,4 years: 1 inhalation DP!: thrush, dermatitis
q12 of 100/50 mcg • 100/50 mcg
• ;,12 years: 1 inhalation • 250/50 mcg
q12 of250/SO mcg or • 500/50 mcg
500/50 mcg
• Max dose: 200/25
mcg/day
• WOF: cough,
DP!: dysphonia, oral
Fluticasone + • ;,12 years: 1 inhalation
• 100/25 mcg thrush, headache,
vilanterol OD
• 200/25 mcg nasopharyngitis, URTI,
pneumonia, abdominal
pain, arthralgia, back
pain, pyrexia
• In practice, commonly
IV/IM
used max dose is 100
• Anti-inflammatoty
mg IV Q6 (as an anti-
1-5 mkday IV/IM q12
• 100 mg vial inflammatory)
or OD
• 250 mg vial • Max for status
• Status asthmaticus
• 500 mg vial asthmaticus: LD 250 mg,
LD (optional)
MD 500 mg/dose q6
4-8 mkdose IV,
• Caution in immuno-
MD 8 mkday IV q6
compromised patients
Hydrocortisone • WOF:burning, itching,
irritation, dryness,
folliculitis,acneiform
eruptions, allergic
Topical
contact dennatitis,
• Apply to affected areas • Topical cream
hypopigmentation,
q6-12
perioral dermatitis,
skin maceration,
infection, skin atrophy,
striae & miliaria
Intranasal
• 2-11 years: 1 spray
• SO mcg/ • WOF: nasal burning
each nostril OD
actuation and irritation
• ;,12 years: 2 sprays
Mometasone each nostril OD
Topical • Do not use with
• Topical cream,
• ;,2 years: Apply to occlusive dressings or
ointment, lotion
affected area OD in diaper dermatitis

• 5 mg tab
• 20 mg tab
• See prednisone • WOF: same as
Prednisolone • 1 mg/mL drops
(equivalent dosing) prednisone
• 15 mg/SmL syr
• 20 mg/SmLsyr

59
 • Max: 60-80 mg/day
• Acute asthma • WOF: mood
2 mkday PO q12 or OD changes, seizures,
• 5 mg tab
x 5-7 days hyperglycemia,
• 20 mg tab
• Anti-inflammatory diarrhea, nausea,
Prednisone • 10 mg/5mL syr
0.5-2 mkday PO q12 abdominal distention,
• 20 mg/5mL syr
or OD GI bleeding, HPA
• Nephrotic syndrome axis suppression,
2 mkday PO q8-24 osteopenia, cushingoid
effects, cataracts

ANTICONVULSANTS
MAXIMUM DOSE/
DRUG INDICATION/DOSE PREPARATION
REMARKS
• <6 years: 10-20 • Max for <6 years:
mkday PO q8/q12, 35 mkday
titrate q5-7 days • Max for 6-12 years:
• 6-12 years: initial initial dose 100 mg/
dose 10 mkday PO dose q12, maintenance
q12, titrate by 100 dose 1000 mg/day
mg/day weekly, • Max for 12-15 years:
• 200 mg tab
maintenance dose lOOOmg/day
Carbamazepine
• 400 mg tab
20-30 mkday PO • Max for >15 years:
• 100 mg/5mL syr
q6-12 1200 mg/day
• > 12 years: initial • WOF: sedation,
dose 200 mg PO ql2, dizziness, diplopia,
titrate by 200 aplastic anemia,
mg/day weekly, neutropenia, urinary
maintenance dose retention, nausea,
800-1200 mg/day SIADH,Stevens-Johnson
PO q6-12 syndrome
• Max for sedation:
0.6 mg/kg
within 8 hours
• Max total dose for
• Sedation
seizures <5 years:
0.04-0.2 mkdose IV/
5 mg, may repeat dosing
IM q2-4 hour
in 2-4 hours as needed
Diazepam • Seizllre • 10 mg amp
• Max total dose for
0.2-0.5 mkdose IV
seizures 2:5years:
q15-30 mins
10 mg, may repeat dosing
in 2-4 hours as needed
• WOF: hypotension,
respiratory depression
• Do not mix with IV J/llids
• 4-15years: • Max for <16 years:
10 mkdose PO q12, 30 mkdose PO q12
may increase by • 250 mg tab • Max for 2:16 years:
10 mkdose q12 • 500 mg tab 1500 mg PO q12
Levetiracetam every 2 weeks • 750 mg tab • WOF: loss of
• 2:16years: • 1000 mg tab appetite, vomiting,
500 mg PO q12, titrate • 100 mg/mL syr dizziness,headache,
by 500 mg/dose q12 somnolence, agitation,
every 2 weeks depression, mood swings

60
 LD
• 15-20 mkdose IV
MD
• Neonate:
3-5 mkday IV/PO
q12 or OD
• Infant:
5-6 mkday IV/PO q12
•
•
15 mg tab
30 mg tab
• Max LO: 40 mg/kg IV
• WOF: drowsiness,
cognitive impairment,
ataxia, hypotension,
I
or OD • 60 mg tab
Phenobarbital hepatitis, skin rash,
• l-5years: • 90 mg tab
respiratory depression,
6-8 mkday IV/PO q12 • 130 mg/mL amp
apnea, megaloblastic
or OD anemia
• 6-12 years:
4-6 mkday IV/PO q12
or OD
• > 12 years:
1-3 mkday IV/PO q12
or OD
LD
• 15-20 mkdose IV • Max LO: 1500 mg/day
• WOF:gingival hyperplasia,
MD • 100 mg cap hirsutism, dermatitis,
• Start with 5 mkday • 30 mg/SmL syr blood dyscrasia, ataxia,
IV/PO q8/q12, usual • 125 mg/5mL syr lupus-like syndrome,
Phenytoin
dose range: • 100 mg amp Stevens-Johnsonsyndrome,
• 6 months-3 years: • 250 mg amp lymphadenopathy, liver
8-10 mkday damage, nystagmus
• 4-6 years: 7.5-9 mkday • IVpush/infusionrate NOT
• 7-9 years: 7-8 mkday to exceedlmgjkg/min
• 10-16years: 6-7mkday
• Max dose: 60 mkday
PO • WOF: GI, liver, blood
• 125 mg tab
• Initial and CNS toxicity,
• 200 mg tab
10-15 mkday q8-24 weight gain, transient
• 250 mg tab
• Increment alopecia, pancreatitis,
• 500 mg tab
5-10 mkday weekly nausea, sedation,
• 200 mg/5mL syr
Valproic acid • MD vomiting, headache,
• 250 mg/5mL syr
30-60 mkday q8/q12 thrombocytopenia, rash,
hyperammonemia
IV
• Same as PO dose but • WOF: same as PO
• 500 mg/5mL vial
give q6, use only when valproic acid
PO is not possible

61
ANTI HYPERTENSIVES

DRUG INDICATION/DOSE PREPARATION MAXIMUM DOSE/

REMARKS

• Child • Max dose: 6 mkday,

0.3-0.5mkdose POq8/q12, 450 mg/day
• WOF:rash, proteinuria,
titrate up as needed
• 25 mg tab neutropenia, cough,
Captopril • Adolescent
• 50 mg tab angioedema,
12.5-25 mg/dose PO
hyperkalemia,
q8/q12, titrate by
hypo tension,
25 mg/dose weekly diminution of taste
• Max dose:
• Child
• Child: 25 mcg/kg/day
5-10 mcg/kg/day PO
PO q6, 0.9 mg/day
q8/ql2, titrate qS-7 days • 75 mcg tab
• 2'12 years: 2.4 mg/day
Clonidine • i!12years • 150 mcg tab
• WOF: dry mouth,
0.1 mg PO q12, titrate
dizziness, drowsiness,
by 0.1 mg/day weekly
fatigue, constipation,
anorexia, arrhythmia
• Max dose: 600 mg/
day, 80 mg/dose
• WOF: ototoxicity,
IV/IM
• 20 mg amp hypokalemia,
• 1-2 mkdose q6-12
alkalosis, dehydration,
Furosemide hyperuricemia,
increased Ca2 - excretion
PO • Max dose: 6 mkdose,
• 10 mg/mL syr
• 2 mkdose 600mg/day
• 20 mg tab
• Increase by 1-2 mkdose • WOF: same as IV
• 40 mg tab
q6-8 furosemide

• Max dose: 10 mg/dose,

1-2mkday
• Max dose for sustained
• 0.25-0.5 mkdose PO/ • Soft gel cap: release tabs: 3 mkday,
SLq4/q6prn 5 mg, 10 mg 120 mg/day
• Sustained release tabs • Sustained • WOF: severe
Nifedipine
0.25-0.5 mkday PO q12 release tabs: hypotension,
or OD 20mg,30mg, peripheral edema,
60mg flushing, tachycardia,
headaches, dizziness,
nausea, palpitations,
syncope

• Hypertension • Max dose: 8 mkday,

0.5-1 mkday PO q6-12, 640 mg/day
titrate qS-7 days • WOF:hypoglycemia,
• 10 mg tab
Propranolol hypotension,nausea,
• Thyrotoxicosis • 40 mg tab
depression, weakness,
Adolescent 10-40 mg/
impotence,bronchospasm,
dose PO q6
heart block
• Max dose: 200 mg/day
• 25 mg tab
Spironolactone
• WOF: hyperkalemia,
• 1-3.3 mkday PO q6-24 • 50 mg tab
GI distress, rash
• 100 mg tab
gynecomastia
62
DRUGS FOR GASTROINTESTINAL COMPLAINTS

DRUG INDICATION/DOSE PREPARATION MAXIMUM DOSE/

REMARKS

Esomeprazole
• 1-11 years:
10 mg PO OD
• ;o,12years:
20-40 mg PO OD
• Cap: 20 mg, 40 mg
• Powder for oral
suspension:
10 mg
• WOF: GI disturbances,
headache
I
• 1-12 years: • Max dose: 40 mg/day
0.6-0.8 mkday IV qB-12 • WOF: headache,
Famotidine • 20 mg tab
• >12 years: constipation, diarrhea,
20 mg IV q12 drowsiness
• <6 years: 1 infant
suppository • WOF: rectal irritation,
• Pediatric 1.9 g
Glycerin PR q12 or OD prn abdominal pain,
• Adult 2.5 g
• ;,6 years: 1 suppository bloating, dizziness
PR q12 or OD prn
• <1 year: 2.5 ml q12
• 1-5 years: 5 ml q12 • Max dose: 60 mlfday
Lactulose • 5-10 years: 10 ml q12 • 3.3 g/5mL syr • WOF: GI discomfort,
• >10 years: 15-30 ml/ diarrhea
day q12 or OD
• Max for GER: 0.8
• GER
mkday, 15 mg/dose
0.1-0.2 mkdose IV/IM/PO
• Max for post-op
up to q6
nausea and vomiting:
• Post-op nausea/ • 5 mg/5mL syr
10 mg/dose
Metoclopramide vomiting • 10 mg amp
• WOF: Sedation,
,;14 years: 0.1-0.2
0

headache, anxiety,
mkdose IV q6/q8 prn
depression, leukopenia,
>14 years:
0
diarrhea, extrapyramidal
10 mg IV q6/q8 prn
symptoms at high doses

• 1 mkday IV/PO OD • Max for ulcers/GERO:

40mg/day
• 20 mg granules
• Max for pathological
• 20 mg cap
Alternative dose by weight hypersecretory
Omeprazole • 40 mg cap
• 5-<10 kg: 5 mg IV/PO OD conditions:
• 40 mg/vial
• 10-<20kg: 10 mg IV/PO OD 120 mg/day PO q8
• ;,20 kg: 20 mg IV/PO OD • WOF:headache, diarrhea,
nausea, vomiting

• Max dose: 200 mg/day

• WOF: headache, GI
IV/IM
• 50 mg amp disturbance, malaise,
• 2-4 mkday q6/q8
insomnia, sedation,
arthralgia, hepatotoxicity

• Max for ulcers:

PO 300 mg/day
Ranitidine • Ulcer treatment • Max for ulcermaintenance:
4-8 mkday q12 150 mg/day
• 150 mg tab
• Ulcer maintenance close • Max for GERO:
• 300 mg tab
2-4 mkday q12 300 mg/day
• G£RD/erosive • Maxforerosiveesophagitis:
esophagitis 600 mg/day
5-10 mkday q8/q12 • WOF: same as IV/IM
ranitidine

63
OTHER MEDICATIONS IN PEDIATRICS

DRUG MAXIMUM DOSE/

INDICATION/DOSE PREPARATION
REMARKS
• Max dose: 2 g
elemental Ca/day
• 45-65 mkday PO q6
• WOF: constipation,
• Doses expressed in mg
Calcium hypercaicemia,
of elemental calcium. • 500 mg tab
carbonate hypophosphatemia,
To convert to mg of salt,
hypomagnesemia,
divide by 0.4
nausea, vomiting,
headache, confusion
• Airway edema
0.5-2 mkday IV/IM q6
(begin 24 hours
before extubation and
continue for 4-6 doses • Maxdose for brain tumor
• 500 mcg tab
after extubation) associated cerebral
• 750 mcg tab
• Croup edema: 16 mg/day
Dexamethasone • 3 mg tab
0.6 mkdose IV/IM/PO • Contraindicated in
• 5 mg amp
x 1 dose active untreated
• 8 mg amp
• Brain tumor associated infections
cerebral edema
• LD 1-2 mkdose IV/IM
• MD 1-1.5 mkday IV/
1Mq4-6

• 250 mg tab
(elemental Fe 75
mg)
• 310mgtab • Max dose: 60 mg
(elementalFe100 elemental Fe q6
• Iron deficiency anemia
Ferrous mg) • WOF: constipation,
3-6 mkday (elemental Fe)
sulfate • 125 mg/mL drops dark stool, nausea,
PO qS-24
(elemental Fe epigastric pain
25 mg/mL)
• 150mg/5mL syr
(elemental Fe
30mg/5mL)
• Max dose:
• Initial: 0.5-1 gkdose IV
• Mannitol (20%) 100 ml IVq4
MannitoI • MD: 0.25-0.5 gkdose
20 g/lOOmL • WOF: hypovolemia,
1Vq4-6
headache, polydipsia

64
 Sedation for procedures
• 6 months-5 years: Max total dose
0.05-0.1 mkdose IV for sedation for
q2-3 mins prn

I
procedures:
• 6-12 years: 0.025-0.05 • 6 months-5 years: 6
mkdose IV q2-3 mins prn mg, 0.6 mg/kg
• >12 years: 0.5-2 mg/ • S mg amp • 6-12 years: 10 mg,
Midazolam dose IV q2-3 mins prn • 15 mg amp 0.4 mg/kg
• >12 years: 10mg
Sedation with
mechanical ventilation • WOF: respiratory
• 0.05-0.15 mkdose IV depression,
ql-2 hours prn, or hypotension,
• 1-2 mcg/kg/min bradycardia
continuous IV infusion
titrated to effect
• Nasal spray
2-6 sprays per nostril • 0.65% nasal
Sodium q2 prn spray • WOF: stinging of nose,
chloride -
• Drops • 0.65% nasal transient sneezing
inhaled
2-6 drops per nostril drops
q2 prn
IV/IM/SC
• Child: 2-3 mg/day OD or
1-2 mg/dose x 1 dose • WOF: flushing,
• 10 mg amp
• Adolescent: 5-10 mg/ dizziness, anaphylaxis,
day OD or 10 mg/dose hypotension, cardiac/
Vitamin K xl dose respiratory arrest
PO • IV injection rate not to
• Child: 2-3 mg/day OD exceed 1 mg/min
or 2.5-5 mg/day • 10 mg/mL
• Adolescent: 5-10 mg/day
OD or 2.5-25 mg/day

REFERENCES
1. Department of Health. (2014) National Tuberculosis Control Program Manual of Procedures
(5th ed.). Manila: Department of Health.
2. Kimberlin OW, Brady MT,Jackson MA, Long SS. (Eds.) (2015). American Academy of Pediatrics.
Red book: 2015 report of the Committee on Infectious Diseases (30th ed.). Elk Grove Village,
IL: American Academy of Pediatrics
3. Kliegman RM, Stanton BF, St Geme )W, Schor NF. (Eds.) (2020) Nelson Textbook of Pediatrics.
Philadelphia, PA: Elseviel'.
4. MIMS Drug Reference Philippines (151" ed.). (2017) Alexandra Technopark, Singapore: MIMS
Pte Ltd.
5. Sanden, E and Fleriage J (eds) (2015). The Harriet Lane handbook: a manual for pediatric
.house officers (20'" ed.). Philadelphia. PA: Mosby Elsevier.

65
INTERPRETATION
OFCOMMON
DIAGNOSTIC
TESTS
 SECTION ONE
ELECTROCARDIOGRAM
ECG WAVES AND SEGMENT
---------~--......,
RR lnterv~"l

.•••.•..•..•
.
..'. .. ..
.
. . ... .
. . .. .
,R.
P?ln~I

....... .
... ..... .-. .
: .........
. :•R••·••·
I
.
.. . .......
. . ,.
.......................................
. . .
.. . . . . ..
.,
I
.
.
•
.
................... .
.
. ~ •

I
.
.
. • •
. '• ll •
.........
•
. .
• •
. .......
..
• • •
. ..
o •
.
..
,.
• • ♦
.
• • • • •
"•
: • ~p~ 5:~: :f:
S~grr~• : ~ • • ~ •: •: •: •: • ~ . : ' • • : · : . ~ '

OT ln(erval

Figure1. Illustrationof the ECGwavesandsegments

P-Wave • Represents atrial depolarization, which begins at the sinus node

• Represents slow conduction within the AV node
PR Interval
• Serves as the bridge between atrial activation and ventricular activation
QRS Complex • Represents ventricular depolarization
• Includes the total duration of ventricular activation and repolarization
QT Interval
(e.g., QRS complex. ST segment, and T wave)
ST Segment& • Represents the plateau (ST segment) and later ventricular
TWave repolarization phases (Twave) of the cardiac action potential
UWave • Low-amplitude wave following the T wave

r
ECG STANDARDIZATION

Smm=
0.5 mV
l~
0.1 mV
'--1--+--+-~1--+---1

.,..._,.__,.__,..._..,._-t
Figure 2. In the standardrecordingspeed
(25 mm/sec)in thehorizontalaxis(time),one
small box (1 mm)is equivalentto 0.04 sec-
ondsor40 msec;andonebigbox(5mm)rep-
resents0.2 secondsor 200msec. In thever-
tical axis (amplitude),one small box (1 mm)
is equivalentto 0.1 mV amplitude;and one

L
big box(5 mm)represents0.5 mVamplitude.
A standardmarkeris usuallyinscribedon the
leftof thetracingas an uprightrectangle,with
a heightof 10 mm (10 smallboxes= 1 mV)

Speed: 25 mm/sec
1 mm= 0.04 sec or 40 msec
5 mm= 0.2 sec or 200 msec
25 mm= 1 sec
69
DIFFERENCES BETWEEN PEDIATRIC AND ADULT ECG

aVR aVL

,v,

Figures 3 and 4. Overview of the 15 Lead ECG in Pediatrics. The figure on the left is a representation of the
hexaxial reference system. The figure on the right shows the precordial leads.

1. A 15 lead ECGis requested for infants and young children

• Aside from the six limb leads & six precordial leads, three other leads are added:
, V3R
, V4R
, V7
Requested because there is RV dominance in infants (most noticeable during the
neonatal period):
Right axis deviation
0 Large rightward and/or anterior QRS forces: tall R wave in aVR & right precordial
leads (V4R, Vl, V2) & deep S waves in lead I & left precordial leads (VS,V6)
• It is also useful in evaluating right-sided congenital heart lesions

• Vl: 4•h JCS, right parasternal border
• V2: 4'h JCS, left parasternal border
• V4: S'h !CS, left midclavicular line
• V6: S'h !CS, left mid-axillary line
• V7: s•h !CS, left posterior axillary line
• V3R & V4R: mirror images ofV3 & V4
respectively
• V7: lateral to V6 at posterior axillary line
• VS: level ofV7 at the mid-scapular line
• V9: level of VS at the paravertebral line
(left posterior thorax, midway from spine
to VS)

70
2. Adult type R/S progression in precordial leads is rarely seen during first month of life
• Usual R/S progression in adults: deep S waves in Vl & V2 and tall R waves in VS & V6
• These findings may vary in pediatric patients:

AGE GROUP FINDINGS

• May have complete reversal of this progression (as compared to
Neonates
adults): tall R waves in Vl-V2 and deep S waves in VS-V6
1 month to 3
years of age

By 3 years of age
• Partial reversal of this progression is seen with dominant R wave in
Vl-V2 and in VS-V6

• Resembles ECGof a young adult

I

Figure5. ECGof a 1 week old infant. Notethe tall R waves in V1-V2and the deep S waves in V5-V6

aVR V1
v~
_J_A_ -r- ~
II aVL
V2~~ V5~

+- T
I~ av~ V3+V6J__
Figure6. ECGof a normaladult. Notethe deep S waves in V1-V2and the typicalR wave progressionto tall R
waves in V5-V6

71
STEPS IN PEDIATRIC ECG INTERPRETATION

• Step 1: Determine patient's age and gender

• Step 2: Rate and rhythm
• Step 3: Axis (P axis, QRSaxis, and Taxis)
• Step 4: Check AV conduction (P wave, PR interval)
• Step 5: Ventricular conduction (QRScomplex/duration, ST segment and Twave, QT interval)
• Step 6: Evaluation for chamber enlargement or hypertrophy

STEP 1. DETERMINEPATIENT'SAGEAND GENDER

• Age and gender may cause normal variations in the ECG
• Some changes are summarized below

A.Aspects Affected by Age

ASPECT EFFECT OF AGE
• Increases from day 1 of life to the first month of age, then decreases
Heart rate
from 3 months of age onward
• Varies from 135 degrees average during the first week of life and
QRSaxis
decreases to 60 degrees by 3-6 months of age
Pwave • P wave amplitude in V2 shows a gradual decrease with age

• Stable until 3 months of age and increases gradually

PR interval
• Also increases as the heart rate decreases
• R wave amplitude decreases with age in leads V3R & V1 and
increases in lead V7
R &S wave
• S wave shows inverse trend
amplitude
• This leads to overall steady decrease in the R/S ratio in leads V3R and
Vl and increased R/S ratio in leads V6 and V7 with increased age
QRS duration • Increases with age and varies with heart rate
• Upright in leads Vl and V3R at birth
Twave • Negative between days 3 and 7 of life
• May remain negative until 8 years of age

QTc interval • There is a small increase with age

B.Aspects Affected by Gender

QRS duration is longer in males
QTc remains relatively stable across the age spectrum, but at ~15 years of age, normal
females have a slightly longer QTc

C. Aspects Affected by Body Habitus and Race

QRS complexes will have lower voltage for those with more adipose tissue (e.g., obese)
0 Normal QRS values differ by race

72
STEP 2: RATE AND RHYTHM
A. Rate (varies with age)
HR greater than the upper limit of normal is termed tachycardia, while HR less than
the lower limit of normal is termed bradycardia
0 Rate is calculated by measuring either the P-P (atrial rate) or R-R interval (ventricular rate)
Lead II is commonly used to calculate the rate

Heart rate (HR)= 1500

# of small squares

21 small boxes
between R-R
I
1500
Heart Rate= 71 bpm
21
Figure 7. In this example, the HR is equal to 1500 divided by 21 small boxes or 71 beats/minute (bpm)

Short-cut method by memorizing selected HRfor R-R Values ("rule o/300")

Number of large boxes
R-R(mm) HEART RATE (bpm)
between R-R
5 1 300

10 2 150

15 3 100

20 4 75
25 5 60
For rapid calculation, the number of large boxes between two consecutive R-R intervals is
measured. Applying this short-cut in the example above, the R-R value is approximately 20
small boxes or 4 big boxes. The estimated heart rate is 75 bpm.

Determining Heart Rate for those with Irregular Rhythm:

Determine if rhythm is regular or irregular by counting the number of small squares in
between adjacent R waves (the rhythm is regular if the R-R intervals are constant)
• For irregular rhythms, the heart rate is computed as follows:

Heart rate=# ofR waves within 30 large boxes x 10 (if using 6 second strip)

Heart rate=# of R waves within 50 large boxes x 6 (if using 10 second strip)

10 11 12 13

Heart Rate= 13 R waves x 10 = 130 bpm

Figure 8. In this 6 second strip (30 large boxes), we multiply13 R-waves by the factor 10. This willgive a
heart rate of 130 bpm. The rhythm in the above tracing is atrial fibrillationwith rapid ventricular response.
73
 B. Rhythm
0 A normal rhythm implies that the cardiac impulse originates from the sinoatrial (SA)
node (e.g., sinus rhythm)
The normal rhythm, sinus rhythm, is characterized by:
P waves preceding each QRS, and
Normal P axis (0 to +90 degrees): P waves must be upright in leads I and a VF
0

STEP 3. P WAVEAND QRS AXIS

• The electric axis (direction of the net electric force in the heart) can change in different
cardiac conditions such as chamber enlargement or conduction disturbances

A. P Wave Axis
Mean vector of atrial depolarization
Electrical activity generated is moving from the high RA to low septa! RA and generally
moving from the right side of the heart to a leftward direction
A sinus P wave will appear positive in leads I, II, and avF and negative in lead avR (this
generates an axis ofO to +90 degrees)

B.QRS Axis
0 Mean vector of ventricular depolarization
0 Right sided forces dominate in the newborn
0 Axis will shift and will be predominantly left sided over time

1 week - 1 month +110 +30to+180

1- 3 months +70 +10to+125
3 months - 3 years +60 +lOto+llO
>3 years +60 +20 to +120
Adult +SO -30 to +105

Abnormal QRS Axis

ABNORMALITY DEFINITION RANGE
• Left ventricular hypertrophy (LVH)
Left axis • QRS axis is less than
• Left bundle branch block (LBBB)
deviation (LAD) the lower limit
• Left anterior hemiblock
Right axis • QRS axis is more than • Right ventricular hypertrophy (RVH)
deviation (RAD) the upper limit • Right bundle branch block (RBBB)
Extreme right • QRS axis from -90 to
axis deviation -180 degrees
- 90 0

Figure9. Illustration
of thedifferentinterpretations
of axes + 90°
74
Steps in Determining the Axis
1. Locate a Quadrant using Leads I and a VF
Axis Lead I Lead aVF Quadrant
.,..

0° to +90°
l l ·-EB.
I .,..

..-EB.
.,..

0° to -90°
l I .,..

+90° to :t:180°
j l ·-©·
....
..t9.
• goo

-90° to :t:180°
j I .,..

2. Among the remaining four limb leads, find the equiphasic QRS (wherein the R wave is
equal to the S wave). The QRS axis is perpendicular to that lead.

Sample Case. Determine the ORS axis for this patient:

I aVR

_L T
aVL
I~
+
II~ av~:

Step 1. Lead I has positivedeflection& lead aVF has a positivedeflection.Thereforethe axis fallssomewhere
in the leftinferiorquadrant

Axis Lead I Lead aVF Quadrant

.,o-

0° to +90°

~ _L ..~.
.so-
'-

75
 tep2. In thetracingbelow,theequiphasicQRSis in leadaVL(ie., theamplitudeof theR waveof aVLapproximates
he S wave)- therefore,the QRSaxisis perpendicular to aVL. Basedon thehexaxialsystem,axisis+ 60 degrees
-90

+90
aVF

STEP 4. CHECK ATRIOVENTRICULAR (AV) CONDUCTION (P WAVE AND PR INTERVAL)

A. P Waves
The P waves are usually symmetric and mound-shaped
0 Biphasic and notched P wave can be seen in children
Important for diagnosis of atrial enlargement
Best assessed in lead II
° Criteria for normal P waves:
Amplitude should be <3 mm
0 Duration <0.08 sec in infants <12 months of age and <0.1 sec in older children

FINDING CRITERIA ILLUSTRATION

-·I+
·Lead II
Right Atrial
Enlargement • Tall, peaked P waves;;, 3mm
(RAE)

Lead II

~
• Wide and notched P waves
• Duration in lead II:
0 >0.08 sec in infants

0 >0.1 sec in children

Left Atrial
Enlargement
(LAE)
V1

~
• Biphasic with prolonged
negative segment in lead Vl

Bia trial
Enlargement
• Combination of increased
amplitude and duration

76
+
8. P-R Interval
Bestassessedinleadll
° Calculated by measuring from the beginning of P wave to the first deflection of the QRS
( negative deflection if a Q wave or positive if an R wave)

The Normal PR Interval

0 The normal value for P-R interval depends on HR and age
Usually <0.2 seconds in older children and <0.13 seconds in newborns

I
0

HR 0-1 1-6 6 mos- 1-3 8-12 12-16

3-8 yrs Adult
(bpm) mos mos 1 yr yrs yrs yrs

<60 0.16 0.16 0.17

(0.18) (0.19) (0.21)

60-80 0.15 0.15 0.15 0.16

(0.17) (0.17) (0.18) (0.21)

80-100 0.10 0.14 0.15 0.15 0.15

(0.12) (0.16) (0.16) (0.17) (0.20)
100- 0.10 0.13 0.14 0.15 0.15
120 (0.12) (0.15) (0.16) (0.15) (0.16) (0.19)
120- 0.10 0.11 0.11 0.12 0.13 0.14 0.15
140 (0.11) (0.14) (0.14) (0.14) (0.15) (0.15) (0.18)
140- 0.09 0.10 0.11 0.11 0.12
160 (0.11) (0.13) (0.13) (0.14) (0.14) (0.17)

160- 0.10 0.10 0.10 0.10

180 (0.11) (0.12) (0.12) (0.12)

>180 0.09 0.09 0.10

(0.11) (0.11)
Valuesshownaboveare in seconds:NormalValue(UpperLimit)

< 12 months 0.075

1-3 years 0.08
3-5 years 0.085
5-12 years 0.09
12-16 years 0.095
Adults 0.120

Phi
' 'PR/
PATHOLOGIC
LOWER LIMIT OF PR INTERVAL (in seconds)
INTERVAL
• Myocarditis (rheumatic fever, viral, diphtheria), digitalis
P.-olonged PR or quinidine toxicity, congenital heart disease (endocardial
interval ( or first cushion defect, ASD,Ebstein anomaly), myocardial
degree AV block) dysfunction, hyperkalemia
• Normal heart with vagal stimulation
• Wolff-Parkinson-White(WPW), Lown-Ganong-Levine
syndrome, myocardiopathies, Friedrich Ataxia, Duchenne
Short PR interval
Muscular Dystrophy, Pheochromocytoma
• May be normal in children
• Wandering atrial pacemaker
Variable PR interval
• Mobitz Type 1 second degree AVblock (Wenckebach)
77
STEP 5. INTERVENTRICULARCONDUCTION
A.Q Waves
Represents left to right depolarization of the ventricular septum
Can be considered benign finding in children
Normal Q waves are small and narrow and usually less than one small box (1mm) wide
and no more than two small boxes (2mm) deep

B. QRS Complex
, R wave is an upward/positive infliction from the baseline
, S wave is a downward/negative deflection from the baseline that occurs after the R wave

Normal Values for QRS Complex Duration

Measured from the beginning of the Q wave to the end of the S wave
Increases with age due to increase in ventricular muscle mass
Best assessed in lead VS
, Usually <0.12 seconds (or 3 small boxes)

AGE MEAN (SECONDS) UPPER LIMIT (SECONDS)

0-1 month 0.05 0.07
1 month - 3 years 0.055 0.075
3-8 years 0.06 0.075
8-12 years 0.06 0.085
12-16 years 0.07 0.085
Adults 0.08 0.10

Causes of Prolonged QRS Complexes {see conduction disturbance & arrhythmias section)
CAUSES EXAMPLES
• RBBB or LBBB
Ventricular • WPW preexcitation
Conduction
Disturbances • lntraventricular blocks (e.g., hyperkalemia, procainamide or
qunidine toxicity, myocardial dysfunction)
• Premature ventricular contraction (PVC)
Ventricular
• Ventricular tachycardia
Arrhythmias
• Implanted ventricular pacemaker

C. QT Interval
The QT interval varies with HR
, Compute for corrected QT (QTc) using Bazett formula

Corrected QT interval (QTc} using Bazett Formula:

QTc = QT measured • Normal QTc is 0.40 ± 0.014 seconds
✓RR interval • Upper limit: .44 seconds in children >6 months

Check for Abnormalities

ABNORMALITIES EXAMPLES
• Long QT syndrome
Prolonged QT • Hypocalcemia
• Others: myocarditis, head injury, severe malnutrition
• Short QT syndrome
Short QT • Hypercalcemia
• Others: digitalis toxicity, hyperthermia

78
 D Check for ST and T wave Abnormalities
• Changes are rare in children
ST Segment • Normally isoelectric
• May have elevation or depression up to 2 mm in the limb leads
• Peaked T waves seen in hyperkalemia and LVH
Twaves • Flat or low T waves may be normal in newborns or seen in
hypothyroidism, hypokalemia, pericarditis, myocarditis, ischemia

STEP 6: EVALUATION FOR CHAMBER ENLARGEMENT OR HYPERTROPHY

A. Criteria for Left Ventricular Hypertrophy (LVH)
PARAMETER < 1 YEAR OLD > 1 YEAR OLD
I
Deep S waves in Vl > 20 mm > 20 mm
Tall R waves in V5 & V6 >20mm >25mm
< 0.20 in 1-5 years old
Low R/S raio in Vl-VZ < 0.8
< 0.10 in 6-12 years old
T waves in V5 & V6 Inverted Inverted
Abnormal Q waves in V5 & V6 >3mm >3mm
Others: InvertedTwave in V5& V6 (strainpattern)
Leftaxis deviation

8. Criteria for Right Ventricular Hypertrophy (RVH)

Tall R wave in Vl (or V4R)
Deep S wave in VS & V6 >7 mm
0 Increased R/S ratio in Vl or V2 (>2) after 6 months
Decreased R/S ratio in V6 (<1)
Upright Tin Vl & V4R (older than 3 days)
qR pattern in Vl (small q wave and tall R wave)
Right axis deviation
C. Biventricular Hypertrophy
0 Positive voltage criteria for RVHAND LVH(with normal QRSduration)
Positive voltage criteria for RVHor LVHand relatively large voltages for other ventricle
° Katz-Wachtel phenomenon: large equiphasic QRScomplexes in two or more of the
limb leads and in the mid precordial limbs (V2 to VS)

Examples:

V41~L
~L-LJJ'-av:,y~r--T-v1nT_
+~-lr-aVL~V2t--rrV5jj:~
1
Tl!•VFTTT VITT V6UL
Figure10.Tracingshowingdeep S wavesinV1(arrows)and largeR wavesinV6(arrows)consistentwithLVH.

+-H--
~aV~~ V1_Jf--.--J.-----J~
V4

aVLT----rT
-"1~--y'-- v2_+,-+~~
V51~r,
~aVF..,._,~ V3~ V6_,°Y~
Figure11.Rhythmis sinuswithrightaxisdeviation
(axis=+ 150degrees).
TheRwavesin leadsII & aVRandS wavesin I & V6are
beyondupperlimit.R/Sratioin V1or V2aregreaterthanupperlimitandin V6is lessthanlowerlimit.Tracingconsistent
withRVH.
79
COMMON ECG ABNORMALITIES
I. RHYTHMS ORIGINATING FROM THE SINUS NODE
Sinus Tachycardia or Bradycardia
• Both are considered "sinus" (because impulse originates in the sinus node
• Tachycardia (upper image): rate faster than upper limit for age (but usually <200 bpm)
• Bradycardia (lower image): rate slower than normal limit for age
·········• ·············•··:··· ······:·······

Sinus Arrhythmia
• Sinus rhythm is maintained
• Some children have phasic variation in heart rate: heart rate increases slightly during
inspiration and decreases slightly during expiration
Inspiration

II. RHYTHMS ORIGINATING IN THE ATRIUM (above the AV node)

Premature Atrial Contraction (PAC)
• Premature P complex, usually followed by a QRS complex
• P wave may be upright in lead II when the ectopic focus is high in the atrium and may be
inverted when the focus is low
• There may be a compensatory pause
RR 2x RR

Atrial Tachycardia
• Narrow QRS complex tachycardia in the absence of aberrancy or bundle branch block
• P waves occur at rapid rate
• P axis is different from sinus rhythm
• Usual heart rate is 110-160 bpm (but may reach 300 bpm

80
 Atrial Flutter
• F wave with sawtooth configuration
• The heart rate may be around 240-360 bpm
• QRS complexes are normal
• Ventricular response may have varying degrees of block (e.g., 2:1, 3:1, 4:1)
Normal ORS

Atrial Fibrillation
I
• Extremely fast atrial rate (F wave rate of 350-600 bpm)
• Irregularly irregular ventricular response with normal QRS
Rapid Ventricular Response

III. RHYTHMS ORIGINATINGIN THE VENTRICLE

• Rhythms originating from the ventricles are characterized by:
, Bizarre and wide QRS complexes
T wave pointing opposite the QRS complex
QRS complexes randomly related to P wave [if visible)

Premature Ventricular Contraction (PVC)

• Premature QRS complexes that are usually bizarre and wide
• Full compensatory pause is usually seen
• The length of two cycles including the premature beat is same as that of two normal cycles
·RR··

Ventricular Fibrillation
• Bizarre QRS complexes of varying sizes and configuration
• Rate is rapid and irregular

81
IV. ATRIOVENTRICULAR BLOCKS
First Degree AV Block
• Prolonged PR interval for age and HR
• PR interval is usually >0.2 seconds

Second Degree Heart Block, Mobitz Type 1 (Wenckebach)

• There is a progressive prolongation of the PR interval (marked by horizontal arrows), until
there is loss of AVconduction (or a drop beat), marked by the "X"

Second Degree Heart Block, Mobitz Type 2

• Constant PR intervals (horizontal arrows) with periodic drop beats

Third Degree Heart Block

• No atrial depolarization is conducted through the AV node
• P and QRS are independent of each other
• In the tracing below, arrows point to the P waves that are independent from the QRS

pp pp pp pp PP pp

V. RHYTHMS ORIGINATING IN THE ATRIOVENTRICULAR NODE (AV NODE)

Nodal or Junctional Rhythm
• AV node functions as main pacemaker resulting in slow HR (40-60 bpm)
• Characterized by absence of P wave or an inverted P wave after the QRS complex
• QRS complex duration and configuration are normal
·•········"·"·... ,. ..

82
 SECTION TWO
ARTE-RIAL BLOOD GAS INTERPRETATION
OVERVIEW OF ARTERIAL BLOOD GAS (ABG)
• Diagnostic exam that determines arterial oxygen (Pa0 2 ), carbon dioxide (pCO,), acidity
(pH), oxygen saturation (Sa0 2 ) and bicarbonate (HC0 3 )
• Venous blood gas (VBG) may be a suitable alternative and correlates with pH, CO, and
bicarbonate levels of ABG, but oxygenation correlates poorly with VBG -
I
._.
• Refer to Chapter 5 for indications, contraindications, and steps in performing ABG

BASIC STEPS IN ABG INTERPRETATION

1. Determine primary acid base disorder
2. Determine if compensation is appropriate
3. Check for secondary acid-base disorder (or mixed acid base disorders)
4. Check for anion gap and 6/ t. when needed
5. Check for oxygenation status

STEP 1. DETERMINE THE PRIMARY ACID-BASE DISORDER

NORMAL VALUES ABNORMAL VALUES

Lower value Higher value

Mean Value Normal Range
suggests ... suggests ..

pH 7.4 7.35-7.45 Acidosis Alkalosis

pC0 2 40 mmHg 35-45 mm Hg Alkalosis Acidosis
HC0 3 24mEq/L 20-28 mEq/L Acidosis Alkalosis
Sources:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted). Elsevier;2020
A. Check pH, pC0 2 , and HC0 3 levels
1. Look at the pH
If pH < 7.35: it is "acidic"
If pH > 7.45: it is "alkalotic"
If value falls within normal range (7.35 - 7.45), acidosis ( < 7.4) or alkalosis (> 7.4)
may still be present but compensated; so next step is to look at pC0 2, HCO,, & anion gap

2. Look at the Respiratory (pCO,) and Metabolic (HCO,) Components

• For pC0 2 : if value is> 45 mm Hg, it is "acidic"; if value is< 35 mmHg, it is "alkalotic"
• For HC0 3 : if value is< 22 meq/L, it is "acidic"; if value is> 28 meq/L, it is "alkalotic"

B Determine Primary Disorder· Match the pCO or HCO with the pH

Check pH
~_P"~, '"""l;.;,
. I PRIMARY
DISORDER

Low pH< 7.4 Low or normal Low (Acidosis) Metabolic acidosis

{Acidosis) High (Acidosis) High or normal Respiratory acidosis

HighpH>7.4 High or normal High (Alkalosis) Metabolic alkalosis

{Alkalosis) Low (Alkalosis) Low or normal Respiratory alkalosis
If pH is abnormal, determine whether the respiratory (pC0 2) or metabolic (HC0 3)
value is consistent with the pH value
• If pH is low (acidotic) + pCO, is high (acidotic)-> then disorder is "respiratory" acidosis
• If pH is high (alkalotic) + HC03 is high (alkalotic)-> then disorder is "metabolic" alkalosis
In some, both pC0 2 and HC0 2 may be consistent with the pH value
If pH is low (acidotic) + pC0 2 is high (acidotic) + HC0 3 is low (acidotic), there is
respiratory & metabolic acidosis
If pH is high (alkalotic) + pC0 2 is low (alkalotic) + HC0 3 is high (alkalotic), there is
respiratory and metabolic alkalosis
83
C. Determine Primary Disorder by Comparing Changes of HC03 and pC0 2
• To determine whether the disorder is primarily respiratory or metabolic,
compare changes of HC03 and pCO, from baseline using the following formula:
6pC0 2 = (pC0,-40) 6HC0 3 = (24-HCO )
40 24

CHECK
Check pH PRIMARY DISORDER
6pCO 2 and 6HCO 3

Low pH< 7.4 6HC0 3 > 6pC0 2 Metabolic acidosis

(Acidosis) LlpCO,> LIHC03 Respiratory acidosis
High pH> 7.4 LIHC03 > LlpCO, Metabolic alkalosis
(Alkalosis) LlpC02 > 6HC0 3 Respiratory alkalosis

STEP 2. DETERMINEAPPROPRIATENESSOF COMPENSATION

PHYSIOLOGIC EXPECTED COMPENSATORY
PRIMARY DISORDER
COMPENSATION* VALUE

Metabolic Acidosis
pC0 2 should decrease pC0 2 =(1.5 x HC03 ) + 8 ± 2
(low pH+ low HC03 )

pC0 2 increases by 7 mmHg for each

Metabolic Aikalosis 10 mEq/L increase in serum HC03
pC0 2 should increase
(high pH + high HC0 3 )
i 6pC0 2 =7 x LIHC0,/10
Acute: HC03 increases by 1 mEq/L for
each 10 mm Hg increase in pC0 2
Respiratory Acidosis i 6HC0 3 = 1 x LlpCO,flO
HC03 should increase
(low pH+ high pC0 2) Chronic: HC03 increase by 3.5 rnEq/L
fo1·each 10 mm Hg increase in pC0 2
i 6HC0 3 =3.5 x LlpCO,f10
Acute: HC03 falls by 2 mEq/L for each
10 mmHg decrease in pC0 2
Respiratory alkalosis i LIHC03 =2 x LlpCO,flO
HC03 should decrease
(high pH+ low pC0 2) Chronic: HC0 3 falls by 4 mEq/L for
each 10 mm Hg decrease in pC0 2
i LIHC03 =4 x LlpC02 /10
·in general.compensatoryresponsesoftenreturnpHtoward,butnotto. normalvalues(EXCEPT
in chronicrespira-
toryalkalosis,wherethereis oftenreturnof pHto normalvalue)

Sources:
Kliegman
R.etal.Nelson
Textbook
ofPediatrics
(21sted).Elsevier;
2020

84
STEP 3. CHECK FOR SECONDARY ACID-BASE DISORDER OR MIXED ACID BASE DISORDER
• Mixed acid-base disorders are defined as independently coexisting disorders (not merely
compensatory responses)
• Example: A patient with metabolic acidosis from diabetic ketoacidosis may develop an
independent respiratory disturbance (acidosis or alkalosis) from pneumonia

PRIMARY ACID- SECONDARY ACID-

COMPENSATION
BASE DISORDER BASE DISORDER

Metabolic
Acidosis
Actual reduction ofpCO, from baseline
is GREATER than that of predicted/
calculated compensation
Actual reduction ofpC0 2 from baseline
Secondary RESPIRATORY
ALKALOSISis present I
Secondary RESPIRATORY
is LESS than that of predicted/
ACIDOSISis present
calculated compensation
Actual increase of pC0 2 from baseline
Secondary RESPIRATORY
is GREATER than that of predicted/
ACIDOSISis present
Metabolic calculated compensation
Alkalosis Actual increase of pC0 2 from baseline is
Secondary RESPIRATORY
LESS than that of predicted/calculated
ALKALOSISis present
compensation
Actual increase of HC0 3 from baseline
Secondary METABOLIC
is GREATER than that of predicted/
ALKALOSIS is present
Respiratory calculated compensation
Acidosis Actual increase ofHC0 3 from baseline
Secondary METABOLIC
is LESS than that of predicted/
ACIDOSIS is present
calculated compensation
Actual decrease of HC0 3 from baseline
Secondary METABOLIC
is GREATER than that of predicted/
ACIDOSIS is present
Respiratory calculated compensation
Alkalosis Actual decrease ofHC0 3 from baseline
Secondary METABOLIC
is LESS than that of predicted/
ALKALOSIS is present
calculated compensation

Sample case to Demonstrate Steps 1, 2 and 3:

Case 1. A 5 year old male was admitted for diarrhea. An ABG was requested and revealed:
pH= 7.32 pCO, = 28 mmHg HC0 3 = 14 mEq/L

• Check the pH, HC0 3 , and pC0 2

0Since pH is< 7.4, it is ACIDOTIC
Step 1: Determine 0Since PC0 2 is< 40 mm Hg, it is ALKALOTJC
the primary
disorder
0Since HC0 3 is < 24 meq/L, it is ACIDOTIC
0In this case, pH (acidotic) and HCO, (acidotic) match,
suggesting that the primary disorder is "metabolic acidosis"
• The expected pC0 1 is computed as follows:
= 1.5 x I-IC03 + 8±Z
Step 2: Determine
appropriateness of = (1.5 x 14) + 8 ± 2 = 29 ± 2 = 27 to 31 mm Hg
compensation
• Since our patient's pC0 2 is 28 mmHg (falls within 27 to 31
mm Hg), the physiologic compensation is adequate

Step 3: Check for

• The patient's CO2 falls within the expected value
secondary acid base
• This is no secondary acid-base disorder
disorder

Final Interpretation • Compensated metabolic acidosis

85
STEP 4. CHECKFORANION GAP AND t,/t-.
A.. Anion Gap formula

• Normal anion gap is 4-11

Anion gap= [Na"] - [Cl} - [HC0 3 }
• High anion gap> 11

B . Usual Causes of Metabolic Acidosis

HIGH ANION GAP NORMAL ANION GAP
METABOLIC ACIDOSIS (HAGMA) METABOLIC ACIDOSIS (NAGMA)
M: Methanol H: Hyperalimentation
U: Uremia
A: Acetazolamide
D: Diabetic ketoacidosis
R: Renal tubular acidosis
P: Paraldehyde
D: Diarrhea
I: lsoniazid, Iron
U: Uretero-pelvic shunt
L: Lactic acidosis
P: Post-hypocapnea
E: Ethylene glycol, ethanol
S: Salicylates

C. For patients with Metabolic Acidosis, check for t,/t,

1. For High-Anion Gap Metabolic Acidosis (HAGMA)

L!Anion Gap • If= 1, there is pure HAGMA

• If< 1. there is HAGMA + NAGMA
!JHCO3 • If> 1, there is HAGMA+ metabolic alkalosis

2. For High-Anion Gap Metabolic Acidosis (HAGMA)

!JChloride Gap • If= 1. there is pure NAGMA

• If< 1, there is NAGMA+ HAGMA
!JHCO3 • If> 1, there is NAGMA + metabolic alkalosis

STEP 5. CHECKFOR OXYGENATIONSTATUS

I. MEASURES OF OXYGENATION
• The partial pressure of oxygen in arterial blood or Pa0 2 is a measure of dissolved oxygen
in arterial blood plasma
• Hypoxemia is defined as a low Pa 0 2 , which can be obtained by blood gas analysis
• A reduced PaO, is a non-specific finding (i.e. it only signifies a disturbance of gas exchange)
• Pao, is differerit from Sa0 2, which describes the amount of oxygen bound to hemoglobin
SaO, can also be measured via blood gas analysis
• When Sa0 2 is measured using pulse oximetry, the more appropriate term then becomes Sp0 2

A. Severity of Hypoxemia Levels at Room Air (Fi0 2 21 %)

SEVERITYOF HYPOXEMIA Pao, VALUE ESTIMATED SpO 2

Mild Hypoxemia 60-79 mm Hg 90-94%

Moderate Hypoxemia 40-59 mm Hg 75-89%

Severe Hypoxemia <40mmHg < 75%
Source:ShelledyDC:et al. Jones& BartlettPublishers:
2014

86
B. Determination of Fi0 2 in Patients with Nasal Cannula or a Simple Face Mask
° Fraction of inspired oxygen (Fi0 2) is the concentration ofoxygen (expressed in percent)
that a person inhales
0 Ambient or room air is made up of 21 % oxygen, hence Fi0 2 of room air= 21 %
0 When you give supplemental 0 2, you are raising the patient's Fi0 2 to a level over 21 %
(ranges from 22-100%)
° For every 1 LPM, there is an estimated increase of 4% in Fi0 2

Formula for Fi0 1 Determination:

Fi0 2 = 4% x liters per minute (LPM) + 20%

Example: A patient is on nasal cannula at 5 LPM. Compute for the Fi0 2 :

I
Fi0 2 = (4 x 5) + 20
=40%

OXYGEN DELIVERY OXYGEN FLOW RATES ESTIMATED FiO2

SYSTEM (in LPM) (in%)
1 24
2 28
3 32
Nasal Cannula
4 36
5 40
6 44
5-6 40
Simple Face Mask* 6-7 50
7-8 60
'Face masks cannotdeliver100%oxygen,unless there is a tightseal. Non-rebreatherface masks can deliver
an Fi02 up to ~80%.AnFi02 of 100%can onlybe deliveredwitha ventilatoror a tight-fitting
face mask.

C. Related Formulas

VARIABLE FORMULA REMARKS

Measured Pa0 2 on ABG should be • Pa0 2 less than 4-5 times

equal to 5 x FiO,- the Fi0 2 suggests poor lung
Expected
Pao, Based function or hypoventilation
A normal Pao, on room air
on Current • This can serve as a rough
(21% FiO,) is 105 mm Hg. A
Fi0 2 gauge for adequate
patient being given 50% Fi0 2
should have a Pa0 2 of ~250 mmHg oxygenation in the patient

Desired Pao,= 104 - (0.43 x age)

Note: In pediatric population, a

desired Pa0 2 oflOO mm Hg is used. • Used to adjust Ventilator Fi0 2
Desired Pao, Using the formula above, the range settings
of desired Pao, for ages 1 year old
to 18 years old would be 103.57 to
96.26 mmHg. Simply using a desired
Pa0 2 of 100 mm Hg would suffice.
• Increasing Fi0 2 can increase
Desired FiO,
Desired Fi0 2 Pa0 2 & Sp0 2 to desired levels
to Reach = (Current Fi0 2 x Desired PaO,) • CmTent Pa0 2 : obtained from ABG
Desired Pa0 2 • Desired PaO,: obtained using
(Current Pa02)
above formuh

87
II. PaO,-FiO, RATIO (Carrico Index or the PF Ratio)
• It is the ratio of arterial oxygen partial pressure (Pa0 2) to fraction of inspired oxygen (Fi0 2)
• Serves as a clinical indicator of hvooxemia
Pa0 2 : arterial oxygen partial pressure; obtained from ABG
Fi0 2 : fraction of inspired 0 2 (in decimal) at the time the ABGwas drawn
Normal PFR value on room air (at sea level) is 100 mm Hg / 0.21 = 476
mm Hg (or more)
PFR = Pao,
Fi0 1 For patients on non-invasive mechanical ventilation, Pediatric Acute
Respiratory Distress Syndrome (PARDS) can be diagnosed with a PFR,; 300

For patients on invasive mechanical ventilation, current recommendations

suggests using oxygenation index (01) and oxygen saturation index (OSI)
for classification of PARDSseverity

Samole Cases:
Case 1. 2 year old male is intubated for severe pneumonia and sepsis. Work-up showed:
pH= 7.25; pC0 2 = 50 mm Hg, HC0 3 20 mEq/L, pa0 2 = 90 mm Hg
Mechanical ventilator setting Fi0 2 - 40% Na·= 140 mmol/L, Cl= 105 mmol/L
• Since pH< 7.4 the primary disorder is ACIDOSIS
• In this scenario pC0 2 is increased while HC03 is decreased. This
suggests both metabolic and respiratory acidosis is present
Step 1: • To determine whether it is primarily respiratory or metabolic
Determine the
primary disorder acidosis see which has a higher change from baseline
0 tipC0
2 = (50-40)/40 = 0.25
0 tiHC0
3 = (24-20)/24 = 0.17
0 The tipC0
2 > tiHC0 3 , therefore it is primarily respiratory acidosis

• By eye-balling the values, there is an uncompensated disorder

because in acute respiratory acidosis, HCO, should increase
Step 2: • i tiHC0 3 = 1 x tipC0,/10
Determine 0 1 x (50-40)/10 = 1 mEq/L
appropriateness 0 Using this formula the HC03 is expected to increase by 1 mEq/L to
of compensation compensate for the increase in CO,
• Our patient's HC03 is 20 which is below the expected HC03 value
• Therefore our patient has UNCOMPENSATED RESPIRATORY ACIDOSIS
• The actual HC0 3 is less than the expected HC03
Step 3: Check for • This suggests a CONCOMITANTMETABOLICACIDOSIS
secondary acid
base disorder • The metabolic acidosis can probably be explained by tissue hypoxia
secondary to pneumonia and sepsis
• Anion gap= [Na·] - [CJ·]- [HCO;] = 140-105-20 = 15
• Our patient has HAGMA
Step 4: Anion gap • Compute fort:,,/ t:,, for HAGMA
and t:,,/t:,,when
0 ti Anion Gap/ tiHC0 3
needed
0(15-11)/(24-20) = 1
0ti/ ti= 1 suggests pure HAGMA
• The patient is intubated and being given an Fi0 2 of 40%
• Computing for Expected Pa0 2 = 5 x Fi02 = 5 x 40 = 200 mmHg
0 The patient's Pa0 is only 90 mmHg despite being given an Fi02 of
2
Step 5: Check 40%. This suggests hypoxemia that is uncorrected
oxygenation • Computing for the PFR = 90/0.4 = 225 (suggestive of PARDS)
status • Compute for Desired Fi0 2
= (Current Fi0 2 x Desired Pa0 2) / (Current pa0 2)
0 Desired Fi0 2 = (40x100)/90 = 44%
0 Therefore, the Fi0 2 should be increased to around 44%

Interpretation • Uncompensated respiratory acidosis & HAGMA

88
Case 2. 2 year old male is admitted for diarrhea with severe dehydration
ABG pH= 7.25; pC0 2 = 30 mm Hg; HC0 3 = 15 mEq/L, pa0 2 = 90 mm Hg at room air
Na= 140 mmol/L, Cl= 110 mmol/L
. • pH of 7.25 is ACIDOTIC
Step 1: Determine • HC0 3 is decreased suggesting ACIDOSIS
the primary • pC0 2 is decreased suggesting ALKALOSIS
disorder

Step 2: Determine
• pH and HC0 3 suggests acidosis therefore this is primarily
METABOLICACIDOSIS
• pC0 2 = 1.5 x HC03 + 8 ± 2
• pCO, = 1.5 x 15 + 8 ± 2 = 28.5-32.5 mmHg
I
appropriateness of
• Our patient's CO2 is 30 mm Hg which falls within the expected
compensation
value, the physiologic compensation is adequate

Step 3: Check for • The patient's CO2 falls within the expected value
secondary acid base
disorder • This is no secondary acid-base disorder

• Anion gap= [Na·] - [Cl"]- [HC0 3 ] = 140-110-15 = 15

• Our patient has HAGMA
Step 4: Anion gap • Compute for A/ A for HAGMA
and A//1 when
needed AAnion Gap/ AHC03
0

(15-11)/(24-15) = 0.44
0

• A/ A <1 suggests HAGMA and NAGMA

Step 5: Check • Patient is breathing room air and has a pa0 2 of 90 mm Hg which
oxygenation status is considered normal
• High anion and normal anion gap metabolic acidosis
Interpretation • Patient has severe dehydration. Lactic acidosis can explain the
HAGMA(diarrhea can account for the NAGMA)

Case 3. 4 year old female post surgery is intubated in the recovery room. Mechanical
ventilator setting of Fi02 60% and RR of 50 breaths per minute.

ABG pH= 7.49; pC0 2 = 30 mm Hg; HC0 3 = 22 mEq/L; pa0 2 = 350 mm Hg

• pH of 7.49 is ALKALOTIC
• pC0 2 is decreased from baseline suggesting ALKALOSIS
Step 1: Determine • HC0 3 is within normal but decreased from mean value
the primary
suggesting ACIDOSIS
disorder
• Since pH and CO2 suggests ALKALOSIS,this is compatible with a
PRIMARYRESPIRATORYALKALOSIS
• i AHC03 = 2 x ApCO,f 10 = 2 x ( 40-30)/10 = 2 mEq/L
Step 2: Determine • Expected HC0 3 is therefore 24 minus 2 mEq/L = 22 mEq/L
appropriateness of
• The patient's HC0 3 is equal to the expected value, the physiologic
compensation
compensation is adequate

Step 3: Check for • The compensation of HCO, is as expected, therefore there is no

secondary acid base secondary acid-base disorder
disorder • Step 4 (anion gap) is not applicable
• Predicted pa0 2 = Fi0 2 x 5 = 60 x 5 = 300 mmHg
0 Patient's pa0 2 is 350 mm Hg which is higher than predicted
Step 5: Check
• PFR = pa0,!Fi0 2 = 350/0.6 = 583 which is higher than normal
oxygenation status
• The patient has overcorrected hypoxemia. This suggests that the
Fi0 2 being given is too high and weaning may be initiated.
• Respiratory alkalosis with.overcorrected hypoxemia
Interpretation
• This can be caused by ventilator RRsettings that is too high for age

89
Case 4. 1 year old female is admitted for severe pediatric community acquired pneumonia.
Patient is tachypneic and has signs of dehydration from poor oral intake.

ABG pH= 7.45; pC0 2 = 25 mm Hg; HC0 3 = 18 mEq/L; pa0 2 = 65 mmHg at room air
Na= 140 mmol/L, Cl= 105 mmol/L
• pH of 7.45 is ALKALOTIC(within normal value but greater than
mean of7.4)
Step 1: Determine • pCO, suggests ALKALOSIS
the primary • HC0 3 suggests ACIDOSIS
disorder • pH and pC0 2 suggests ALKALOSIStherfore the primary disorder
is RESPIRATORYALKALOSIS
0 Respiratory alkalosis probably secondary to the tachypnea
• i 11HC03 = 2 x 11pC02/10 = 2 x [( 40-25)/10] = 3 mEq/L
Step 2: Determine • Expected HC0 3 is 24 mEq/L- 3 mEq/L = 21 mEq/L
appropriateness of • Patient's HC0 3 decreased more than the expected compensation.
compensation It is therfore compensated but also suggestive that there is a
secondary acid-base disorder

Step 3: Check for • The HC0 3 falls more than the expected compensation
secondary acid base • This suggests a CONCOMITANTMETABOLICACIDOSIS
disorder 0 Probably secondary to the dehydration
• Anion gap= [Na•] - [CJ·]- [HCO;J = 140-105-18= 17
• Our patient has HAGMA
Step 4: Anion gap • Compute for Ii/ Ii for HAGMA
and 11/11when
0 /iAnion Gap/ /iHC0
needed 3
0 (17-11)/(24-18) =1
• 11/11=1 suggests pure HAGMA
• Patient is breathing room air and has a pa02 of 65 mm Hg
= mild hypoxemia
• Compute for the desired Fi0 2
Step 5: Check
= (current Fi0 2 x desired pa0 2) / (current pa0 2) = 21x100/65 =
oxygenation status
32%
0 Patient should be given supplemental oxygen of at least 3LPM
via nasal cannula
• Compensated respiratory alkalosis with concomitant high anion
Interpretation
gap metabolic acidosis with mild hypoxemia

90
 SECTION THREE
READING CHEST RADIOGRAPHS
OVERVIEW OF RADIOGRAPHS
I. PRINCIPLES OF EVALUATING RADIOGRAPHS
A. Densities of Structure Determine Opacity (high density structures appear white)
Bone Soft Tissues Fat Air I

Figure16.Normalchestradiograph(imageon the left is a PAview,whileimageon the rightis a lateralview).RUL

- rightupperlungfield,RML- rightmiddlelungfield,RLL- rightlowerlungfield,LUL- leftupperlungfield,LLL- left
lowerlungfield,RA- rightatrium,LV- leftventricle,RV- rightventricle,LA- leftatrium,MPA- mainpulmonaryartery

C. Systematic Review of Structures: "ABCD"Style in Reading Radiographs

A-Abdomen • Check the gastric bubble or pneumoperitoneum (if taken upright)
B-Bones • Check for fractures
C - Chest • Check the heart, great vessels, and lungs
D-Devices • Check for devices (e.g., endotracheal, orogastric, chest tubes)

II. STANDARD PROJECTIONS

• Performed on older patients (teenage years), not advisable for younger
PA Upright patients due to their attention span (looking away from the 'camera' and
everyone else can make for a very agitated child)
AP Upright • Ideal for cooperative children
• Ideal for uncooperative younger children due to the ease of positioning
AP Supine
• AP supine (neonatal) performed mobile in the neonate unit
• To highlight pathology in mediastinum, costophrenic recess & localize lesions
Lateral • Requested to further assess and clarify lesions seen on front x-ray views
• Leftlateral view: preferred position as it provides better anatomic detail of heart
• Special projection used to demonstrate pleural effusions and pneumothorax
Lateral
• For pleural effusions, the side of interest should be down
decubitus
• For pneumothorax or pneumoperitoneum, the side of interest should be up
91
BASICS STEPS IN READING CHEST/BABYGRAM RADIOGRAPHS

• Step 1: Identify general data of the patient

• Step 2: Determine view (PA,AP,lateral, decubitus)
• Step 3: Assess quality of film
• Step 4: Assess anatomy
• Step 5: Determine the presence of abnormalities

Step 1: IDENTIFYING GENERAL DATA OF THE PATIENT

• Patient's name and age
• Date/time chest x-ray was taken

Step 2: DETERMINE THE VIEW

ANTEROPOSTERIOR (AP) VIEW POSTEROANTERIOR (PA) VIEW
Patient is supine Patient is upright
No winging of scapula Winging of scapula
Heart and other structures more magnified Heart is not magnified
Usually for infants, young children, and
very ill patients who cannot stand Usually for older children/adolescents

Straighter ribs Angulated ribs

Vertebral bodies are rectangular Mongolian hat sign may be appreciated

Clavicle is more horizontal V-shaped clavicle

92
Step 3: ASSESS THE QUALITY OF THE FILM
ASPECT QUALITY INDICATOR
• Good visualization is characterized by inclusion of entire thoracic cage
• Lung apices and first ribs caudally
Visualization
• Edges of ribs laterally
• Reported as: "good visualization"

Inspiratory
Effort
• Chest x-ray are conventionally taken while patients are in the
inspiratory phase of respiratory cycle
• One should count 6-8 anterior ribs, 9-11 posterior ribs
• Diaphragm should be intersected by the 6th rib in the midclavicular line
I
• Inadequate inspiration leads to crowding of structures
• Reported as: "good inspiratory effort"
• Of no value in infants
• This pertains to the degree to which x-rays have passed through the body
• A well-penetrated/exposed chest x-ray is one where the upper four thoracic
ve1tebrae and ve1tebrae behind the heart are just visible
• Reported as: "good exposure/penetration"
- p::

Exposure/
Penetration

Figure 011 the left displays an overexposed film (since >T4 are exposed)
Figure 011 the right displays an underexposed film, since <T4 are exposed)
• The spinous processes should be in the midline and should be
Obliquity/ equidistant from medial ends of the clavicle
Rotation • Rotation may lead to misinterpretation of structures
• Reported as: "no obliquity or significant rotation"
Presence of • Artifacts may be due to radiographic technique, patient factors (e.g.,
artifacts poor cooperation, obesity)or non-anatomical objects (e.g., devices)

Step 4: ASSESS ANATOMY

A Normal Lungs and Pleura
• The left lung is divided into two lobes: left upper lobe (LUL) + left lower lobe (LLL)
(dividing the two is the oblique fissure)
The right lung is separated into three lobes: right upper lobe (RUL}+ right middle lobe (RML}
Lateral chest x-ray is useful in delineating the fissures
Pleura: located in the interface between the lung zones & the ribs

B.Normal Bony Structure

• The bony structures are the densest and most radiopaque (whitest) structures seen on
a normal CXR
• The bones visible on the CXRare the clavicles, ribs, scapulae, parts of the spine & the
proximal humerus
• The bony structures are often used references by which obliquity, adequacy of
inspirations and exposure are assessed
93
C. Normal Heart and Vessels
l. Vascular Markings
Normal vascular markings are more prominent in the lower lung zones, show gradual
tapering towards the periphery of the lungs and are usually symmetric

2. Normal Mediastinum
The mediastinum contains the heart, great vessels (middle mediastinum) and
potential spaces anterior to the heart (anterior mediastinum), posterior to the heart
(posterior mediastinum) and above the heart (superior mediastinum)
Thymus increases in size from birth to puberty, but as the child grows, the thymus
appears smaller
3. Cardiothoracic Ratio (CTR) of the Heart
Size of heart is assessed by CTR (increased CTR suggests cardiomegaly)
Calculated by getting the ratio between the cardiac size and the thoracic width:
Cardiac size is measured by drawing vertical parallel lines down the most lateral
points on each side of the heart, and measuring between them
Thoracic width is measured by vertical parallel lines down the inner aspect of the
widest points of the rib cage & measuring between them (above the diaphragms)
Normal CTR measured in PAview
Since AP view causes magnification of heart size, the normal pediatric CTR varies
AGE MEAN CT RATIO RANGE
0-3 weeks 0.55 0.45-0.65
4-7weeks 0.58 0.49-0.70
1 year 0.53 0.45-0.61
1-2 years 0.49 0.39-0.60
2-6 years 0.45 0.40-0.52
>7 years <0.5 0.40-0.50

4. Cardiac Chambers and Great Vessels

See illustration above "Overview of anatomy in Chest Radiograph"
Left heart border corresponds to the lateral border of the left ventricle
Right heart border corresponds to the lateral border of the right atrium
Upper zone vessels are normally smaller than lower zone vessels

D. Other Structures
• Trachea is at the center (or slightly to the right) as an area of
Airways radiolucency
• It eventually branches at the carina into the two main bronchi
• Lung roots at the hila are occupied by major bronchi & pulmonary vessels
Hilar
• The left hilum is often higher than the right (both should be of similar
Structures
density and overall size)
Costophrenic • Created by the dome of each hemidiaphragm and the lateral chest walls
Angles • On frontal chest x-ray, costophrenic angles should form sharp acute angles
• Well-defined domed structures occupying the caudal end of the film
• Right hemidiaphragm is slightly higher than the left because of the liver
Berni-
below it pushes it cranially
diaphragm
• Below the left hemidiaphragm, a round area of lucency is occasionally
found (gastric bubble)

94
Step 5: DETERMINETHE PRESENCEOF ABNORMALITIES
A. Lungs
• Appear as areas which are whiter than surrounding structures
Increased density
• Area may be filled with exudates, transudates, blood and
in lung zones
malignant cells
Decreased density • Appear in areas which are darker than the surrounding structures

I
in lung areas • Area may be filled with air
Displacement of • Volume changes within a certain lung lobe/zone may lead to
lung fissures pushing or pulling of fissure
Lung
• >10 posterior ribs visualized in the lung parenchyma
hyperexpansion
Pleural thickening • Seen in asbestosis, mesothelioma
Prominence of
pleural spaces • Air may be trapped in pleural space (pneumothorax)

8 Mediastinum and Vessels

• Uniformly enlarged vessels at the hila and within the lungs
Increased • Main pulmonary artery is enlarged (greater than the diameter of
Vascularity the trachea) and forms a convex bulge
• Vessels are seen even in the periphery (loss of tapering)
Decreased
• Uniformly small·pulmonary vessels
Vascularity
• May be due to technical factors (e.g., improper patient
Mediastinal
Widening positioning, suboptimal inspiration, rotation) or presence of
masses or vessel widening

C. Heart
Left Ventricular
• Apex displaced inferiorly and laterally (drooping apex)
Enlargement
Right Ventricular
Enlargement • Apex displaced superiorly and laterally (e.g., uplifted apex)

• Prominence of left atrial appendage

Left Atrial • Loss of cardiac waistline
Enlargement • Widening ofcarinal angle (>70 degrees)
• Double density sign on right cardiac border
Right Atrial • Bulging right heart border (height> 1/2 of right cardiac
Enlargement silhouette and >l/3 ofright hemithorax)

D.Others
• Anything that increases volume or pressure in one hemithorax will
Tracheal push the trachea (and medastinum) towards contralateral side
displacement • If it causes volume loss, it will pull the trachea towards the
ipsilateral side
• Obscured hemidiaphragm may be due to adjacent lung disease
Diaphragmatic
• Displaced may be due to air /viscus underneath, phrenic nerve
abnormalities
palsy, or hernia

95
COMMON PATHOLOGIES & CHEST RADIOGRAPH FINDINGS
Pneumonia Consolidation
• Left lower lobe pneumonia showing • Inhomogeneous opacities with prominent
opacification in the lung field air bronchogram

Pulmonary Edema Pulmonary Tuberculosis

• Prominent hiilar vessels in a batwing • Hilar nodularities in primary infection
distribution & cephalization of vessels • Chon focus
• Thickened subpleural interlobular septae • Opacities in the upper lung fields and
at the lung periphery (Kerley B lines) cavitation (see image below)

Atelectasis (collapsed lung) Hyaline Membrane Disease

• Opacification of the lung fields, usually in a • Pronounced pulmonary underaeration
triangular shape • Fine granular (ground-glass) opacities in
• Elevation of the diaphragm both lung parenchyma.
• Narrowing of the rib interspaces • Peripherally extending air-bronchograms

96
Pneumothorax
• Lung collapse do to air in pleural space
• Absence of lung markings
Pneumoperitoneum
• Presence of gas underneath the diaphragm
• In this image, the dark area beneath
the hemidiaphragms correspond to air
occupying that compartment
Foreign body (coin in esophagus)
• Discoid metallic density (coin) in the
posterior tracheal region
• Coin is flat on lateral view
97
Pleural Effusion
• Blunting of costophrenic sulci
• Meniscus sign
• In massive effusion, there may be
shifting of the mediastinal structures to
the contra lateral side
Pericardia! effusion
• Generalized enlargement of cardiac
shadow ("water bottle sign") with normal
vascular markings
Congenital Diaphragmatic Hernia
• Presence of bowel loops in hemithorax
• Mediastinum is shifted contralaterally
• Abdomen is devoid of gas
BASIC
PROCEDURES
FOLEY CATHETER INSERTION

INTRAVENOUS LINE INSERTION

NASOGASTRIC/OROGASTRIC TUBE INSERTION

CAPILLARY BLOOD SAMPLING

ARTERIAL BLOOD SAMPLING

INTRAOSSEUS INFUSION

UMBILICAL VESSEL CATHETERIZATION

LUMBAR PUNCTURE

NEEDLE ASPIRATION

ENDOTRACHEAL INTUBATION

MANTOUX TEST (TUBERCULIN SKIN TEST)

 GENERALREMINDERSBEFOREDOINGBEDSIDE PROCEDURES
Perform aseptic technique when needed and observe proper hand hygiene. Always
introduce yourself to the patient and caregivers, and verify that the procedure to be done
is intended for the said patient. Explain the nature of the procedure to both the patient (if
possible) & caregivers and obtain consent as needed. Practice proper waste disposal and
hand hygiene after each procedure.

FOLEY CATHETER INSERTION

INDICATIONS AND MATERIALS NEEDED

I
CONTRAINDICATIONS
Indications • Foley catheter (appropriate size)
• Collection of urine for analysis and culture • Urine bag
• Management of urina,y retention and • Drapes
obstruction • 10 cc syringe
• Accurate monitoring of urine output • 1 vial sterile water
• Drainageof bladder p1im;dwing, or after surge1y • Clean (non-sterile) gloves
• Neurogenic bladder • Sterile gloves
• Cotton
Contraindications • Rubbing alcohol
• Presence of urethral trauma • Povidone-iodine
• Presence of acute pelvic fracture • Sterile lubricant and/or Xylocaine jelly
• Careful consideration should be given in syringe (plain sterile lubricant for infants)
patients with the following: • Micropore or Foley catheter holder
0Recent genitourinary surgery • Sterile specimen bottles (if for urine
0Genital abnormality collection)

METHODS
1. Observe hand hygiene and prepare materials using aseptic technique
2. Provide as much privacy as possible
3. Position patient properly and comfortably
° For males: supine position
0 For females: supine position with knees bent and hips flexed
4. Clean genital area if soiling is evident
5. Open foley catheter package, put aside but maintain sterile zone around foley catheter
6. Wear clean gloves
7. Expose the urethral meatus
0 For males: lift the penis and retract foreskin. Be careful when retracting the foreskin
because it may lead to complications such as paraphimosis and infection. The foreskin
should not be retracted beyond the point where it has naturally separated. A safer method
of catheterization is to do it without retracting the foreskin (see illustration on next page)
° For females: spread the labia
8. Clean urethral opening and surrounding tissue aseptically
° For males: circular motion from the urethral opening to the base of the penis
° For females: from above the urethral opening down towards the rectum
9. Change to sterile gloves
10. Lubricate the tip of the catheter generously
11. Insert the catheter into the urethral opening until the "Y" of the catheter
° For males: extend the penile shaft so that it is perpendicular to the abdomen
° For females: upward at approximately 30-degree angle
12. Observe for urine flow
13. Inflate the balloon slowly using sterile water. If the catheter has a guide wire (in very
small caliber catheters) remove the guide wire
14. Withdraw the catheter until resistance is met
15. Attach the end of the catheter to the urine bag
16. Secure the catheter to the upper leg with the Foley catheter holder or tape
17. Place urine bag below the level of the bladder
18. Dry patient's perineum
19. Instruct patient on catheter care
101
 Technique for catheterization without retraction: the thumb is used to stablize the penis,
while the index and middle fingers are used to occlude the preputial space and guide the
catheter through the preputial opening into the urethral meatus

Conditions Influencing the Selection of Foley Catheter Size

AGE WEIGHT(kg) FOLEY SIZE (Fr)

< 1.2 3.5 umbilical catheter

Neonate 1.2 - 1.5 5 umbilical catheter
1.5 - 2.5 5 umbilical catheter or size 6 Nelaton
0-6 months 3.5- 7 6
1 year old 10 6-8
2 years old 12 8_

3 years old 14 8-10

5 years old 18 10
6 years old 21 12
8 years old 27 12
12 years old Varies 12-14

102
INTRAVENOUS LINE INSERTION
INDICATIONS AND MATERIALS NEEDED
CONTRAINDICATIONS
Indications • IV cannula
• Administration of the following: • Microset/lV tubing
0 IV fluids • IV trolley
0 IV medications • Clean (non-sterile) gloves
0 IV chemotherapy • Tourniquet
0 IV nutritional support • Cotton
0 Radiologic contrast agents
• Blood sampling for laboratory tests

Contraindications
• Alcohol
• Antiseptic solution (2% chlorhexidine in
70% isopropyl alcohol)
• Micropore or Zinc Oxide Tape (usually
more appropriate because it adheres to
I
• Presence of injury, infection, or burns in
the extremity the skin better. Example is Leukoplast)
• Saline solution
• 2 mL syringes (2)
• Dressing pack
• Splint (optional)
METHODS
1. Hand hygiene and prepare materials needed
2. Wear clean gloves
3. Select position/site ofvenipuncture. A strong penlight may be used as an improvised vein
finder. The light is placed under the hand of the patient. If done inside a dim room, the
veins maybe visualized.
4. Draw up saline solution into 2-mL syringes (2)
5. Prime cannula and assembled equipment with saline solution
6. Swab puncture site with antiseptic solution and allow to air-dry
7. Apply tourniquet
8. Stretch the skin and stabilize the selected vein
9. Insert the needle bevel up
10. Observe for blood backflow
11. Remove the needle while pushing cannula further into the vein
12. Release tourniquet
13. Flush with saline solution to check patency
14. Use tape around the end of the hub of the cannula (without touching the insertion site)
15. Attach extension tubing and secure with tape
16. Apply splint
17. Instruct the patient on care of IV site

103
NASOGASTRIC/OROGASTRIC TUBE INSERTION
INDICATIONS AND MATERIALS NEEDED
CONTRAINDICATIONS
Indications • Nasogastric/orogastric tube (appropriate
• Disorders of oral feeding for intended purpose and size appropriate
• Increased nutrient or metabolic requirement to patient)
• Nutritional support/ feeding administration 0 OGT is used for neonates because
• Medication administration neonates are preferential nasal
• Bowel decompression in gastrointestinal breathers. Beyond the neonatal period,
obstruction common clinical practice is to use OGT
• Evaluation of upper GI bleeding when the patient has no erupted tooth/
• Aspiration of gastric fluid content from teeth yet, otherwise NGT is used.
recent ingestion of toxic material • Water-soluble lubricant
• Administration of radiographic contrast • Oral syringe or luer-lock syringe with
to GI tract syringe adapter (appropriate to type of
tube used)
Contraindications • Suction tubing and container
• Problems with the GI function • Clean (non-sterile) gloves
• Intestinal perforation • Zinc oxide tape
• Severe maxillofacial (midface) trauma • 2% viscous lidocaine ( 54mg/kg)
• Basilar skull fracture • Stethoscope
• Coagulation abnormality • pH strips
• Esophageal varices • Glass of water with straw
• Acid/alkaline ingestion
METHODS
1. Wear clean gloves
2. If patient is able to follow commands, instill 10 mL of lidocaine down nostril with the head
tilted backwards and ask patient to sniff and swallow
3. Ensure that the patient's vital signs and indicators of adequate oxygenation and
ventilation are monitored
4. Position the patient properly (supine)
5. Approximate length of insertion by measuring the distance from the tip of the nose to the
earlobe, then from the earlobe to the midpoint between the xiphoid and the umbilicus
(mark this point on the NGT/OGT)
6. Lubricate the distal tip of the tube
7. Gently and steadily insert tube through either the nostril or mouth to previously identified mark
(if patient cannot follow instructions, gently advance the tube. Do not force against resistance)
° For NGT: insert the tube into a nostril, aiming posterior and parallel to the nasal septum.
When the tube touches the pharynx, flex patient's head forward and ask patient to
swallow. Advance the tube as the patient swallows.
0 For OGT: position the end of the tube downward and insert the tube into the oral cavity
over the tongue. Aim the tube back and down towards the pharynx. When the tube
touches the pharynx, flex the head forward. Ask patient to take sips of water through a
straw while tube is advanced.
8. Verify tube placement by injecting 2-5 mL of air using oral syringe or luer-lock syringe
with syringe adapter (for high-risk patients: radiographic confirmation of placement)
9. lfph strip is a available, aspirate gastric content and test for pH reading (should be 56)
10. Once placement of tube is confirmed, secure the tube using zinc oxide tape
11. Loop the tube and cover the tip by partially inserting a part of the tube to the opening
(or keep open if used for decompression)
12. Instruct the patient and relatives on care of NGT/OGT

104
Selection of NGT/OGT Size based on Weight and Age

NASOGASTRIC EITHER AGE OR WEIGHT

TUBE SIZE (Fr) AGE WEIGHT(kg)
8 0-5 months 3-6
10 6-12 months 4-9
10-12 1-3 years 10- 15
12 4-7 years 16-20

Estimation o Tube Size Formula

Appropriate tube size (Fr) = (Patient's age + 16)
2
I

Approximation of length of insertion

NEMU - Nose, Ear, Mid-Umbilicus

105
CAPILLARY BLOOD SAMPLING
INDICATIONS AND MATERIALS NEEDED
CONTRAINDICATIONS
Indications • Lancet or automated disposable incision
• Blood sampling for laboratory tests (e.g., device
CBC,glucose, newborn screening) • Blood collection tubes (capillary tubes or
Microtainers)
Contraindications • Cotton
• Presence of local inflammation or hematoma • Alcohol
• Recent puncture in the same site • Clean (non-sterile) gloves
• Micropore
METHODS
1. Assemble equipment and supplies
2. Perform hand hygiene
3. Put on well-fitting, non-sterile gloves
4. Select the site and apply 70% isopropyl alcohol & allow to air dry
5. Puncture the skin with one quick, continuous, and deliberate stroke
6. Wipe away the first drop of blood and allow a second drop to form
7. Place the capillary tube in the drop of blood and invert the proximal end of the tube to fill
the capillary tube until blood reaches the demarcation line (avoid squeezing too tightly)
8. Dispose used sharps in an appropriate container
9. Dispose other used supplies in an appropriate container
10. Perform hand hygiene
TIP for heel-prick: allow the foot of the baby to "refill" by observing for capillary refill
(i.e. foot turns back to red) before pressing/squeezing it from time to time. Continuous
vigorous squeezing without waiting for refill will less likely lead to good flow of blood.

106
Conditions influencing the Choice of Heel or Finger-Prick (for capillary blood sampling)
CONDITION HEEL-PRICK FINGER-PRICK
Age 0 -'6 months Over 6 months
Weight 3 -10 kg Greater than 10 kg
On the side of the ball of the finger
Placement of On the medial or lateral
perpendicular to the lines of the
lancet plantar surface
fingerprint
Recommended Second and third finger (i.e. middle

I
. Not applicable
finger and ring finger)

ARTERIAL BLOOD SAMPLING

INDICATIONS AND MATERIALS NEEDED

CONTRAINDICATIONS
Indications • Pre-heparinized syringe
• Arterial blood gas analysis for evaluating • 20-, 23-, or 25-gauge needle
the respiratory status and acid-base • Safety syringe with a needle cover that
equilibrium in patients with respiratory allows the syringe to be capped before
distress or metabolic derangements transport
• For routine laboratory analysis or blood • Cotton
culture if venous blood is difficult to obtain • Alcohol or povidone-iodine
Contraindications • Sterile gloves
• Presence of local inflammation, infection, • Micropore
burns, or other damages • Dressing
• Bleeding problems • Ice-filled container

METHODS
1. Position patient properly (supine) and comfortably and restrain the child if needed
2. Locate radial artery by performing an Allen test for collateral circulation
3. Disinfect sampling site with antiseptic solution and allow to air-dry
4. Penetrate skin at a 30- to 45-degree angle. Neonates have arteries that are more shallowly
located. Therefore, the needle is inserted at a lesser angle (i.e. more parallel to the skin).
5. Advance the needle slowly until radial artery is punctured or resistance (bone) is met
6. Observe for blood flashback
7. Provide continuous but gentle suction with the plunger of the syringe
8. Allow syringe to fill to the appropriate level
9. Withdraw the needle and syringe
10. Place gauze or cotton over puncture site and apply pressure to stop bleeding
11. Expel air bubbles, cap syringe, and roll specimen between the hands to gently mix it
12. Cap the syringe, label and send specimen to laboratory immediately

NOTE: A common clinical practice is to extract arterial blood using the "fountain" method
wherein a bare needle is used instead of a syringe. Blood is then allowed to drip from the needle
and collected. The reason for such practice is that a bare needle is less likely to be dislodged as
compared to a needle with a syringe attached to it. This procedure is NOT ideal.

107
INTRAOSSEOUS INFUSION
INDICATIONS AND MATERIALS NEEDED
CONTRAINDICATIONS
Indications • Chlorhexidine or povidone iodine
• Vascular access emergently needed but not • Surgical mask
immediately available via peripheral vein • Latex-free sterile gloves
• Emergency administration of medications • Eye protection
and fluids • 10 mL syringe
• Syringe with saline flush
Contraindications • 10 needle and/or device (e.g. manual
• Bone fracture IO needle). If IO needle is not readily
• Recent 10 attempt in same insertion site available, an alternative would be a
• Osteogenesis imperfecta regular gauge 16/18 needle.
• Osteopetrosis • 1% lidocaine (awake patients) in small-
• Overlying infection, cellulitis, burns, or volume syringe
osteomyelitis at proposed insertion site • Micropore
• Dressing
METHODS
1. Wear surgical mask, eye protection, and sterile gloves
2. Prepare the injection site with an antiseptic
3. If patient is awake, infiltrate the skin, subcutaneous tissue, and periosteum with 1 %
lidocaine
4. Place the leg with knee extended in neutral position and then slightly externally rotate at
the hip to expose the flat part of the tibial surface, and externally rotate the foot
5. Check the needle to ensure that the bevels of the outer needle and the internal stylet are
properly aligned
6. Grasp the leg distally and lateral to the insertion site with the palm and fingers of the non-
dominant hand to brace the leg against the force of IO placement and to prevent distal leg
movement during the procedure
7. Palpate the landmarks to identify the flat surface of the tibia approximately 1 to 2 cm
below and slightly (up to 1 cm) medial to the tibial tuberosity, and insert the IO needle
through the skin
8. Direct the IO needle perpendicular to the entry site 01; in skeletally immature children, at
a slight angle (10 to 15 degrees) from vertical (caudad for the proximal tibia); cephalad
for the distal tibia or femur
9. Apply pressure with a twisting motion
10. Unscrew the needle cap and remove the stylet
11. Confirm correct needle placement by aspirating blood
12. Once proper placement is confirmed, flush the needle with 10 mL of normal saline and
connect it to conventional IV tubing
13. Secure the bone marrow needle with tape and a dressing that does not obscure the
needle placement site so that infiltration can be rapidly detected
14. Remove the needle by grasping the shaft and pulling up with a slight rotary motion
15. Apply pressure to IO site
16. Dress site using aseptic technique
17. Provide analgesia, as needed and depending upon patient status

108
INTRAOSSEOUS NEEDLE INSERTION SITE

Altcrnato
SIio

Intraosseous needle insertion sites in Proximal tibia intraosseous needle

I
infants or small children insertion site in infants or small children

Femur

lntraosseous needle insertion sites in older Distal tibia intraosseous needle insertion
children and adolescents site in older children and adolescents

STEPS IN INTRAOSSEOUS NEEDLE INSERTION

1 2

Position and stabilize the leg Insert the needle at a 90° angle and apply
pressure with a twisting motion

Confirm position with marrow aspiration

109
UMBILICAL VESSEL CATHETERIZATION
INDICATIONS AND MATERIALS NEEDED
CONTRAINDICATIONS
Indications • Umbilical catheter
Umbilical Artery Catheterization 0 Less than 3.5 kg: Fr 5
• For frequent sampling of arterial blood 0 More than or equal to 3.5 kg Fr 8
gas, continuous monitoring of arterial • Antiseptic solution (e.g. Povidone-iodine)
blood pressure and route for infusion of • Sterile gloves
parenteral fluid • Medical tape
• Reserved for seriously ill infant because of • Scalpel
high rates of complications; blanching of • Mosquito
extremity, infection, thrombosis, etc.
• CDCrecommends a maximum of? days
Umbilical Vein Catheterization
• For early and emergency vascular access
in neonates
Contraindications
• Omphalitis
• Omphalocele
• Peritonitis
• Necrotizing enterocolitis
• For umbilical artery (UA): evidence of
ischemia in lower extremities
• For umbilical vein: pmtal venous hypertension
METHODS
1. Determine catheter length
2. Hand hygiene and aseptic technique
3. Place infant in a supine position
4. Tie the base of the umbilicus using a firm half knot that will help control bleeding while
allowing the passage of the catheter
5. Cut the cord approximately 2 cm above skin
6. Identify the vein (located at the 12 o'clock position, thin-walled) or artery (thick-walled)
7. Hold the base of the umbilicus and insert the pre-flushed umbilical catheter
8. Observe the blood to flow through the catheter
9. Once adequate placement confirmed, secure the catheter using a tie or adhesive medical tape

How to Verify Positioning of the Catheter

UMBILICAL ARTERY UMBILICAL VEIN CATHETER
CATHETER (UAC) (UVC)
• Low position (between T3-T4 • For emergency vascular access:
vertebra): 3-5 cm in full term, 2-4 cm in
Length (cm)= BW [kg)+ 7 preterm
Length of • For continuous infusion:
Catheter to be through the ductus venosus into
inserted • High position (between T6-T10
vertebra): the inferior vena cava, position
Length (cm)= (3 x BW [kg])+ 9 is verified by radiograph
Length= [(3 x BW [kg]) + 9]/2
• In AP view, catheter shows an • In AP view, the catheter extends
Radiographic initial downward course from immediately superior from the
examination
the umbilical insertion into the umbilicus
can be used
to determine internal iliac artery • In lateral view, the catheter
placement • In lateral view, the catheter courses anteriorly
courses posteriorly

110
IDENTIFYING THE UMBILICAL VESSELS

1 2

I
VERIFYING THE CATHETER POSITION

Umbilical venous catheter

Umbilical artery catheter

AP View showing UAC initially coursing down in contrast to the UVC extending
superiorly

/,Umbilical artery catheter

,~ ~mbilical venous catheter

Lateral view showing the course of the umbilical catheters (UAC versus UVC)

111
LUMBAR PUNCTURE
INDICATIONS AND MATERIALS NEEDED
CONTRAINDICATIONS
Indications • Spinal Needle
• Diagnosis of • Sterile Gloves
Suspected CNS infection
0 • Drapes
Suspected subarachnoid hemorrhage
0 • Antiseptic solution (Povidone-iodine)
• Introduce contrast agents into the CSF for • Specimen bottles
diagnostic purposes • Syringe
• Measure pH, enzymes, neurotransmitters • Lidocaine
and trace constituents
• Measure and fractionate CSF proteins in
suspected immunologic disease especially
Multiple Sclerosis or Guillain-Barre
syndrome
• Identify neoplastic invasion or seeding
of the subarachnoid space by gliomas,
carcinomas, leukemias, lymphomas
Contraindications
• Suspected mass lesion in the brain
(especially posterior fossa or
supratentorial lesions with midline shift)
• Impending cerebral herniation
• Critical illness/unstable condition
• Skin infection
• Thrombocytopenia < 20 xl0 9 /L
METHODS
l. Prepare materials
2. Positioning: Position an acutely ill patient on the side with the legs drawn up and the
spine flexed to increase the distance between the processes and lamina of the adjacent
vertebrae
3. Palpate the iliac crest and slide down to L4 to LS
4. Needle Insertion and manometry:
Scrub skin with antiseptic
0

Insert needle in the interspace between the dorsal processes of vertebra L4-L5 or LS-Sl
0

towards the umbilicus

Angle the needle slightly cephalad, inserting with its bevel turned parallel to the long
0

axis of the spine

Remove stylet to check for backflow of the cerebrospinal fluid (CSF)
0

Once the back flow of CSF is constant, remove sty let and attach to manometry tube
0

5. Collection of the CSF:collect 10-lSmL of CSF by allowing several mL to drip into the tubes
0 1: Gram stain, culture and sensitivity

2: Cell count, differential count

0

3: Chemistries - protein, sugar

0

4: Special studies
0

1 2

Position the patient Needle insertion

112
NEEDLE ASPIRATION
INDICATIONS AND MATERIALS NEEDED
CONTRAINDICATIONS
Indications • Large-bore intravenous catheter (Gauge
• Urgent management of tension 14or 16)
pneumothorax or primary spontaneous • Syringe
pneumothorax • Three way stopcock
• Sterile gloves
Contraindications (Relative) • Povidone-iodine
• Anticoagulation, coagulopathy and
bleeding diathesis

METHODS
•
•
•
Sterile gloves
Cotton
Micropore I
1. Prep the site with povidone-iodine
2. Locate the site at the second intercostal space superior to the third rib at the
midclavicular line
3. Hold the needle that is connected to a syringe via a 3-way stopcock perpendicular to the
chest wall
4. Puncture the site in the middle third to minimize the risk of injury to the internal
mammary artery
5. Place the catheter just above the cephalad border of the rib
6. Remove the needle leaving the cannula in place
7. Air is withdrawn manually until no more can be aspirated
8. If no further air can be aspirated, close the stopcock
9. Send patient for tube thoracostomy

113
ENDOTRACHEAL INTUBATION VIA DIRECT LARYNGOSCOPIC TECHNIQUE
INDICATIONS ANO MATERIALS NEEDED
CONTRAINDICATIONS
Indications • Endotracheal tube of appropriate length
• Acute respiratory failure • Stylet
• Airway protection • Sterile lubricant
• Preoperative setting in patients requiring • Laryngoscope
general anesthesia • Zinc oxide tape
• Advance airway in patients suffering from • Sterile gloves
cardiac arrest • Oxygen tubings
• Bag valve mask
Contraindications • Suction device
• Trauma to upper airway • Lidocaine spray
• Pulse oximeter
• Cardiac monitors
• Sedative medication
• 10 cc syringe
• Stethoscope
METHODS
1. Obtain assistance
2. Prepare materials
3. Wear gloves
4. Assess, preoxygenate and position patient
5. If properly trained with Rapid Sequence Intubation, may administer medications for RSI.
Otherwise, skip this step.
6. Open the patient's mouth and carefully position the laryngoscope
7. Deflect the tongue and soft tissue to the left side of the mouth with the flange
8. Identify and locate the epiglottis
9. Improve view by using bimanual laryngoscopy, head elevation, and lower neck flexion
(not done when there is suspected neck trauma/injury)
10. Insert the endotracheal tube through the vocal cords into the trachea under direct vision
11. Remove the stylet and inflate balloon
Note: It is a common clinical practice to use uncuffed ET in neonates and children.
Cuffed is mostly used for adolescents and adults. Howevet; literature states that both
cuffed and uncuffed are acceptable for intubating infants and children.
12. Confirm positioning of the tube within the trachea through visualization, auscultation,
and CO2 detection
13. Check for any secretions/output from the ET tube
14. Secure the tracheal tube
15. Hook to mechanical ventilator
16. Provide sedation and analgesia
17. Send specimens for culture studies (ETA GS/CS, ETAAFB, ETAGeneXpert)
18. Send for post-intubation AP films to confirm tube placement and adjustment
19. Send for post-intubation blood gases to check oxygenation and gas exchange as well as
to guide adjustment of mechanical ventilator settings

114
DIRECT LARYNGOSCOPIC TECHNIQUE

I
2

DETERMINING THE ENDOTRACHEAL TUBE SIZE:

For Neonates
SIZE WEIGHT GESTATION
(uncuffed)
2.5mm < 1000 g < 28 weeks
3.0mm 1000 to 2000 g 28 to 34 weeks
3.5 mm 2000 to 3000 g 34 to 38 weeks
4.0mm > 3000 g > 38 weeks
Formula for Depth ofET· Length (cm)= weight (kg) + 6
For 1 kg Depth of 7 cm
For 2 kg : Depth of8 cm ET should not extend> 4 cm or< 4 cm from infant's lip
For 3 kg Depth of9 cm

For Children >1 Year Old

Size (uncuffed) = Age (Years) +4

4
*subtract 0.5 if using cuffed ET tube

Length (cm)= Age (Years)

-----+12
2
Source:PediatricAdvancedLifeSupport,2005
115
MANTOUX TEST AND INTERPRETATION (TUBERCULIN SKIN TEST)
INDICATIONS AND MATERIALS NEEDED
CONTRAINDICATIONS
Indications • Tuberculin syringe
• Diagnosis of latent tuberculosis • Tuberculin material (purified protein
derived tuberculin material)
• Cotton
• Alcohol (70%)
METHODS
1. Hand hygiene
2. Prepare materials
3. Identify the ventral aspect of the arm
4. Clean site with cotton and alcohol
5. Inject 5 tuberculin units (0.1 mL) intradermally
6. Assess the site after 48 to 72 hours
7. Measure the induration, the palpable, reel & hardened area (not the erythema), in
millimeters
8. Measurement is done across the forearm, perpendicular to the long axis. Measure for the
widest cliamete1; a pen maybe used to mark the area of measurement.

Interpreting the TST

TST SIZE SITUATION IN WHICH REACTION IS CONSIDERED POSITIVE
• History of close contact with a known or suspected case of TB
• Clinical findings suggestive of TB
• Chest X-ray findings suggestive of TB
• lmmunocompromised condition
• Positive TST

REFERENCES
1. Pediatric Advanced Life Support. Circulation 2005;112;1V-167-IV-187
2. Kliegman, R., ST GEME Ill,)., Blum, N., Shah, S., Taske1; R., Wilson, K., & Behrman, R. (2020).
Nelson Textbook of Pediatrics (21st ed.). Philadelphia: Elsevier
3. Cloherty, J.,Eichenwald, E., Hansen, A., & Stark, A. (2012). Manual of Neonatal Care (7th ed.).
Philadelphia: Lippincott Williams & Wilkins.
4. Center for disease control and prevention. Targeted tuberculin testing and treatment for
latent tuberculosis infection. 2005
5. Carmack.A.,& Milos, M. (2017). Catheterization without foreskin retraction. Canadian Family
Physician, 36.

116
GROWTH
AND
DEVELOPMENT
 SECTION ONE
ANTHROPOMETRIC MEASUREMENTS

OVERVIEW OF ANTHROPOMETRIC MEASUREMENTS

The ideal method to determine if a neonate or child's anthropometric measurements are within
normal values is to use standardized charts (e.g., Lubchenco chart for neonates, WHO or CDC
growth charts). The fonnulas discussed in this chapter may be used to estimate anthropometric
measurements, but their usefulness are not proven in clinical studies. It is commonly used in
situations where the weight cannot be determined immediately (e.g., resuscitation of a critical
patient brought to the emergency room), and for quick assessment of a child's anthropometric
measurements. However, the authors highly suggest to use the standardized growth charts
(WHO Charts) for interpretation of anthropometric measurements.

I. AVERAGEBIRTH MEASUREMENTS
The following values approximate the average weight, length, and head circumference of
I
a Filipino infant at birth and at 1 year of age. It serves as a quick guide to assess an infant's
anthropometric measurements, but the ideal method is to check appropriate growth charts.
EXPECTED VALUES BY THE FIRST
PARAMETER AT BIRTH
BIRTHDAY
Weight 3000 g 3x the birth weight - 9-10 kg
Length 50cm 50% increase from birth length ~ 75cm
Head 35 cm
Circumference (32-37 cm)
Increase by 10 cm ~ 45 cm
Source:KliegmanR.et al. NelsonTextbookof Pediatrics
(21sted.).Elsevier;2020
Competency-Based
of Pediatrics
NavarroX, et al. Fundamentals 2014
(1sted.).C & E Publishing;

II.WEIGHT
The average weight of a Filipino newborn is 3000 g. The following are used to approximate
the average weight of a Filipino child and to quickly assess a child's weight if it is within
normal range. Growth charts are still the ideal method for assessment of nutritional status.

A. Expected Changes in Weight During the First Year of Life

, There is a 10% physiologic weight loss for normal full-term infants in the 1" week of life
, Birth weight is regained or exceeded at the 2nd week of life
An average of 30 g (1 oz)/day is gained during the 1st month of life
• In the 3rd-4th month, weight gain is approximately 20 g/day
• Birth weight is doubled at 4 months and tripled at 1 year

Sample cases
1. A full term baby is delivered with a birth weight of 3000 g. On the 5"' day of
life, his weight is 2800 g. Is this normal?

• During the first week of life, a normal full-term infant is expected to lose
approximately 10% of the birth weight (10% of 3000 g is 300 g)
• The loss of 200 g by the patient is still within physiologic levels
2. What is the expected weight at 4 months and 1 year of age if a baby was born
with a weight of2500 g?

• At 4 months, birth weight doubles= 5000 g

• At 1 year, birth weight triples= 7500 g

119
 B. Formulas to Estimate Weight

AGE

56 months Weight in grams • Age in months x 600 + Birth Weight in grams

6 to 11 months Weight in grams • Age in months x 500 + Birth Weight in grams
1 to 6years Weight in kg • Age in years x 2 + 8
• (Age in years x 7) - 5
7 to 12 years Weight in kg
2

Sample cases
1. What is the expected weight of a 5 month old with a birth weight of 3000 g?
• Weight in grams= Age in months x 600 + Birth Weight in grams
= 5 months x 600 + 3000 g
= 6000 g
• At 5 months old, the expected weight of the infant with a birth weight of 3000g is 6000 g

2. What is the expected weight of a 3 year old?

• Weightin kg= Age in years x 2 + 8
=3x2+8
= 14 kg
• The expected weight of an average 3 year old is 14 kg
• A shortcut method is to remember that at 1 year old, the expected weight is 10 kg.
Add 2 kg for each year up to 6 years old. The expected weight at 2 years is 12 kg, 3
years is 14 kg, 4 years is 16 kg, 5 years is 18 kg, and 6 years is 20 kg

3. What is the expected weight of a 10 year old?

• Weight in kg= (Age in years x 7) - 5
2
= [(10 X 7) -5]/ 2
= [70-5]/2
= 32.5 kg
• The expected weight ofan average 10 year old is 32.5 kg
Source:.NavarroX. el al. Fundamentals of PediatricsCompetency-based (1s1ed.).C & E Publishing:
2014
Parthasaralhy A. et al. IAPTextbookof Pediatrics(4thed.).NewDelhi:JaypeeBrothers:2009

lll. LENGTH
A. Expected Changes in Length or Height
The following formulas are used to approximate the average length/height of a Filipino

~r
child and to quickly assess a child's length/height if it is within normal range
Howevet; growth charts are still the ideal method for assessment

".~};;~~::-••_Fo~
0

AGE 'st!•"';.
Birth to 3
+9cm 3 cm per month
months
3-6 months +8cm 2.67 cm per month
6-9 months +5cm 1.67 cm per month
9-12 months + 3 cm 1 cm per month
2-12 years old Height in cm= Age in years x 6 + 77

120
B. Proper Measurement of Length
For Children < 2 Years Old: measure the recumbent length
Movableheadboard Ltngthboard Filedheadboard
• Use a length board or infantometer placed
on a flat surface
• Ideally taken with child's clothing and
footwear removed
• Place the child on his/her back with the
head against the fixed headboard

• Position head so that an imaginary vertical

line from the ear canal to the lower border
of the eye is perpendicular to board (i.e.,
child's eyes should be looking straight up)
• Make sure that the child lies straight along
the board, shoulders touching the board,
and the spine is not arched
I
• Hold down the legs with one hand & apply
gentle pressure to straighten the knees
• Pull footboard against the child's feet with
both soles are flat against the footboard &
toes are pointing upwards
• Read and record the measurement
• If child is <2 years old but cannot lie down,
may use standing height but add 0.7 cm
to the measurement (recumbent height=
standing height+ 0.7 cm)

For Children > 2 Years Old: measure the standing height

• Use a length board or stadiomete,; ideally

Mov,.ble
with child's clothing & footwear removed
headbo:ird • Help the child to stand on the baseboard
with feet slightly apart
• Make sure that the following points are
touching the vertical board: back of head,
shoulder blades, buttocks, calves and heels
• The child's knees and ankles may be held to
keep the legs straight and feet flat

• Position the head so that the horizontal line

from the ear canal to the lower border of
the eyes run parallel to the baseboard
• Pull down the moveable headboard to rest
firmly on top of the head
• Read and record measurement
• If the child is >2 years old but cannot stand,
may use recumbent length but subtract
0.7cm from the measurement (standing
height= recumbent length - 0.7 cm)

Source:NavarroX. et al. Fundamentals of Pediatrics

Competency-based (1sted.J.C & E Publishing;
2014
Parthasarathy A. et al. IAPTextbookof Pedialrics(4thed.).NewDelhi:JaypeeBrolhers;2009

121
Sample cases
1. What is the expected length of a 3 month old baby who was born with a length of SO cm?
• Expected length gain from birth to 3 months is 9 cm
0 Length= 50 cm+ 9 cm= 59 cm
0 By 3 months, the expected length of a baby with a birth length of 50 cm is 59 cm
2. What is the expected height of a S year old?
0 Height in cm = Age in years x 6 + 77
0 Height in cm= 5 x 6 +77 = 107 cm
0 The expected height of an average 5 year old is 107 cm

C. Expected Age per Length (or Height) Change

0 Aside from the above formula for length/height, some institutions also ask when a
child is expected to reach a certain height in clinical rounds and exams
0 The table below will help you answer these questions

AGE EXPECTED LENGTH

At birth • 50 cm or 20 inches
1 year • 1.5 x the birth length or 30 inches
2 years • 1/2 of mature adult height
3 years • 3 feet tall
4years • 2x the birth length or 40 inches
13 years • 3x the birth length or 60 inches
andChildHealth.4thed.JMCPress:2000
ol Pediatrics
Source:DelMundoF et al.Textbook

• Head circumference (HC) is measured routinely up to 3 years

• HCis an indicator of brain growth
Small HC: may suggest a small brain or microcephaly
0

(HC <2 SD below the mean)

Large HC: may suggest a large brain or macrocephaly
0

(HC >2 SD above the mean)

• How to measure: measuring tape is placed over the mid-
forehead/supra-orbital ridge and extended circumferentially
Measuring Head Circumference to include the most prominent portion of the occiput
Proper positioning of measuring tape:
Widest circumference, avoiding ears

A. Expected Head Circumference per Age

0 Formulas to calculate for the expected head circumference per age (see table below)
may vary per institution

AGE
r~~:-: CIRCUMFERENCE
----------
Expected
I ge increase in HC
1 to 4 months + 2 Inches • 1/2 inch per month
4 to 12 months + 2 Inches • 1/4 inch per month
1 to 3 years + 2 inches • 1 Inch/year
3 to 6 years + 1.5 Inches • 1/2 inch per year
6 to 20 years + 1.5 inches • 1/2 inch per 5 years
andChildHealth.4thed.JMCPress:20C0
of Pediatrics
Source:DelMundoF el al.Textbook
122
8. Using Growth Charts
0 To interpret if measured head circumference is within normal, refer to growth charts
' Aside from WHO growth charts, this head circumference chart is commonly used in:

HEAD CIRCUMFERENCE (BOYS)

Ct,1 IN
I I 'I
62 I I
I 24
GO I
I I
58
- _L -
-~..... ,___- .,I_. .,._f- 23

60 I I I
I
I I. ....
---r- ,- 22

64
:
I
I
I
!
!

. r-r: ~--
,.. I
I
- 21
52 l""'l_.. ~-- I
,,, 1.1

-- ..
I
j_..1-- -t I
_..._20

I
I ;a,i·

___, ~•"~-
60
.;, ""' ·r

, I..""''-----
I
I 10
II I I I I I
I -2~12'1
•• - I I I
,...10
... //° I .. --- I
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I
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I

./A ,.,,,,;.-·:
~--- j
I I I I ~
17
42 I I

,o I /, • ~' I I I f- 16

38/ 'I',, I I
I
I 15
,· I I '
/2
I
I I
36
34 I
'---'-- --
i
I
I
I
I - 14
i I I I
32 / I i I '
13

30
A------•
I
__ 10
i
~~Hs14__ 1_•_1_a ____
+
, ______
1
I

a ve~s
r
12 14 16
r

10
12

HEAD CIRCUMFERENCE (GIRLS)

CMr---.--,----.-----.--~,~-~~-~--~-,~--.-~~-,~--.-~~~~ IN

62 t---+-~-+---+-.,__-"---,---+--i-+-.......J'c.+-++-+--+-1-+'-+-+-!-+-++--1
I I I
601---t-----+---+-+----t---+---l-+-+-<'-+-++-....+-..-+-+--t-++--1--t,-J
-H ._....,_
....,_I➔: l-t-++-1-1-1--1-24
58 ~ -l- -+---+-j-+-_1 __ /_,._-+---,--+--,..--<-+-+-+-+-+-+ -+-+-t-t-+-1--r-+-I- 23
1~-+---f-+-if-++- 1
5<l ,__,..._i,__,..
__, -+-- ·++--1--~-22
r-'-'+..:.'-+-+--f..,,.--,·9-
~t---t--,---+----+ \ I i-+-+--1-,',-,-,-+-~~~r''-t-,t-t-+-~--~

!
6<1,--,...,-..~-+- ~~~1!!.1t'"·fl~·i~-1·,~t:11j'f-~:..
62 t--t--'-t-..,...--.._l+-_1~~l::i~~~11~-.1 I ..-- ➔f;;tfJ:"rt3-
21
t 2 1..- -~

,8 I.~....!1-,~""-++-+--1.,....,,.-f--+--t-++-!-+-1
, _!___ i__
46
rr-r
1 ·-:::;;_~,t::±
71,--1~-+::;;
..
:;-.
-
1 1:::!l=1i- .tlj:;:.::1.:t
1-1::.t,,1•~-.:1:.t• 1J,jtt.:tj•'
9

~
I /'
I , _•.-
,,,,,
~--,
.........-i
l .... -· I
~---'r-r-·'
1 ,

i I
I
,
f
I I
18

17

} '%,_/-r ,
42 •'' ~ , .. , I I

1 l : , : ,,: '
I I I

I
16

: :!
I -15
:~/, I i : I ! I : : I
3,ll j I 1 I I '. I
I
I
14

I H-++-+-1--t-t-+-i:f-+l-+-+-f-+l-+I + 13
32 I I
I I
30'---'--'--'-'-'-'---'-'-'--'--~-'--'-'-~-'-'-'-_,_...,_.,_,'--"--'--'-''--"---"---'-'-'-'----'-'-'-'----'-'-''--'
I I I
12
10 12 14 16 18 8 10 12 14 16 18
B-------- MO: THS --------i------YEARS -----

Source:MenkesJ, el al.ChildNeurology
(7thed).Lippincott
Williams& Wilkins;2006

123
V. MID-UPPERARM CIRCUMFERENCE
(MUAC)
Measuring the Mid-Upper Arm Circumference
• In infants 6-59 months old, MUACis an alternative method
commonly used to determine wasting (acute malnutrition)
• Procedure
0 Place measuring tape from the tip of shoulder to the elbow
0 Mark the mid-point between the shoulder and the elbow
0 Measure the circumference at this mid-point, making sure that
correct tape tension is placed (not too tight and not too loose)
0 Record and interpret MUAC
• Interpretation ofMUAC
0 115 to <125 mm= moderate acute malnutrition
0 <115 mm = severe acute malnutrition
Source:MwangomeM,et al. BulletinOfTheWorldHealthOrganization;2012
WHO,Management of severeacutemalnutrition
in infantsandchildren
WHO,Supplementary
foodsfor the management of moderateacutemalnutrition

Sample cases
Using the formulas described above, compute for the estimated weight and length/height
Case 1: A 3 year old with a birth weight of 3 kg and birth length of 50 cm

• Weight
0 Weight in kg= Age in years x 2 + 8
0 Weight = 3 x 2 + 8
, Weight= 14 kg

• Height
0 Height in cm= Age in years x 6 + 77
0 Height= 3 x 6 + 77
0 Height= 95 cm

• Conclusion: At 3 years old, the patient's expected weight is 14 kg and expected height is 95 cm

Case 2: A 7 month old with birth weight of 3 kg and birth length of 50 cm

• Weight
0 Weight in grams = Age in months x 500 + Birth Weight in grams
0 Weight= (7 x 500) + 3000
0 Weight= 6500 grams

• Length
0 Birth to 3 months=+ 9 cm
0 3 to 6 months = + 8 cm
0 6 to 7 months=+ 1.67 cm
0 Total gain in length= 18.67 cm
0 Length= 50cm + 18.67cm = 68.67 cm

• Conclusion: At 7 months old, the expected weight is 6500 g and the expected length is
68.67 cm

124
INTERPRETATION OF ANTHROPOMETRIC MEASUREMENTS
• Interpretation of anthropometric measurements using the WHO Growth Charts are discussed
in this section (this is the ideal method of interpreting a child's weight & height/length)
• The step-by-step approach for interpretation is illustrated through the sample cases
provided below (please see WHOgrowth charts available 011/ine:www.who.int/
childgrowth/standards/en)

Sample Case 1: Plot the anthropometric measurements of a 1 year and 2 month-old

male with weightof 10 kg and length of75 cm

STEP 1: Choose the appropriate chart

• Use the appropriate chart for the patient's age, gender, and parameter to be interpreted
(ex. weight for age, length for age, weight for length)
• For this patient, the appropriate growth charts are weight-for-age boys from birth to 2 years, IJ,·,_
length-for-age boys from birth to 2 years, and weight-for-length boys from birth to 2 years
(see succeeding pages for chosen charts)

STEP 2: Plot the measurements in the chart

• Plot the variables in the X and Y axis of the chart
In general, the age is plotted based on completed months/years on a vertical line. (e.g.
if a child is 4 ½ months old, it is plotted at 4 months and not in between 4 and 5 months)
All other parameters are plotted as precisely as possible (may plot in between lines)
• Mark the plotted point (intersection of the lines extended from the X and Y axis values)
• For our sample case, see plotted dots on the charts shown in the succeeeding pages

STEP 3 : Determine the Z-score

• Read the Z-score based on the plotted point
For Z-scores between 1 to -1, this is read as Z-score = 0 or median
Plotted points above this range are read as "above"
Plotted points below this range are read as "below"

Z Score INTERPRETATION*
Between 1 to -1 • 0 (median)
Between 1 to 2 • Above 1 (Z-score >l)
Between 2 to 3 • Above 2 (Z-score >2)
Higher than 3 • Above 3 (Z-score >3)
Between -1 to -2 • Below -1 (Z-score < -1)
Between -2 to -3 • Below -2 (Z-score <-2)
Lower than -3 • Below -3 (Z-score <-3)

*If the point is exactly on a z-score, it is interpreted in the less severe category (e.g., plotted
point falls exactly on -3, interpret as below -2)

125
STEP 4: Interpret
• Interpret the Z-score using the table below
• Note that wasting is the most appropriate measure of severe acute malnutrition
PARAMETERS INTERPRETATION
Normal • 0 to below -1
• Above 1 (better assessed with
Risk for Overweight
Weight for age weight for length/height or BMI)
Underweight • Below-2
Severely underweight • Below-3
Normal • Below -1 to above 2

Height/Length for Tallness • Above 3

age Stunting • Below-2
Severe Stunting • Below-3
Normal • 0 to below-1
Wasting • Below-2
Weight for height
Severe Wasting • Below -3
(forchildren <Syearso/d)
Overweight • Above 2
Obesity • Above 3
Normal • 0 to below -1
BM! forage Wasting • Below -2
{children 5-19 years aid)
Severe Wasting • Below-3
= Weight (in kg)
Height (in m) 2 Overweight • Above 2
Obesity • Above 3

Source:PPSPreventive
PediatricHealthCareHandbook
2018

Weight-for-age for Boys: Birth to 2 years (Z score)

~fl -Id~l"_J!
Hf-:L__
T_:r_-__l~J_1
~ ; ~ ~-: '

-J_L : -~
-r L

The given weight of 10 kg intersects with the given age of 14 months, yielding a z-score of 0
(median). This means he has a normal weight in relation to his age.

126
Length-for-age for Boys: Birth to 2 years (Z-score)

I 1
-·r-i-r-~ i 1

i
i

, I
.
r -,.
.
•
t
II
t
-{- -~
' I
The given length of 75 cm intersects with the given age of 14 months, yielding a z-score of 0
(median). This means he has a normal length in relation to his age.

Weight-for-length for Boys: Birth to 2 years (Z score)

-·-~t-
__ ,·'.-:f-=r-
•
, ·-·--•--~
♦ • • :
. -
~
.2
3

• 1

0
-1
·,2

.:~:=:
.··
......
,·
-3
I .
- ... .L.... .. .. . . ·- . 1 -

:r:;' . '

~ r· · I

The given weight of 10 kg intersects with the given length of 75 cm, yielding a z-score of 0
(median). This means he has a normal weight in relation to his length.

Source:TheWHOChildGrowthStandards.Retrievedfromhltp:l/www.who.inV
WorldHeallhOrganization.
TrainingCourseon ChildGrowthAssessmentGeneva,WHO,2008.

127
 Sample Case 2: A 2 year old male was brought to your clinic for a general check-up.
Weight= 7 kg Length= 85 cm

STEP 1: Choose the appropriate chart

• For this patient, the appropriate growth charts are weight-for-age boys from birth to 2 years,
length-for-age boys from birth to 2 years, and weight-for-length boys from birth to 2 years

STEP 2: Plot the measurements in the chart

STEP 3: Determine the Z-score

• Weight for age: Z-score <-3
• Length for age: Z-score O = median
• Weight for length: Z-score < -3

STEP 4: Interpret
• Interpret the Z-score using the table shown above
Weight for age: Z-score < -3 (interpretation: severely underweight)
• Length for age: Z-score O = median (interpretation: normal)
• Weight for length: Z-score < -3 (interpretation: severely wasted)
• Based on the growth charts, the child is severely wasted. His length is within normal range.

Weight-for-age for Boys: Birth to 2 years (Z score)

i .
t j.
i ;
! -···-
I-+t i~-
--f-!-'--'-

i TT-'.

The given weight of 7 kg, with the given age of 2 years, yields a z-score below -3.
This means he is severely underweight for his age.

128
length-for-age for Boys: Birth to 2 years (Z-score)

I
The given length of 85 cm with the given age of 2 years yields a z-score between O and -2,
which still falls within the median (see table under Step 3). This means he has a normal
length in relation to his age.

Weight-for-length for Boys: Birth to 2 years (Z score)

The given weight of7 kg with the given length of85 cm yields a z-score below-3.
This means he has severe wasting (see table under Step 4).

Source:TheWHOChildGrowthSlandards. Retrieved
fromhttp://www.who.in1/
WorldHealthOrganization.
TrainingCourseon ChildGrowthAssessment.Geneva,WHO,2008.

129
 SECTION TWO
DEVELOPMENT

INTRODUCTION
I. PRINCIPLESOF DEVELOPMENT
• Development is a continuous process of reaching maturity with increase in function and skills
• Developmental sequence is orderly and predictable
0 Progresses in a cephalocaudal manner and proximodistal pattern
0 Responses proceed from generalized reflexes to discrete voluntary actions
• Rate varies from child to child and is affected by biologic and psychosocial factors

II. DEVELOPMENTALDOMAINS
• The developmental domains consist of gross motor, fine motot; social, and language
(expressive and receptive)
• Developmental milestones are present in each domain that serve as criteria in deciding
whether a child is developing accordingly
Developmental delay refers to a slower than acceptable rate of development in at least 1
of the domains

Ill. CLASSICSTAGETHEORIES OF DEVELOPMENT

The development of children can be described as a series of stages in relation to their age
as shown in the table below
• Psychologists have identified different stages based on psychosexual, cognitive, psychosocial,
and moral development

INFANCY TODDLER PRESCHOOL SCHOOL AGE ADOLESCENCE

(0-1 year) (1-3 years) (3-6 years) (6-12 years) (12-20 years)
1) Freud (Psychosexual Development)
• Phallic/ • Latency • Genital
• Oral • Anal
Oedipal
2) Piaget (Cognitive Development)

• Concrete • Formaloperations
• Preoperational
(abstract thinking,
[symbolic operations
• Sensorimotor (thinking is tied deductive
thinking; able ( use of logic
to immediate sensations) reasoning&
to form mental and inductive hypothetical
images) reasoning) thinking)
3) Erikson (Psychosocial development)
• Trust • Autonomy • Industry • Identity versus
• Initiative
versus versus shame versus role diffusion
versus guilt
mistrust and doubt inferiority
4) Koh/berg (Moral Development)
• Preconventional
• Conventional • Conventional • Postconventional:
(stage 1 & 2):avoid
(stage 3): (stage 4): law moral
punishment/
conformity and order principles
obtain rewards
Source:KliegmanR.el al NelsonTextbookof Pediatrics(21sted}.Elsevier:2020
NavarroX, et al. Fundamentals
of PediatricsCompetency-based (1sted}.C&EPublishing: 2014

130
DEVELOPMENTAL MILESTONES
• The actual age at which a milestone is achieved by a normal child may range within 2
months of this age level in the first year of life to 4 months from the second year onwards

I. DEVELOPMENTAL MILESTONES DURING THE NEONATAL PERIOD

DOMAIN SKILLS
• Arms and legs are flexed while prone
• Head held below the body while in ventral suspension
Gross motor • Arms and legs semi-flexed while in supine position
• Head lags when pulled to sit
• Legs extended when held upright

Fine motor

Language
• Presence of grasp reflex
• Hands remain fisted
• Sweeping movements towards object are observed
•Crying/ whimpering (expressive language)
I
"

• Startled by loud sound (receptive language)

Personal-social • Gaze at faces, bright lights, and colored objects
Source:KliegmanR,et al NelsonTextbook
of Pediatrics
(21sted).Elsevier:
2020

II. DEVELOPMENTAL MILESTONES DURING THE INFANCY PERIOD

DOMAIN AGE SKILLS
3 months • Hold head without head lag
5 months •Rollover
Gross motor 7 months • Sit (6 months with support, 8 months without support)
9 months • Pull himself/herself to stand
12 months • Walk independently
3 months • Observed to have unclenched hands
5 months • Hands are brought to mid line
Fine motor 7 months • Transfer object from one hand to another
9 months • Pincer grasp
12 months • Voluntary release of objects/ able to throw objects
3 months • Alert to human voice
6 months • Localize to sound
Language
9 months • Understand and follow the command "no"
12 months • Follow 1-step command with gesture
2 months • Social smile

3 months • Coo
Personal-social 6 months • Babble
9 months • Say "mama/papa" but non-specific
12 months • Speak single words with meaning

Source:KliegmanR,et al NelsonTextbook
of Pediatrics
(21sted).Elsevier:2020
NavarroX et al. Fundamentals
of Pediatrics
Competency-based (1sted).C & E Publishing:
2014

131
III. DEVELOPMENTAL MILESTONES FROM TODDLERHOOD TO SCHOOL-AGE
DOMAIN AGE SKILLS
15 months • Run, pivot, walk backwards, and crawl upstairs
18 months • Walk upstairs with rails and run stifny
24 months • Jump with both feet and run well; climb on furniture; walk up
and down the stairs 1 step at a time

• Jump forward and pedal a tricycle; goes upstairs with

Gross motor 30 months
alternating feet
3 yea,·s • Ride a tricycle

4 years • Hop; throws ball overhead; use scissors to cut out pictures
5 yea,·s • Skip
7 years • Climb and run
15 months • Scribble spontaneously; make tower of 3 cubes
18 months • Imitate stroke on paper; make tower of 4 cubes
24 months • Imitate vertical lines; make tower of 7 cubes
• Draw circle with series of perseverating lines; make tower of
30 months
9 cubes

Fine motor 3 years • Draw circle and a person with 2 body parts; make tower of 10 cubes
3.5-4 years • Draw a cross

4-4.5 years • Draw a square

5 years • Draw a triangle
6 years • Copy letters but reverse some ofit; draw a person with 12 parts
7 years • Know right and left sides
24 months • Two-word phrases; follow 2-step commands; know full name
3 years • Speak 3-4 worded telegraphic sentences; understand prepositions
• Speak in complete sentences, tells story; understand concept
4 years
Language of size
• Understand concept of time; follow 3-step commands; pronounce
5 years
most of the sounds of the English alphabet except F,V,S, Z
6 years • Able to verbalize emotions; follow 3-serial commands
• Cooperate when being dressed by putting out limbs; egocentric
12 months
symbolic play; play simple ball game
15 months • Indicate some desires by pointing; imitate chores; hug parents
18 months • Feed self; seek help when in trouble; may complain when wet/soiled
• Handle a spoon well; parallel play; toilet-trained by day; listen
24 months to stories when shown pictures; often tell about immediate
Personal- experiences
social
30 months • Help in simple tasks (e.g., like putting things away}; pretend in play
3 years • Dress under supervision; wash hands
4 years • Dress independently/correctly; group play; go to toilet alone

• Do simple errands and help in household chores; ask questions

5 years
about meaning of words; engage in domestic role·playing

6 years • Dress up completely; tie shoelaces

132
SECOND YEAR OF LIFE TO MIDDLE CHILDHOOD
3-5 YEARS OLD 6-11 YEARS OLD
2YEARSOLD
(PRESCHOOL YEARS) (MIDDLE CHILDHOOD)
Growth
• Growth rate slows • Gain of 3-3.5 kg and 6-7 cm per year
down by the end of • Head grows only 2-3 cm during
the first year • Weight gain of about 2 this period
• 2"'1 year of life kg per year • Dentition:
0 3-5 lbs weight gain • Growth of about 6-8 0 6 years old: loss of deciduous teeth
0 10-12 cm increase cm per year 0 9 years old: 8 permanent
in height incisors & 4 permanent molars
0 90% of adult head
size attained
Other Aspects
• Appetite declines
0 11-12 years old: pre-molars erupt

• Hypertrophy of lymphoid tissues

I
.

'

• Interest in sexuality
(physiologic anorexia) • Muscle strength, coordination, and
(may play with their
• Myelination ofd1e brain stamina increase progressively
genitals as a sign of
is most rapid & dramatic • Issues of overweight from
normal curiosity)
• Protuberant abdomen, sedentary habits
• Make-believe games
baby fat, lordosis • Social:
• May make up stories
• Emotional: temper 0 Attachment to parents decrease &
• Encourage child to
tantrums; seeks to peer increase by 7 years of age
sleep in own bed
independence but still 0 Reliance on peer groups
• Teach the child not to
relies on parental ties 0 Group loyalty, commitment to
talk to strangers
for confidence best friends by 9 years old
Source.KltegmanR, et al NelsonTextbookof Ped1atncs (21sted). Elsevier,2020
NavarroX el al. Fundamentals
of PediatricsCompetency-based (1st ed). C & E Publishing;2014

ADOLESCENCE
I. GENERAL
• Adolescence is the transition from childhood to adulthood
• Rapid physical and sexual changes:
Growth spurt
Sexual maturation (appearance of secondary sex characteristics)
Menarche (event to indicate fertility)
Ejaculation (comparative indicator of reproductive potential)

II. LINEAR GROWTH

• Linear growth during puberty accounts for 20% of the final adult height
• By the time menarche has occurred, girls would have attained 90-95% of their adult height
• Boys undergo a growth spurt in mid-puberty or about 2 years later than girls

A. Predicted Final Adult Height

0 The mid parental height is a good estimate of predicted final adult height
° Formula for calculating mid parental height (in cm) in children:

(Father's height- 13 cm)+ Mother's height

For girls
2
(Father's height+ 13 cm) + Mother's height
For boys
2

B. Bone Age
0 An index of physiological maturation
Radiographs of the hand, wrist, or knee are compared to the standards of maturation
in a normal population
Any value >2 SD above or below the mean for age is considered abnormal
133

Adipose tissue
Average gain in height
Muscle mass
Peak height velocity
Peak weight velocity
Rate of linear growth
• Increases (to 25% of body
• Decreases
weight)
• 28 cm • 25 cm
• Increases from 80 to 90% • Decreases from 80 to 75%
of body weight of body weight
• 13.S years • 11.S years
• Occurs with peak height • Occurs 6 months after
velocity (13.S years) peak height velocity
• 9-10 cm per year • 8-9 cm per year

Timing of growth • Mid-puberty (SMR 3-4) • Early puberty (SMR 2-3)

spurt* • Average: 11.5 years • Average: 10 years

·SMR= SexualMaturityRating(referto partIll below)

Source:NavarroX et al. Fundamentals (1sted). C & E Publishing;2014
of PediatricsCompetency-based

III. SEXUALMATURITYRATING
SMR
ILLUSTRATION PUBIC HAIR PENIS TESTES 0
STAGE

1 y J
• None • Preadolescent • Preadolescent

2
y J
• Scanty, long
• Slightly
pigmented
• Slight
enlargement
• Enlarged
scrotum
• Pink texture
altered

3
y !
• Darker
• Starts to curl
• Small amount
• Longer • Larger

4 y !
• Resembles
adult type
but less in
quantity
• Coarse, curly
• Larger
• Glans and
breadth
increases in
size
• Larger
• Scrotum dark

y
• Adult
distribution
• Spread to
5 • Adult • Adult
the medial
! surface of
thighs

134
 SMR
ILLUSTRATION PUBIC HAIR BREAST
STAGE

1
(y);f~(0 • Preadolescent • Preadolescent

• Sparse • Breast and

2
(y)ft~ (0 • Lightly
pigmented
• Straight
• Medial border
oflabia
papilla elevated
as small mound
• Areolar
diameter
increased
I
\y/;t~((/
• Darker • Breast
• Beginning to and areola
3 curl enlarged
• Increased • No contour
amount separation

lr/A{O
• Coarse, curly • Areola and
• Abundant but papilla form
4
amount less secondary
than in adult mound

• Mature

ITIW<~~
• Adult feminine
• Nipple
triangle spread
projects
5 to the medial
• Areola part of
surface of
general breast
thighs
contour
Source:KhegmanR. et al NelsonTextbookof Ped,atncs
(21sted).EIsevier·
_ , 2020
Navarrox et al. Fundamentals
of PediatricsCompetency-based (1sted).C & E Pubhsh,ng;2014

BOYS GIRLS
• Sequence of pubertal events: thelarche,
adrenarche, peak height velocity,
• Complete sexual maturity is usually
continued breast and hair development,
achieved by 17-18 years old
menarche, completion of puberty
Sequence of pubertal events: testicular
, Average duration of completion of
enlargement, adrenarche, continued
puberty is 4 years
testicular and penile enlargement, peak
• Menarche is achieved by 90% of girls
height velocity with peak weight velocity
during SMR breast 4
• Gynecomastia is seen in about 60% of
, Filipino girls' average age of menarche
boys and is noted at SMR 2-4
is 13.3 years and cycles may be
• Ejaculation usually occurs at SMR 3
anovulatory for 2 years after menarche
• Voice change noted between SMR 3-4
Full fertility usually reached within 2
• Fertility is usually established at SMR 4
years of menarche (average age
between 14-15 years)

135
IV. CHARACTERISTIC BEHAVIORS OF ADOLESCENTS
EARLY ADOLESCENCE
(10-13 Years Old}
1)Autonomy
• Argumentative, disobedient,
and tends to challenge family
and authority
• May have mood swings;
daydreams
• Rejects things of childhood
• Displays a growing need for
privacy
2) Body Image
• Critical of and preoccupied
with physical appearance
and changes
• May be anxious about wet
dreams, masturbation,
breast or penis size
3) Cognitive Development
• Focuses thinking on "here
and now" because of a
lack of understanding
of consequence despite
concrete thinking
• Need for instant gratification
and a lack of impulse control,
thus engages in risk-taking
behavior
4) Identity Development
• Asks the question "Am I
normal?"
• Emerging sexual feelings and
exploration
• Magnifies own problems
and thinks that "no one
understands"
SJ Peer Group
• Development of intense
friendships with the same sex
• Contact with opposite sex in
peer groups
6) Vocation
• Resets priorities for social
life rather than study
• May have unrealistic
academic goals and
frequently changes them
MIDDLE ADOLESCENCE
(14-17 Years Old)
• Shows ambivalence on
emerging independence
which may lead to family
conflicts
• Increased interest in making
body image more attractive
• Excessive physical activity
alternating with lethargy can
be shown
• Emergence of formal
operations that lead to
effective planning
• Understands better the
consequences of behavior
• Feelings of omnipotence
and immortality are present
which may lead to risk-
taking behaviors
• Experimental (drugs,
alcohol, sex)
• Increased intellectual ability
and creativity
• Formation of strong peer
allegiances
• Susceptible to peer pressure
• Fad behavior
• Emerging sexual drives lead
to exploration and ability to
attract a partner
• Able to set more realistic
academic or vocational goals
• Begins to realize strengths
and limitations
136
LATE ADOLESCENCE
(18-21 Years Old}
• Emancipation (college, work,
vocations, adopts an adult
lifestyle)
• Usually comfortable with
body image
• Emergence of abstract
reasoning
• Able to compromise as they
are able to engage with their
conscience
• Able to delay gratification
• Relates to family as adults
• Established ethical and
moral value system
• Realizes own limitations and
mortality
• Established sexual identity
• More capable of intimate
relationships
• Makes decisions and
maintains values as they are
less influenced by peers
• Relaxes to individuals more
than to peer group
• Selects partner based on
individual preference
• Pursues realistic academic/
vocational goals with
training or actual
employment
 SECTION THREE
BEHAVIORAL DISORDERS

AUTISM SPECTRUM DISORDER (ASD)

I. ETIOPATHOGENESIS
• As much as 15% of ASDare associated with a known genetic mutation
• Males are more commonly affected than females (4:1)
• The best established prognostic factors for individual outcome are
Presence or absence of associated intellectual disability
, Language impairment

II. MANIFESTATIONS
• Symptoms are typically recognized and observed during the second year of life
• Deficits in 2 core domains: social communication/social interaction AND restricted
repetitive patterns ofbehaviot; interests, and activities
I
Ill. DIAGNOSTIC CRITERIA

A. Persistent deficits in social communication and social interaction

1. Deficits in social-emotional reciprocity
Abnormal social approach
Failure of normal back-and-forth conversation
Reduced sharing of emotions, interests, or affect
Failure to initiate or respond to social interactions

2. Deficits in nonverbal communicative behaviors used for social interaction

Abnormal eye contact and body language
Deficit in understanding and use of gestures
Total lack of facial expressions

3. Deficits in developing, maintaining, and understanding relationships

Absence of interest in peers
Difficulties in making friends

B. Restricted, repetitive patterns of behavior, interests, or activities as manifested by at

least 2 of the following
1. Stereotyped or repetitive motor movements, use of objects, or speech
2. Insistence on sameness, inflexible adherence to routines, or ritualized patterns of
verbal or nonverbal behavior
3. Highly restricted, fixated interests that are abnormal in intensity or focus
4. Hyper or hypo-reactivity to sensory input or unusual interest in sensory aspects of
the environment

C. Symptoms must be present in the early developmental period

D. Symptoms cause clinically significant impairment in social, occupational, or other

important areas of current functioning

E. Disturbances are not better explained by intellectual disability or global developmental

delay. Intellectual disability and autism spectrum disorder frequently co-occur

Source:APADiagnosticandStatisticalManualof MentalDisorders.5th Ed.AmericanPsychiatric

Association;
2013

137
ATTENTION-DEFICIT/HYPERACTIVITY DISORDER
I. ETIOPATHOGENESIS
• Characterized by difficulty paying attention, excessive activity, and acting without regards
to consequences, which are otherwise not appropriate for a person's age
• Males are more commonly affected than females
• Exact cause us unknown in the majority

II. DIAGNOSTICCRITERIA
A. Persistent pattern of inattention and/or hyperactivity-impulsivity
1. Inattention & disorganization: ~6 of the following persisting for at least 6 months
Fails to give close attention to details or makes careless mistakes in school
Difficulty sustaining attention in tasks or play activities
Does not seem to listen when spoken to directly
Does not follow through on instructions and fails to finish schoolwork
Difficulty in organizing tasks
Avoids, dislikes or is reluctant to engage in tasks that require sustained mental effort
Loses things necessary for tasks
Easily distracted by external stimuli
Forgetful in daily activities
2. Hyperactivity-impulsivity: ~6 of the following persisting for at least 6 months
Fidgets with hands or feet or squirms in seat
Leaves seat in classroom
Runs about or climbs excessively
Difficulty playing or engaging in leisure activities quietly
"On the go"
Talks excessively
Blurts out answers before questions have been completed
Difficulty awaiting turn
Interrupts or intrudes on others
B. Symptoms are present prior to age 12 years
C. Symptoms are present in ~2 settings (e.g., at home, school, or work; with friends or
relatives; in other activities)
D.Symptoms interfere with, or reduce the quality of, social, academic, or occupational functioning.
E. The symptoms do not occur exclusively during the course of schizophrenia or another
psychotic disorder and are not better explained by another mental disorder
Source:APADiagnosticandStatisticalManualof MentalDisorders,
5th Ed.AmericanPsychiatric
Association;
2013

REFERENCES
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders,
Fifth Edition. Arlington, VA,American Psychiatric Association, 2013.
2. Engorn, B., and Flerlage, J. The Harriet Lane Handbook 20th ed. 2015. Philadelphia: Elsevier.
3. Hazinski, MF. Manual of Pediatric Critical Care 1st ed. 2009. Elsevier.
4. Hazinski, MF. Nursing Care of the Critically Ill Child 3rd ed. 2013. Elsevier.
5. Kliegman, R.,Stanton, B.,St. Geme,J., Schor, N.,and Behrman, R..Nelson Textbook of Pediatrics
21st ed. 2020. Canada: Elsevier.
6. Mwangome, M.,Fegan, G.,Fulford, T.,Prentice.A., and Berkley,). Mid-upper arm circumference
at age of routine infant vaccination to identify infants at elevated risk of death: a retrospective
cohortstudyin the Gambia. Bulletin OfThe World Health Organization, 2012 90(12), 887-894.
doi: 10.2471/blt.12.109009
7. Navarro,X., Bauzon,A.,AguilatJ., and Malanyaon, 0. Fundamentals of Pediatrics Competency-
based 1st ed. 2014. Quezon City: C and E Publishing, Inc.
8. Parthasarathy A, Menon PSN, Agarwal RN, Choudhury P,Thacker NC,Ugra D.!AP textbook of
pediatrics 4th ed. 2009. New Delhi: Jaypee Brothers
9. World Health Organization. Training Course on Child Growth Assessment. Geneva, WHO,2008.
10. The WHOChildGrowth Standards. Retrieved from http:j /www.who.int/childgrowth/standards/en/
138
 PEDIATRICS
PREVENTIVE
 SECTION ONE
IMMUNIZATION

OVERVIEW OF IMMUNIZATION
• Ultimate goal: eradication of disease
• Immediate goal: prevention of disease

I. TWO TYPES OF IMMUNIZATION:ACTIVEAND PASSIVE

ACTIVE IMMUNIZATION PASSIVE IMMUNIZATION
• Exposure to antigen leads to
• Transfer of humoral immunity in the
immunity through creation of
form of antibodies
antibodies by the recipient

Definitions
• Natural: person contracted the
disease and the immune system
creates antibodies
• Artificial: all or part of the
• Natural: transfer of maternal antibodies
to the fetus via the placenta

• Artificial: high levels of pathogen- or

I
microorganism are introduced to toxin-specific antibodies are given to
stimulate creation of antibodies non-immune persons

• Congenital/acquired 8-cell defects

alone or in combination with other
immunodeficiencies
• Vaccination aims to induce, without
• When time does not allow adequate
harm to the recipient, protective
protection by active immunization alone
immune responses against an attack
(e.g.,rabies, hepatitis 8)
of the natural infection
• When a person susceptible and with
• Once immunized, the individual
Indications high risk of complications is exposed to
is less likely to be a source of
a disease (e.g., child with leukemia and
infection thereby reducing onward
exposed to varicella)
transmission to susceptible people
• When a disease is already present and
in the community
administering antibodies may help
• See specific vaccines below
in suppressing its toxin effects or the
inflammatory response (e.g., tetanus,
Kawasaki)

II. TYPES OF ANTIGENFORACTIVEIMMUNIZATION*

LIVE ATTENUATED VIRUS OR INACTIVATED VACCINE
BACTERIA (WEAKENED) (KILLED MICROORGANISMS)
• Hepatitis B vaccine
• BCGvaccine
• DTwP or DTaP or Tdap
• Measles vaccine
• H. influenzae b vaccine
• MMRvaccine
• Pneumococcal vaccine
• Varicella vaccine
• Hepatitis A vaccine
• Rotavirus vaccine
• Meningococcal vaccine
• Influenza attenuated vaccine (intranasal)
• Influenza vaccine (IM)
• Typhoid fever (oral) vaccine
• Human papillomavirus vaccine
• Oral polio vaccine
• Typhoid fever vaccine (IM)
• Dengue tetravalent vaccine
• Rabies vaccine
• Oralcholera vaccine
• Inactivated polio vaccine (IPV)
• Yellow fever vaccine
• Cholera vaccine
• Japanese b encephalitis vaccine
• Japanese b encephalitis vaccine
'Some vaccineshave both inactivatedand live forms dependingon the manufacturer(e.g., Japaneseb
encephalitis,
influenzavaccine,typhoidfevervaccine)

141
III. IMPORTANTPOINTS TO REMEMBER

• For IM injections. the choice of site is based on the volume of the injected material and the
muscle mass
• In children younger than 1 year of age, the anterolateral aspect of the thigh is the
preferred site.
• In children older than 1 year, the deltoid muscle is usually large enough for IM injection.
• The upper, outer aspect of the buttocks should not be used
Source:KliegmanR, el al NelsonTextbook
of Pediatrics
{21st ed).Elsevier:2020
Kimberlin
DW.et al. ReportoftheCommittee
on Infectious Diseases.
American Academy of Pediatrics:
2018

PRINCIPLES OF IMMUNIZATION
I. SIMULTANEOUSADMINISTRATIONOF MULTIPLEVACCINES
• Most vaccines can be safely and effectively given simultaneously
• Use separate syringes and sites
• Exception is yellow fever and cholera vaccine (separate by at least 3 weeks)
• If vaccines are not given simultaneously, the recommended minimum interval varies
depending on type of vaccine:

2: 2inactivated vaccines, or • Can be given simultaneously or at any interval

Inactivated and live vaccine combinations between doses

• Can be given simultaneously

Two parenteral live vaccines • If not given simultaneously, interval of at least
4 weeks

II. LAPSED IMMUNIZATIONS

• A lapse does not require reinstitution of the entire series
• Subsequent immunizations should be given at the next visit as if the usual interval had elapsed

Ill. UNKNOWN OR UNCERTAINIMMUNIZATIONSTATUS

• In general, immunizations should be initiated without delay on a schedule commensurate
with the person's current age
• No evidence that giving vaccines to already immune recipients is harmful

IV. CONTRAINDICATIONSTO VACCINATION

TWO TEMPORARY
TWO PERMANENT CONTRAINDICATIONS TO LIVE
CONTRAINDICATIONS VACCINES BUT NOT WITH
INACTIVATED VACCINES
• Anaphylactic reaction • Pregnancy
• Encephalopathy not due to another • lmmunosuppression
identifiable cause occurring within 7
days after pertussis vaccination
Source:Kimbe~inDW,el al.AmericanAcademyof Pediatrics;2018
Redbook:
2018-2021
Reportof the Committee
on InfectiousDiseases,31stedilion

V. PRE-EXPOSUREVERSUS POST-EXPOSUREPROPHYLAXIS
• Consists of a course of vaccination given prior to an
Pre-Exposure Prophylaxis (PrEP)
exposure

• Preventive medical treatment given after an exposure

Post-Exposure Prophylaxis (PEP) or suspected exposure to a pathogen in order to
prevent the infection from occurring

142
2019 CHILDHOODIMMUNIZATIONSCHEDULEOF THE PHILIPPINES

MINIMUM TIMING OF
VACCINE ROUTE DOSE/NOTES
AGE ADMINISTRATION
• Birth (or preferably
within first 2 months)
• Healthy >2 months old,
PPD prior to BCGnot
necessary
• PPD is needed prior to
Bacillus • 0.05 mL <12 months
BCGif any is present:
Calmette- ID Birth • 0.1 mL >12 months
congenital TB, history
Guerin (BCG)
of close contract to
known cases, and
clinical symptoms
suggestive of TB

I
and/or chest. X ray
suggestive ofTB
• Birth, 6-10-14 weeks
• See discussion on
Hepatitis B IM Birth • Booster may be given
succeedingpages
based on titers
• 5 th dose not needed
if 4'" dose given at 4
• Primary series: 6-10-
years old or older
14 weeks
• DTwP-Hib-Hep B
DTwP-Hib-HepB • Boosters given at 12-
(given usually in health
or IM 6 weeks 18 months (interval
DTaP-Hib-lPV centers; "w" in DTP
between 3 rd and 4 1h
stands for whole cell)
dose is 6 months) and
• DTaP-Hib-lPV (in
at 4-6 years old
institutions / clinics;
"a" stands for acellular)

• Given if pentavalent
• Primary series: 6-10- vaccine DTwP-Hib-
Oral Polio
Oral 6 weeks 14 weeks HepB was used
Vaccine
• Booster at 4 years old • IPV is given with
3rd OPV dose
• Healthy children 2-5
years old without
Pneumococcal • Primary series: 6-10-
previous vaccine may
conjugate 14 weeks
be given 1 dose PCV
vaccine IM 6 weeks • Booster 6 months
(PCV13 or 13 or 2 doses PCV 10
after the 3,d dose
PCVl0) 8 weeks apart
• Not routinely given
>5 years old
• Monovalent (RVl):
2 doses 6-10 weeks • Maximum age: 32
Rotavirus weeks old
Oral 6 weeks • Pentavalent human
vaccine • Do not begin in older
bovine vaccine (RVS): than 15 weeks old
3 doses 6-10-14weeks
• 6 months to 8 years old:
2 doses with interval
• 0.25 mL for 6-35
of 4 weeks if receiving
Influenza months
IM 6 months vaccine for the is1 time
vaccine • 0.5 mL for 36
then annually thereafter
months-18 years old
• 9-18 years old:
1 dose annually

143
 MINIMUM TIMING OF
VACCINE ROUTE DOSE/NOTES
AGE ADMINISTRATION

• May be given as
Measles • 9 months old
SC 9 months early as 6 months
vaccine • Booster given as MMR
during outbreaks
• 9 months to 17
years old: 2°• dose
Japanese given 12-24 months
encephalitis SC 9 months
(IE) vaccine after 1st dose
• Single dose
for =1:18years old
• Children <12
Measles • 2 doses at least 4 months given any
Mumps weeks apart measles containing
SC 12 months
Rubella • 2"' dose usually at vaccine should
(MMR) vaccine 4-6 years old receive additional 2
doses ofMMR
• <13 years old:
interval between
2 doses ideally
• 2 doses
Varicella at least 3 months
SC 12 months • 2°• dose
vaccine (but 4 weeks is
at 4-6 years old
acceptable)
• =1:13years old:
4 weeks interval
Hepatitis A • 2 doses • See discussion on
IM 12 months
vaccine 6 months apart succeeding pages

• History of sexual
abuse: begin series
• 9-14 years old:
at 9 years old
Human 0 and 6 months
• lmmunocompromised
Papilloma (2 doses)
Virus (HPV) IM 9 years old 9-26 years old: 3
• 15 years and older:
vaccine doses (0, 1-2, 6
0, 2, 6 months
months)
(3 doses)
• Pregnancy: not
recommended
• Primary series:
6-10-14 weeks
• Not routinely given
Hib vaccine IM 6 weeks • Booster at 12-15
>5 years old
months (6 months
from the 3rd dose)
• For fully immunized 7-18 years old (received 5 doses of DTP
or 4 doses of DTPif 4th dose was given =1:4 years of age): 1 dose
Tdap then Td booster every 10 years
Tetanus- • For unvaccinated 7-18 years old: 3 doses at 0,1,6 months,
reduced with Tdap as the first dose and Td for the remaining doses
diphtheria- • For incompletely vaccinated 7-18 years old: 1 dose Tdap
acellular
IM
then Td for the remaining dose at least 1 month after
pertussis • Fully immunized pregnant adolescent: give 1 dose Tdap any
(Tdap) time after 20 weeks AOG
• Unimmunized pregnant adolescent: give 3 dose Td
containing vaccine (Td/Tdap) following 0-1-6 month schedule.
Tdap should replace Td preferably after 20 weeks AOG

144
 (")
AGE IN BIRTH WEEKS MONTHS YEARS :t
0 2 4 6 8 10 12 14 16 18 20 22 6 8 10 12 14 16 18 20 2 4 6 8 10 12 14 16 18 r
C
:t
Legend: 0
Rangeof Recommended
Age BCG ,BJ 0
C
Hep B HJB ~
I
Hep B• Hep B•
s:
C
Catchup immunization (DTwP-Hib-
I I I Hep B• .. : z
~
Hep B) and
other DTaP
c,,.......
p,.,.. DTwP•/OTap•
I I I l I I I I I
NOTE:Forthe latest
ChildhoodImmunization Schedule
combo

IPV/OPV
'0~1
.•~
II
OPV•/IPV
.I
OTwP•/oTap•

I !l I
OTaP
IPV
HiB
DT•P..
OPV
Tdap/Td
6
z
I I OPV•/IPV en
(usuallyupdatedeveryFebruary) ..- -
visit www.philvaccine.org. PCV
PCV pcv• I I --l
I PCV• l l I !
(")
:t
I I
I I i I I
PCV m
C
RV

Influenza
RV Series•
I
I 11 II III Influenza Yearly
·,-,
C
r
m
I I I
...
N

I I I II I !
0

I
Measles Measles
1· (0

JE Vaccine
I I I JEV
I I I

. I I I I I I I I i I I I I
MMR MMR MMR
I I I I I I !, ! I
Varicella j llrh:ell:.
I
Varicella
I I I I I I I I I
Hep A Hep A Series
'·'"'
I I I I

IIII
I
HPV
I I I
HPVScries
I I I I

-
Source:ChildhoodImmunization
Scheduleby PediatricInfectiousDiseaseSocietyof the Philippines,PPPS,andPhilippineFoundationfor Vaccination:
2019.
Accessedat www.philvaccine.org

.
I. HEPATITIS B VACCINE
• Administration of a 4th dose is permitted when a combination vaccine containing Hep B
is used after the birth dose (as in the case of our EPI).
• Minimum age for the final dose is 24 weeks old (if 3 dose series is used)
• If 3rd dose is given earlier, give 4th dose at least 4 weeks from the 3rd dose

A. Administration of Hepatitis B Vaccine to Infants:

• Give Hepatitis B vaccine: 1 dose within 24 hours of birth for
Mother is HBsAg negative medically stable infants ;,2,000 grams
(HbsAg • mother) • If <2,000 grams: give 1 dose at chronological age of 1 month
or at hospital discharge
• Give Hepatitis B vaccine and 0.5 mL of HBIGat separate sites
Mother is HBsAg positive within 12 hours of birth, regardless of birth weight
(HBsAg + mother) • Test for HBsAgand anti-HBs at 9-12 months: if Hepatitis B
series is delayed, test 1-2 months after the final dose
• Give Hepatitis B vaccine within 12 hours of birth, regardless
of birth weight
• For infants <2,000 grams, give 0.5 mL ofHBIG in addition to
Mother's HBsAg status
Hepatitis B vaccine within 12 hours of birth
is unknown
• Determine mother's HBsAgstatus immediately: if mother is
HBsAgpositive, give 0.5 mL HBIGto infants ;,2,000 grams as
soon as possible, no later than 7 days of age

B. Booster Doses for Hepatitis B

Not recommended in healthy normal hosts
Recommended for immunocompromised and hemodialysis patients due to continued
risk of infection

II. BCG VACCINE (Bacillus Calmette-Guerin)

• PPD is recommended prior to BCGvaccination if any of the following is present:
Congenital TB
Close contact to known or suspected infectious cases of TB
Clinical findings suggestive of TB
Chest X-ray suggestive of TB
• If any of these conditions are met, an induration of ;eSmm is considered positive and BCG
is no longer recommended

III. HEPATITIS A VACCINE

• Special populations needing vaccination:
Persons traveling to or working in countries with high or intermediate endemicity
Male having sex with male (MSM)
Users of injection and non-injection drugs
Those with clotting factor disease
Those with chronic liver disease
Those who work with hepatitis A in a research lab
Those who anticipate close, personal contact with an international adoptee during the
first 60 days after arrival in the US from a country with high endemicity

VACCINES FOR SPECIAL GROUPS

I. MENINGOCOCCAL VACCINE
• Indicated for those at high risk for invasive disease:
0 Persistent complement component deficiencies
0 Anatomic/ functional asplenia ( e.g. sickle cell disease)
HIV
0 Traveler to or resident of areas where meningococcal disease is hyperendemic or epidemic
• Administration depends on brand of vaccine (please refer to individual product information)

146
II. RABIES VACCINE
A. Post-Exposure Prophylaxis
CATEGORY I CATEGORY II CATEGORY Ill
(No exposure) (Exposure) (Severe exposure)
• Transdermal bites
(puncture wounds,
lacerations, or
avulsions), scratches
• Feeding/ touching
or abrasions with
an animal
spontaneous bleeding
• Licking of intact • Nibbling of
• Licks on broken skin
skin uncovered skin with
or mucous membrane
• Sharing of eating or or without bruising
• Exposure to a rabies
drinking utensils or hematoma
patient through bites,
with a person with • Minor or superficial
Description contamination of
rabies scratches /abrasions
mucous membranes,

I
• Casual contact and without bleeding,
or open skin lesions
routine delive1y including those
with body fluids
of health care to induced to bleed
• Unprotected handling
patients with signs
of infected carcass
and symptoms of
• Ingestion of raw
rabies
infected meat
• Exposure to bats
• AllcategoryIIexposures
on the head and neck
Management
• Immediate vaccination
using any of the
following schedules
regardless of the status
• No vaccine or rabies of the biting animal • Immediate
immunoglobulin 0 2-sites ID vaccination using
For (RIG) needed on days 0, 3, 7 any of the schedules
immunologically • Pre-exposure 0 1-site IM suggested in Category
naive patients prophylaxis may be on days 0, 3, 7 and II exposure
considered for high- between day 14-28 • RIGadministration
risk persons 0 2-sites IM on day 0
and 1 site IM on
days7,21
• Rabiesimmunogiobulin
(RIG) is not indicated
• Immediate vaccination using ANY of the following:
0 1-site ID on days 0,3
For previously
• No vaccine or rabies 0 4-sites ID on day 0
immunized
immunoglobulin 0 1-site IM on days 0,3
(i.e. received
(RIG) needed • Immediate vaccination not needed if complete
PrEP or PEP)
PEP received <3 months prior
• RIGnot indicated
Notes:
• Forall categories,exposedskinshouldbe washed immediately withsoap and water
• Vaccinationregimenin BOLD text are the ones commonlyused in localpractice
Rabiesimmunoglobulin (RIG)
0ERIG(equine)40 units/kgor HRIG(human)20 units/kgIM
02018WHOguidelinessuggest that maximuminfiltration of RIGintoand aroundthe woundis effective
0Benefitof IMadministrationof remainingRIGis likelyto be verylimited(WHOno longerrecommendsthis)
0RIGshouldnot be givenafter7 days followingthe firstrabiesvaccinedose
0Skintestingis not recommended

147
 8. Pre-Exposure Prophylaxis (PrEP)
0 WHO recommends PrEP for individuals at high risk of exposure and in populations
living in rabies endemic areas (dog bite incidence >5% per year or vampire bat rabies
is known to be present)
0 Pre-exposure Prophylaxis Anti-Rabies Act 2007: for children ages 5-14 years old (high
incidence: >2.5 human rabies/million population)
0 Latest recommendations (2018) for schedule of PrEP as follows:
Two-site intradermal vaccine 0.1 mL on days O and 7
• One-site intramuscular vaccine 0.5 mL or 1 mL (depending on manufacturer) on days O& 7

C.Antibiotics
0 Given for all Category Ill wounds and all frankly infected wounds
0 Drug of choice: Co-amoxiclav 40-50 mg/kg/day in 2-3 divided doses for 7 days
0 Alternative: Clindamycin 20-30 mg/kg/day in 4 divided doses

WorldHealthOrganization.
Sources: WHOposition
Rabiesvaccines: 2018
paper.Vaccine;
MP committee oninfectious 2018
diseases.Pediatrics;
RobinsonC. el al. MMWR.MorbidityAndMortalityWeeklyReport:2018
PPS/ PIDSP/ PFVChildhoodImmunization Schedule2019
III. TYPHOID FEVERVACCINE
• Recommended for travelers to areas where there is risk of exposure & for persons with
frequent exposure to 5. typhi
• Single IM dose may be given as early as 2 years old
Revaccination every 2-3 years if there is continued exposure to S. typhi
In spite of vaccine availability, sanitation and good hygiene remain the best preventive measures
Two available forms
0 Live oral for 6 years old and above: 4-dose schedule at 0, 2, 4, 6 days
0 Inactivated polysaccharide vaccine: 1 dose IM every 2 years

IV. PNEUMOCOCCALPOLYSACCHARIDEVACCINE
Minimum age: 2 years old
• Given for high risk population
• Give 1 dose at least 8 weeks after final dose of PCV to high-risk children 2 years and older

p . _ . Patients aged 2-64 years with any of

with any of
atl~nts aged _264 year_s_ the following immunocompromised
the listed medical cond1t1ons should conditions should get 2 doses of
get 1 dose of PPSV23 PPSV23, 5 years apart

• Congenital immunodeficiencies
• Chronic heart disease, heart failure, and
• HIV infection
cardiomyopathies
• Chronic renal failure or nephrotic syndrome
• Chronic lung disease (COPD, asthma,
• Leukemia or lymphoma
emphysema)
• Generalized malignancy
• Diabetes mellitus
• Solid organ transplant
• CSF leaks
• Multiple myeloma
• Cochlear implant
• Iatrogenic immunosuppression
• Alcoholism
(treatment with immunosuppressive drugs,
• Chronic liver disease
radiation therapy)

V. POSTEXPOSUREPROPHYLAXISFORCOMMONVIRAL EXANTHEMS

EXANTHEM POSTEXPOSURE PROPHYLAXIS

Measles • Measles lg within 6 days
(Rubeola) • Measles vaccine within 3 days
• Rubella vaccine within 72 hours of exposure
German Measles
• Immune globulin is not routine but may be considered if termination
(Rubella)
of pregnancy is not an option
• Vaccine within 5 days
Chickenpox
• Anti-VZV lg within 96 hours for pregnant, immunocompromised, and
(Varicella)
newborns whose mothers had varicella 5 days before to 2 days after delivery
148
 SECTION TWO
PREVENTIVE PEDIATRICS AND ANTICIPATORY CARE

SCREENING TESTS
I. RECOMMENDEDSCREENINGTESTS
AGE GROUP TEST
Infancy • Developmental surveillance and screening
• Developmental surveillance and screening
Childhood
• Done at 9 months, 18 months, 30 months, and every year thereafter
• Screening for alcohol and drug use
0 14 to 21 years old, at every health encounter

0 Example tool: CRAFFTTool

• Screening for depression

0 Annual screening from 12-21 years old
0 Start at 10 years old if with high risk factors for depression
I
(e.g., family history, significant stressors, foster care)
0 Example tools: Patient Health Questionnaire 9, Kutcher Adolescent
Adolescence Depression Scale, Child Depression Inventory
• Annual health screening and preventive services for adolescents
using the HEADSSSformat (see Chapter 1)
• CBCfor every stage of adolescence
• Urinalysis on first adolescent health encounter
• Vaginalwet mount and Pap smear for sexuallyactive female adolescents
• Serologic tests for syphilis for sexually active male adolescents
• Non-culture tests for gonorrhea and chlamydia for both sexually
active male and female adolescents
Source:
2017Recommendations
forPreventive Pediatric
HealthCare.Pediatrics;
2017
PediatricPreventiveHeallhCareHandbook2018

II. VISUAL SCREENING

• The Clinical Practice Guideline on "The Routine Eye Examination as a Screening Tool
for Retinoblastoma" recommends routine eye examination of infants and children for early
detection ofleukocoria and strabismus (the most common presenting signs ofretinoblastoma)

A. Screening for Abnormalities

REFER TO OPHTHALMOLOGISTS FOR ANY OF THE
CHECK THE FOLLOWING:
FOLLOWING --

• Steady eyes • Droopy eyelid • Preterms that meet criteria for

• White lustrous
conjunctiva
• Clear cornea
• Non-droopy eyelids
• Pupillary reflex
• Red-orange reflex
• Jiggly eyes screening for retinopathy of
• Non-reactive pupil prematurity (see chapter 8)
• Red eye • Infants with metabolic disorders
• Dirty frothy conjunctiva • Conditions with associated eye
• Opacities problems (e.g., Down syndrome,
• Absent/dull/ neurofibromatosis)
asymmetric red-orange • History of"squinting" or "head tilt"
reflex • History of visual difficulties and/
or learning problems
• Family history of eye problems
(e.g., strabismus, congenital
cataract, congenital glaucoma)
149
 B V- IA
COMPONENTS REMARKS
• Tumbling E test/ HOTV test/ picture test or
Visual acuity tests Lea symbols (for 3-5 years old)
• Snellen chart (for 6 years old and older)
• 2.5 years old • 20/60
Acuity levels in • 3 years old • 20/40
developmentally
appropriate children • 4 years old • 20/30
• 5-6 years old • 20/20
Source:PediatricPreventiveHealthCareHandbook2018

DENTAL CARE
I. DENTAL VISITS (Recommendation from Philippine Pediatric Dental Society, Inc)
• First dental visit should be done at the time of eruption of the first tooth and no later than
12 months of age
• Follow-up dental visits at 18 months through 6 years old and every year thereafter
• Adult supervision on oral hygiene is recommended until 6-8 years old

II. FLUORIDE TREATMENT

• The American Academy of Pediatric Dentistry recommends that children at moderate
caries risk should receive a professional fluoride treatment at least every 6 months
• Use of fluoride-containing toothpaste is recommended
FLUORIDE
AGE CONCENTRATION AMOUNT OF TOOTHPASTE
(in parts per million)
• Twice daily
6 months to< 2 years old 1000 ppm
• Smear 2.5 mm or 0.125 grams
• Twice daily
2-6 years old 1000 ppm
• Pea-size 5 mm or 0.25 grams
• Twice daily
6 years old and above 1500 ppm • Full length of bristle 10-20 mm
or 0.5- 1.0 grams
Source:PediatricPreventiveHealthCareHandbook2018

RED FLAGS FOR CHILD MALTREATMENT

(RA No. 7610: Special Protection of Children Against Child Abuse, Exploitation, and
Discrimination Act)

I. RISK FACTORS FOR VICTIMIZATION

• Female sex • Poverty
• Unaccompanied children • Children caught in the middle of war or
• Children in foster care, adopted children, armed conflict
stepchildren • Single parent homes/ broken homes
• Physically or mentally handicapped • Social isolation
children • Parent with mental illness or alcohol/drug
• History of past abuse dependency

150
II. SIGNS AND RED FLAGS

• Discrepancy between elicited history & mechanism of injury

Red flags for • The story changes or varies
possible child • There was an unreasonable delay in seeking care
abuse • The parent/ caretaker seems to be hostile towards the child and
denies there was injury
Signs suggestive • Bruises over multiple areas (lower back, buttocks, thighs, cheeks, ear
of physical abuse pinna, neck, ankles and wrists and corners of mouth and lips)
• Negative self-image: withdrawn or depressed
Signs suggestive • Self-destructive acts: cutting oneself, reckless behavior
of verbal/ • Antisocial behavior: physical aggression, delinquency, cruelty to
emotional abuse animals, frequent quarrels with classmates
• Delayed development: bedwetting, thumb sucking
• Dorsum of hands or feet ("glove and stocking burn pattern"),

Red flags for

intentional burn
injuries
anogenital area ("donut sign" in the buttocks)
• Repeated burns
• Symmetric or patterned burns
• Uniform thickness with clear border
• Delay in seeking care
I
• Metaphyseal-epiphyseal (<3 years old) or bucket handle or comer fractures
Red flags • Thoracic cage (posterior ribs)
specific for • Shoulder (scapula, acromion)
fractures due to • Clavicle (medial or lateral)
abuse • Vertebral body (from lateral compression of spinous processes)
• Occipital fractures
• Suspicious findings for sexual abuse
° Focal erythema in the vestibule
0Localized abrasions within the vestibule
0Laceration of the posterior fourchette without history of straddle injmy
0Peaked notch in the posterior hymen
0STDs like herpes or chlamydia
Red flags for
• Definitive evidence of sexual abuse
sexual abuse
0Recent bleeding from a laceration or transection of the hymen
0Presence of sperm
0Recent anal laceration
° Complete absence ofhymenal tissue between 3 & 9 o'clock position
0Pregnancy in a pubertal minor
0Syphilis or gonorrhea (non-neonatal)

III. SHAKEN BABY SYNDROME

Responsible for at least 50% of the deaths of children caused by non-accidental trauma
Involves forceful, intentional, repetitive, and violent shaking of the baby (usually <2 years old)
• Leads to traumatic brain injury (subdural hematoma)

Source:PediatricPreventiveHealthCare Handbook2018

151
INFANT & CHILD NUTRITION
• Proper nutrition is crucial to optimal growth and development
• Rapid growth rates in children are accompanied by high maintenance needs

I. GUIDELINESON INFANT & YOUNGCHILDFEEDING

• UNICEF & WHO recommend that infants be exclusively breastfed on demand for the first
6 months oflife (refer to neonatology chapter for details on breastfeeding)
• Early introduction of food & other liquids leads to:
Reduced breastmilk intake
0 Decreased full absorption of nutrients from breastmilk
0 Increased risk of diarrhea and upper respiratory tract infections
A. Feeding the 6-12 Month Old
° Complementary feeding:
Introduction of solid and liquid food other than breast milk or formula
Begin at 6 months
Introduce new food one at a time
Additional new food spaced 3 days to detect adverse reactions
Encourage cup rather than bottle
0 Give iron-containing food/supplement (meat, iron-fortified cereals)
Encourage zinc intake (e.g., meat, dairy products, wheat, rice)
Discourage fluids other than formula or water (maximum of 4-6 oz/day for fruit juice)

B. Feeding the 2 Year-Old

3 meals/day with 2-3 snacks
0 Reduced food intake is expected due to decrease in growth rate (do not force-feed and
understand that lack of interest in food is only temporary)
Allow for self-selection of diet and respect likes & dislikes

II. MICRONUTRIENTSUPPLEMENTATION
AGE PREPARATION DOSE/ DURATION
1) Iron Supplementation*
For low birth weight • Drops with 15 mg elemental • 0.3 mL once a day to start at 2
(1,500-2,499 grams) iron per 0.6 mL months old until 6 months old
Infants • Drops with 15 mg elemental • 0.6 mL once a day for 3
6-11 months old iron per 0.6 mL months
• 5 mL once a day for 3 months
• Syrup with 30 mg elemental or 30 mg once a week for
1-5 years old
iron per 5 mL 6 months with supervised
administration
• Tablet with 60 mg elemental
Adolescent females
iron with 400 mcg folic acid • One tablet once a day
10-19 years old
(coated)
2) Vitamin A Supplementation
• One dose only (one capsule is
given anytime between 6-11
Infants 6-11 months
• 100,000 1.U. months old, but usually given
old
at 9 months old during the
measles immunization)
Children 12-71
• 200,000 1.U. • One capsule every 6 months
months old
'Recommendationfromthe PhilippineSocietyof PediatricHematology:a CBCto screen for anemiaat least once
betweenthe time intervalsfor those at risk:6-24 monthsold,2-6 years old, 10-19years old.The Societyof Adoles-
cent Medicineof the Philippinesrecommendsat least once at each stage of adolescence.

Source:DOHPhilippines.
UpdatedGuidelineson Micronutrient
Supplementation. DOH:2013
KliegmanR.et al NelsonTextbook
of Pediatrics(21sted).Elsevier:2020

152
DEWORMING
I. INDICATIONS& CONTRAINDICATIONS
INDICATIONS CONTRAINDICATIONS
• Severe malnutrition
• High-grade fever
• Profuse diarrhea
• Dewonning for children 1-12 years old • Abdominal pain
• Serious illness
• Previous hypersensitivity to
anti-helminthic drug

II. REGIMENS
DRUG* AGE GROUP** DOSE FREQUENCY
• 12-23 months old • 200 mg • Single dose, every 6 months
Albendazole

Mebendazole
• 24 months and above

• 12 months and above

'Either drug shall be taken on fullstomach
• 400 mg

• 500 mg
• Single dose, every 6 months

• Single dose, every 6 months I

"DOH National Filariasis Elimination Program implemented in municipalities endemic for filariasis: mass
treatment with diethylcarbamazine citrate (DEC)and albendazole is given to children 2 years old and above

Source:
DOHRevised
Guidelines
onMassDrugAdministration
& Management
ofAdverse
EventsFollowing
Deworming

REFERENCES
1. AAP Committee on infectious diseases. Recommended Childhood and Adolescent
Immunization Schedules: United States, 2018. Pediatrics. 2018;141(3): e20180083
2. ClinicalPractice Guideline on "The Routine Eye Examination as a Screening Tool for Retinoblastoma"
3. Department of Health. DOH Philippines Administrative Order No.119 s. 2003. "Updated Guidelines
on Micronutrient Supplementation (Vitamin A, Iron and Iodine)."Available from< https:/ /ww2.fda.gov.ph/>
4. Department of Health. DOH Administrative Order 2015-0054 Revised Guidelines on Mass
Drug Administration and Management of Adverse Events Following Deworming and Serious
Adverse Events. Available from <l1ttps://www.doh.gov.ph>
5. Department of Health. Updated Guidelines on Micronutrient Supplementation. DOH; 2013.
Available from <https:/ /www.doh.gov.ph>
6. Kimberlin DW, Brady MT.Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee
on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018
7. Kliegman, R., Stanton, B., St. Geme, J., Scho1; N., and Behrman, R.. Nelson Textbook of
Pediatrics 21st ed. 2020. Canada: Elsevie1:
8. Navarro, X., Bauzon, A., Aguila 1:)., and Malanyaon, 0. Fundamentals of Pediatrics
Competency-based 1st ed. 2014. Quezon City: C & E Publishing, Inc.
9. Pediatric Preventive Health Care Handbook 2018. Philippine Pediatric Society. Philippines, 2018
IO.Pediatric Infectious Disease Society of the Philippines, Philippine Pediatric Society and
Philippine Foundation for Vaccination. Childhood Immunization Schedule 2019. Available
from < http://www.phitvaccine.org/>
11.Republic of the Philippine Congress. Republic Act No. 7610 - Special Protection of Children Against
Child Abuse, Exploitation, and Discrimination Act. Available from< https:/ /pcw.gov.ph/law /
republic-act-7610>
12.Richerson JE, Simon GR,Abularrage JJ,et al., Committee On Practice And Ambulato1y Medicine; Bright
Futures Periodicity Schedule Workgroup. 2017 Recommendations for Preventive Pediatric Health
Care. Pediatrics. 2017 Apr;139(4). pii: e20170254. doi: 10.1542/peds.2017-0254. Epub 2017 Feb 17.
13.Robinson, C.,Romero,)., Kempe, A., Pellegrini, C.,& Szilagyi, P. (2018). Advisory Committee on
Immunization Practices Recommended Immunization Schedule for Children and Adolescents
Aged 18 Years or Younger- United States, 2018. MMWR. Morbidity And Mortality Weekly Report,
67(5), 156-157. doi: 10.15585/mmwr.mm6705e2
14.World Health Organization. (2018). Rabies vaccines: WHO position paper, April 2018 -
Recommendations. Vaccine, 36(37), 5500-5503. doi: 10.1016/j.vaccine.2018.06.061
153
154
NEONATOLOGY
 SECTION ONE
EVALUATING THE NEWBORN

DEFINITION OF TERMS
I. PERIODS OF DEVELOPMENT

PERIOD DURATION
Perinatal Period • 28'h week of gestation to 7'" day after birth
• First 28 days after birth
• Further subdivided into:
Neonatal Period 0Very early: birth to < 24 hours
0Early: birth to 7 days
0Late: 8 days to 28 days
Infancy

II. GESTATIONALAGE
• Birth to 1 year

Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Elsevier:2020 I

AGE DEFINITION SUBCATEGORIES
• Defined as live birth occurring • Late preterm: 35-36 weeks
before the 37'" week of gestation • Moderate preterm: 32-34 weeks
Preterm
• Early preterm: < 32weeks
• Extreme preterm: < 28 weeks
• Born at 37 to 41 & 6/7 weeks • Early term: 37 to 38 & 6/7 weeks
Term • Term: 39 to 40 & 6/7 weeks
• Late term: 41 to 41 & 6/7 weeks
Postterm • Infants born after 42 weeks

Source:KliegmanR, et al. NelsonTextbockof Pediatrics(21sted.).Elsevier:2020

FanaroffA, et at FanaroffandMartin'sneonatal-perinatal
medicine(10thed.).Philadelphia; 2015

SPECIFIC ASPECTS OF HISTORY TAKING

PREGNANCYAND DELIVERY
ASPECT DETAILS
• Age, parity, and gravidity of mother atthe time of birth
• Prenatal care:
0 Regularity of check up
0Work-up done such as CBC,urinalysis, Group B Streptococci
(GBS) screening, HbsAg, test for syphilis, ultrasound, oral
glucose tolerance test
• Maternal illnesses during pregnancy
Maternal History • Drug intake during pregnancy
• Exposure to ionizing radiation (e.g., radiographs, CT scan) or
injurious chemicals (e.g., alcohol, smoking, illicit drug use),
emotional stress during pregnancy
• Prenatal ultrasonography: progress of growth of the fetus and
presence of congenital anomalies
• Previous maternal reproductive problems such as stillbirths,
premature delivery, neonatal sepsis in previous babies

157
 • Age of gestation upon delivery
• Was the delivery induced and reason for induction
• Mode of delivery (spontaneous vaginal, cesarean, forceps,
vacuum assisted, anesthesia or sedation)
• Total duration oflabor and delivery
• Oligohydramnios / polyhydramnios
Peripartum • Premature rupture of membranes (PROM) defined as rupture
> 18 hours prior to delivery
• Place of delivery
• Attendant during the delivery (OB, midwife, traditional birth
attendant)
• Appearance of the cord and the placenta
• Presence of complications
Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Elsevier:2020
GleasonC, et al. Avery'sdiseasesof the newborn(10thed.).Elsevie:2017

PHYSICAL EXAMINATION OF THE NEWBORN

I. GENERALAPPROACH
TIMING OF
PERTINENT DATA
EXAMINATION
• Condition of the baby at birth:
0Spontaneous breathing
0APGARscore
° Cord coil
Initial examination 0 Meconium staining
(done as soon as
possible after delivery)
0Vital signs
• Ballard score/maturity scoring
• Anthropometric measurements (ideally plotted against
nomograms)
• Quick and general physical examination
Second examination
• More detailed and comprehensive physical examination
(done within 24 hours)
Discharge
examination • Note changes in the physical examination
(if neonate stays >48 • Normal anatomic variations should be explained to the parents
hours in the hospital)
Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Elsevier:2020

II. VITALSIGNS
PARAMETER NORMAL VALUES/REMARKS
Respiratory Rate (RR) • 30-60 counts per minute (cpm)
Heart Rate (HR) • 120-160 beats per minute (bpm)
Core Temperature • 36.5° - 37.5° C
• Not routinely taken in newborns unless there are signs and
Blood Pressure (BP)
predisposing factors associated with circulatory problems

Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Elsevier:2020

GleasonC. et al. Avery'sdiseasesof the newborn(10thed.).Elsevie:2017

158
Ill. APGAR SCORE
• Taken at 1 and 5 minutes after birth
• May be repeated at 5-minute intervals for infants with 5 minute scores <7
• It is used as method of reporting the status of the newborn and the response to resuscitation
• However, it is important to note the following:
0 Several factors may affect the APGARscore (e.g., prematurity, sedative drugs,
congenital anomalies, neuropathies)
0 There is no consistent data on the significance of APGARscore on preterm infants
It is not used to established asphyxia
It does not predict specific neurologic outcomes
• A low 1-minute score does not correlate with outcome; but the 5-minute score is a valid
predictor of neonatal mortality

APGAR Evaluation of Newborn Infants

SIGN 0 POINT 1 POINT 2 POINTS
A ctivity • Some flexion of
(muscle tone) • Limp • Active motion
extremities
p ulse
(heart rate)

G rimace
(reflex irritability)*
• Absent

• No response
• < 100 bpm

• Grimace
•>l00bpm

• Cough or sneeze
• Pulls away, crying
I
A ppearance • Body pink,
(skin color) • Blue, pale • Completely pink
extremities blue

R espiratory effort • Absent • Slow, irregular • Good, crying

'Elicitedthroughinsertionof a catheterin nostril,testedonceoropharynxis clear

Interpretationof theAPGARScore:
• 7-10 points:goodcardiopulmonary adaptation,newbornwilldo well
• 4-6 points:needforresuscitation,
especiallyventilatory
support,& medicalintervention
• 0-3 points:needforimmediateresuscitationand mayneed NICUcare
Source:ApgarV.A Proposalfor a NewMethodof Evaluationof the NewbornInfant.Anesthesia& Analgesia:1953
KliegmanR. et al. NelsonTextbookof Pediatrics(21sted.). Elsevier:2020
GleasonC, et al.Avery'sdiseasesof the newborn(10thed.). Elsevie:2017

Case: At the 1" minute of life, a newborn was noted to have acrocyanosis, HR of 130 bpm
grimaces to stimulation, good respiration, and with active movement of extremities. What
is the APGAR score at this time?
A= 2 (active movement)
P = 2 (HR> 100)
G = 1 (grimace)
A= 1 (acrocyanotic)
R = 2 (regular respiration)

SCORE= 8

159
IV. BALLARDSCORINGSYSTEM (MATURITY RATING)
• Used for maturational assessment of gestational age
• Accuracy is +/- 2 weeks the actual gestational age
• High risk for mmtality or morbidity if a discrepancy exists between the estimation of gestational
age by examination, the mother's estimated date of her LMP,and fetal ultrasound evaluation
• Full term infants are scored as early as possible and may be reliably scored within 72
hours of birth
• Extremely preterm infants should be scored within the first 12 hours of life
• Divided into two groups of scoring system:
0 Physical maturity
0 Neurologic maturity
• The physical and neurologic scores are added, the sum of which calculates gestational age

A. Physical Maturity

I -1 I 0 1 2 3 4 5
Smooth. Superiicial Parchment
Sticky, Gelatinous. Cracking. Leathery,
Pink peeling deep
SKIN friable. red. pale areas, cracked
visible and/or rash cracking
transparent translucent rare veins Wrinkled
veins few veins No vessels

LANUGO None Sparse Abundant Thinning Bald Areas Mostly Bald

Heel-toe Anterior Creases

PLANTAR
40-SOmm: -1
>50mm. Faint red Creases on
transverse over entire
SURFACE
<40mm: -2
no crease marks ant. 2/3
crease only sole

Stripped Raised
Barely Flat areola, Full areola.
BREAST Imperceptible areola, areaola.
perceptible no bud 5-10mm bud
1-2mm bud 3-4mm bud
Slightly Well-curved
Lids fused: lids open, Formed and Thick
curved pinna. soft
EYE/EAR loosely (-1). pinna flat. firm. instant cartilage, ear
pinna: sett. but ready
tightly (-2) stays folded recoil stiff
slow recoil recoil
Scrotum Testes in Testes Testes Testes
GENITALS Scrotum flat.
empty. faint upper canal. descending, down, good pendulous.
MALE smooth
rugae rare rugae few rugae rugae deep rugrae
Prominent Prominent Majera and
Clitoris Majera Majera
GENITALS clitoris. clitoris, minora
prominent. large, cover clitoris
FEMALE small labia enlarging equally
labia flat minora small and minora
minora minora prminent

8. Neurologic Maturity
-1 0 1 2 3 4 5

Posture ~ ~ o¢=c ~ ~
Square
Window
(Wrist) r r r
>90° go• 60°
~
45°
~
30°
I
o•

Arm Recoil
~180°
11'-i}- ½
140-180° 110-140° 90-110°
{}
<90°

c6 ~ ~
Popliteal
Angle ~ ~ ~ c6
180° 160° 140° 120° 100° goo <90°

Scarf Sign
tr ~ & ~ ~ §
Heel to Ear GE:=) ci=:3 CC3 ~ CX:B ~
--------------- . ···--·--·----- ----- I
160
 PARAMETER INSTRUCTIONS ILLUSTRATION

• Wait until the infant is

settled into a relaxed,
supine position
Posture
• May do gentle manipulation
to allow the infant to seek
baseline position

Square window
• Straighten the infant's
fingers and apply gentle
pressure on the dorsum of the
hand, close to the fingers
I
• With the infant lying supine,
the examiner places one
hand beneath the elbow for
support.
• Elbow is then placed into
flexion then momentarily
extends the arm before
Arm recoil
releasing the hand.
• The angle of recoil to which
the forearm springs back to
flexion is noted
• A score of 4 is when there is
contact between the infant's
fist and face

• Infant is placed on supine

position
• Thigh is gently placed on
the abdomen with the
knees fully flexed
• After the infant has relaxed
into the position, the
examiner gently holds the
Popliteal Angle
foot at the sides with one
hand while supporting the
side of the thighwith the other
• Leg is then extended until a
definite resistance is felt
• The angle formed at the
knee by the upper and
lower leg is measured

161
 • With the infant lying supine
and head in the midline,
the examiner supports the
infant's hand across the
upper chest with one hand
Scarf sign
• Thumb of the examiner's
other hand is placed on the
infant's elbow
• Elbow is nudged across the
chest until resistance is felt
• Infant is placed in supine
position & the flexed lower
extremity is brought to rest
on mattress alongside the
trunk of the infant
• Other hand is used to hold
Heel to ear
foot at the side & to pull it
toward the ipsilateral ear
• Location of heel where a
significant resistance to
extension of the posterior
pelvic girdle flexors is noted

C. Maturity Rating Score (physical and neurologic scores are addec/)

SCORE WEEKS
-10 20
-5 22
0 24
5 26
10 28
15 30
20 32
25 34
30 36
35 38
40 40
45 42
50 44
Source:BallardJL,et al: NewBallardScore.J Ped,atncs;
1991

V. ANTHROPOMETRICS
• Birth weight, birth length, head circumference and chest circumference are plotted
against gestational age on standard growth curves shown on next page {Lubchenco Chart)
• A different chart is used for premature infants (Fenton Chart)

A.Classification based on Birth Weight

0 Low birth weight:< 2500 grams
Very low birth weight:< 1500 grams
' Extremely low birth weight:< 1000 grams

162
B. Classification based on Birth Weight versus Gestational Age (using the Lubchenco Chart)
Small for gestational age: birth weight below 10'" percentile
0 Appropriate for gestational age: birth weight between 10'" to 90'" percentile
Large for gestational age: birth weight above 90'"-percentile
PRETERM TERM
GM I I I
4250 WEIGHT
I

-
4000
3750 90 %
3500
3250
3000
.,.i...- - - I
·50 %

~ ,
2750 ,
2500 , ,, ,,. -i- 10 %

2250
2000
,,
,J,,
, V
, I'
1750 .,,,. ,

---,,,,.-~, /

- --
1500
/
1250
1000
750 ·i
500
'""1'""
I

I
250
o
24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43
CM
53 90%
52 ~LENGTH
51
49
48
47
45 10%
44
43
42
40
39
38
37
35
34
33
31
30
29
28
26
25
26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42
CM
39
39 HEAD CIRCUMFERENCE
38
37
90%
36
35
50%

H~~
31
10%

31
30
29
28
27
26

~~ ii
22
21
20 Source:
LubchencoL.,etal.Journal
ofPed,ilrics;
1966
26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 F.,etal.
Battagl,i Journal
ofPed@lrics;
1967
WEEK OF GESTATION LowdermilkDL,etal.SLLouis;
2007
163
VI. SPECIFICEXAMINATIONOF THE NEWBORN
ASPECT DESCRIPTION
1) Genera IAppearance
'
• Assess overall appearance/color
Observe infant
• Assess respiratory effort and any signs of distress
in a resting non-
stimulated state • Assess position/posture & movement/activity ( e.g., asleep, alert)
• Assess quality of cry /suck
i) SRin II " ~ -c

• In premature infants: thin, delicate, and tends to be deep red

• In extremely premature infants: almost gelatinous and translucent
General
• In post-term infants: peeling, parchment-like, the most severe
degree of which mimics ichthyosis congenita
• Harmless cyanosis of the hands and feet, especially when they are cool
Acrocyanosis
• Body is pink
• Fine, soft, immature hair
• Frequently covers the scalp and brow
Lanugo
• May cover the face of premature infants
• Usually replaced by veil us hair in term infants
• Common in dark-skinned races
Mongolian spots • Flat, blue-gray with well-defined margins usually over the buttocks
and back
• Benign small pa pules or pustules on an erythematous base, usually
Erythema 1-3 days after birth, and persist for as long as 1 week
toxicum • Filled with eosinophils
• Found on the face, trunk, and extremities
• Small inclusion cysts, pearly-white
Milia
• Usually on the face
• Lacy pattern on the skin similar to cobblestones
Cutis marmorata
• Vasomotor response to cold stress
Neonatal acne • Open and closed comedones from circulating androgens
Pustular • Vesiculopustular eruption around the chin, neck, back, extremities,
melanosis palms or soles (benign lesion which lasts for 2-3 days)
Harlequin color • Division of the body from forehead to the pubis into red & pale halves
change • Transient and harmless
• Salmon patch over eyelids, glabella & nuchal area; disappears with time
Birthmarks
• Capillary (strawberry) hemangiomas
Mottling • Associated with general circulatory instability or serious illness
Jaundice • Normally not seen on the first examination (<24 hours)
Meconium • Brownish-yellow staining of the vernix, skin, and the cord if the
staining amniotic fluid has been by colored by the passage of meconium
• May be caused by anemia, asphyxia, shock, or edema
Pallor
• Postmature infants tend to have paler & thicker skin
• Over the lumbosacral spine
Tufts of hair
• Suggests an underlying problem (e.g., occult spina bifida, sinus tract)
,•-
3) l;lead It Ill

• Molding due to intrauterine positioning or vaginal delivery

General Findings
• Fontanels should be open and flat (anterior and posterior)

164
 Caput • Edema of scalp skin
succedaneum • Crosses suture line
• Minimal bleeding
• Resolves in 2-3 days
Cephalhematoma • Subperibsteal in location
• No edema or discoloration of the scalp
• Does not cross the suture line
• Bleeding is rarely severe
• Resolves in 2-3 weeks
Subgaleal • Beneath epicranial aponeurosis
hemorrhage • May extend to orbits (racoon eyes) & nape
• Bleeding may be massive
Check for Trauma • Resolves in 2-3 weeks

I
Skm

Gal:::~eE:r~s1s
....
•··. ....•····
... ..
Penosteum ···· ··
Skull
Dura

4J Eyes
• Often open spontaneously if the infant is held up and tipped gently
General Findings forward and backward
• Pupillary reflexes present after 28-30 weeks AOG
• White pupillary reflex (warrants immediate ophthalmologic consult)
Leukocoria
• Suggests cataracts, tumor, chorioretinitis, retinopathy of prematurity
Subconjunctival • Regresses spontaneously (85% resolve by 2 weeks of age; in all
Hemorrhage infants by 4 weeks; no treatment needed)
• Congenital malformation of the eye causing defects in the iris, lens,
Coloboma
or retina (small notch to a large cleft)
Aniridia • Absence of the iris
Congenital
• Tearing, photophobia, and cornea > 1 cm in diameter
Glaucoma
5) Ears
Skin tags, pits,
malformed ears • Associated with hearing and other anomalies

• Imaginary line joining the medial canthi and perpendicular to

Normal ear
vertical axis of the head
position
• Low set ears: helix lies below this line
6) 011aicav.ify
• Temporary accumulations of epithelial cells on the hard palate or
Epstein pearls
either side of the rap he
Ankyloglossia or • lfthere are problems with feedings (breast or bottle) in an infant
tongue-tie with short frenulum, a frenulotomy may be indicated

165
 • Presence of teeth at birth
Natal teeth
• Should be removed by a dentist to prevent the risk of aspiration
• Examine closely the throat and check for palatal or uvular clefts
Throat • Contour of the palate must be noted if the arch is excessively high
or the uvula is bifid
,.
7) N·ose ~r -
General Findings • Newborns are obligate nose breathers
Choanal atresia • Inability to pass a feeding tube through the nares
C
8) Neck ., lJ
Branchial cleft • Incomplete closure of branchial clefts
cysts • Found lateral and anterior to the sternocleidomastoid muscle
Thyroglossal duct
• Cystic dilatation of thyroglossal duct remnants seen in the midline
cysts
Congenital
• Head turned toward and the face turns away from the affected side
torticollis
Redundant skin
• Suggests Turner syndrome if in females
or webbing of neck
9) Chest I~ ;,,

Breast • Due to maternal hormones

hypertrophy • Milk may be present ("witch's milk") but should not be expressed
Supernumerary • Along the mammary line
nipples • May have associated renal and cardiac anomalies
• Protrusion abnormalities of the anterior chest wall
Pectus carinatum
• Commonly described as "Pigeon chest" or "Keel Chest"
• Caved-in or sunken appearance of the chest wall
Pectus excavatum
• Commonly described as "Funnel chest"
Widely spaced
nipples with • Suggests Turner syndrome
shield-shaped chest
., <1' ~
10) Cardiovascular Systi:m . , ·-"' '-"· - "
Hyperdynamic
precordium • Often seen at birth but if persistent, suggests cardiac pathology

• On 4th left intercostal space just medial to the midclavicular line

Point of Maximal
• Changed location of PM! may be seen in pathologic conditions (e.g.,
Impulse (PM!)
situs inversus, dextrocardia, pneumothorax, masses)
• Left-sided obstructive lesions (aortic stenosis, hypoplastic left heart
syndrome) present with diminished peripheral pulses
Peripheral pulses
• Coarctation of aorta: stronger pulses in upper extremities
• Patent ductus arteriosus: bounding pulses & wide pulse pressure
• Innocent murmur: soft, vibratory, sometimes intermittent
Murmurs • Pathologic murmur: associated with thrill, loude1; heard over wider
area of chest beyond the first few days of life
,.,,
11)Abdomen u "' ~ " c.
"'

• Slightly protuberant in the neonate

General Findings • Umbilical cord made ofwharton's jelly, 2 umbilical arteries and 1
vein (stump falls off by 2 weeks of life)
Omphalitis • Redness, edema, foul odor, discharge of the umbilical stump
166
Diaphragmatic
• Scaphoid abdomen, bowel sounds auscultated over the chest wall
hernia
• Omphalocoele: herniation of abdominal contents into base of
umbilical cord, abdominal contents covered by a protective membrane
• Gastroschisis: laterally located full thickness abdominal wall defect,
External masses extruded intestine not covered by membrane. Defect is usually
located on the right side.
• Umbilical hernia: intraabdominal contents protrude through a
fascia! defect at the umbilicus that is covered by skin
• Must pass meconium within 48 hours
Anus
• Inspect for patency to rule out imperforate anus
~
12) Genit,;>Urinah S~stem C Ill
""
• Voiding must be within the first 24 hours of life
General Findings • Ambiguous genitalia: newborn's gender cannot be determined due
to masculinization of the female or feminization of the male
• Inspect external genitalia: labia majora pigmented more than skin,

I
Females covers labia minora
• Blood tinged discharge is due to withdrawal from maternal estrogen
• Inspect phallus, retract prepuce to view the urethral orifice
• Hypospadia: urethral orifice located on the glans or ventral surface
of the shaft
• Chordee: caudal curvature of the penis due to incomplete
development of the foreskin
Males • Testes: commonly palpable in the scrotum, may be retractable
• Persistence of processus vaginalis:
0 Inguinal hernia: opening at the inguinal ring allows extrusion of
the abdominal viscera with increased intra-abdominal pressure
0 Hydrocele: narrow opening at the inguinal ring allows peritoneal

fluid to leave the abdominal cavity and accumulate in the scrotum

"
13) Ext~e,mities (,inspect limbs for deformi~ies) ~
'
• Polydactyly (extra digits), syndactyly (fused digits), or clinodactyly
Check for (curved digits)
Anomalies • Simian crease (single transverse palmar crease)
• Clubfoot/ talipes equinovarus (hyperextension & incurving of foot)
• Brachia! Palsies
0 ~45% are associated with shoulder dystocia in large infants
0 Erb-Duchenne palsy (CS-C6, upper trunk)
Ann adducted and pronated and the forearm internallyrotated
Absent biceps reflex and Moro reflex on the affected side
If with C4 involvement: ipsilateral alteration in diaphragm
excursion or ipsilateral diaphragmatic paralysis
° Klumpke palsy (C7-C8, Tl, lower trunk)
Claw hand: paralyzed, no wrist movement, absent
Check for Trauma grasp reflex
If with involvement of the Tl sympathetic fibers:
Horner syndrome (ptosis, miosis, enophthalmos)
• Clavicular fracture
0 Most commonly fractured bone during labor and delivery

° Common in LGAinfants with shoulder dystocia

0 Infant does not move the arm freely on the affected side

° Crepitus and bony abnormality may be palpated

0 Absent Moro reflex on the affected side

Source:FanaroffA, et al. FanaroffandMartin'sneonatal-perinalal

medicine(10thed.).Elsevier:2015
GleasonC, et al.Avery'sdiseasesof thenewborn(10thed.).Elsevier;2017
167
Vil. DEVELOPMENTAL (PRIMITIVE) REFLEXES
• Primitive reflexes that indicate integrity of the brainstem and spinal cords
• Disappearance indicates maturation of the cerebral hemispheres
• Persistence beyond the expected date suggests maturational lag or impaired central
nervous system (may be seen in cerebral palsy and other neurodegenerative disorders)

HOW TO ELICIT AGE OF AGE OF

& EXPECTED ILLUSTRATION APPEARANCE DISAPPEARANCE
RESPONSE
1) Rooting Reflex
• Touch cheek or lip of
the infant
• Response: infant turns
• 32 weeks AOG • 1 month
head to the stimulus
and makes sucking
motion
2) Palmar Grasp

• Place a finger in the

open palm of the infant
• 28 weeks AOG • 2-3 111onths
• Response: infantgrasps
the examiner's hand

3) Placing/Stepping Reflex

• Stimulating dorsum of
the foot against the edge
of the examining table • 37 weeks AOG • 4-5 111onths
• Response: infant lifts
leg on the table

4) Moro (Startle) Reflex

• Support the infant in
a se111i-erect position
and allow the head to
fall backwards toward
the examiner's hand
• Response: symmetric
• 28-32 weeks AOG • 5-6 months
extension and
abduction of fingers
and upper extremities
followed by flexion of
the upper extremities
and an audible cry

168
5) Tonic Neck (Fencing) Reflex

• Rotate the infant's

head to one side
• Response: fencing
position ( extension
• 35 weeks AOG • 6-7 months
of the arm on the side
to which the face is
rotated with tlexion of
the contralateral arm)

6) Plantar Grasp Reflex

• Placing pressure on
the palm of the sole
• Response: similar • 11 weeks AOG • 7-9 months
response as the palmar
grasp reflex but weaker

7) Landau Reflex
I
• Examiner lifts the
infant with one hand
under the trunk
facing downward • Covered up by
• 3 months
• Response: extension voluntary action
of the vertebral
column causing the
infant to lift head
8) Parachute Reflex

• Examiner supports
the infant's trunk and
suddenly lowers the
• Covered up by
infant as if he/she • 4-9 months
voluntary action
were falling
• Response: the arms
extend to break the fall

9) Crawling Reflex
• If baby is placed on
stomach, he/she will
pull legs under the
body & kick them out
in a crawling motion
• Disappears after
• When babies are • 28 weeks AOG
just a few weeks
placed on mother's
stomach, they are
able to crawl up to the
breasts and start
suckling
Source:KliegmanR.et al. Nelsontextbookof pediatrics(21sted.).Canada:Elsevier;2020
MenkesJ, et al. ChildNeurology(7thed.).LippincottWilliams& Wilkins;2006

169
 SECTION TWO
ES.SENTIAL INTRAPARTUM AND.NEWBORN.CARE

ESSENTIAL INTRAPARTUM AND NEWBORN CARE (EINC)

• A basic component of the DOH's Maternal, Newborn, and Child Health and Nutrition strategy
• Represents the highest standard for safe and quality care for birthing mothers and
healthy newborns
• Series of time bound chronologically ordered standard procedures that a baby receives at birth

I. EINC CORESTEPS

STEPS MANAGEMENT
• Immediately dry the baby to stimulate breathing and to avoid hypothermia
for a full 30 seconds unless the infant is both floppy/limp and apneic
• Sequence of drying is as follows (5 seconds each): face, head, trunk, back,
Immediate arms, legs
and
1 • Hypothermia can lead to infection, coagulation defects, acidosis, delayed
Thorough
fetal to newborn circulatory adjustment, hyaline membrane disease, or
Drying
brain hemorrhage
• Routine suctioning of nose and mouth has no proven benefit unless
meconium stained and the baby is limp or apneic
• Place the baby on mother's chest or abdomen to provide warmth & allow the
"good bacteria" from the mother's skin to infiltrate the newborn
• Increases the duration of breastfeeding
• Technique (if breathing or crying):
• Position prone on the mother's abdomen or chest in between the breasts
• Cover the newborn
0 Dry linen for back

Early Skin-To- 0 Bonnet for head

2
Skin Contact 0 Temperature check: (room: 25-28°C; baby: 36.5 - 37.5°C)
• Benefits include:
• B: Breastfeeding success
• L: Lymphoid tissue system stimulation
• E: Exposure to maternal skin flora
• S: Sugar (protection from hypoglycemia)
• T: Thermoregulation
• Delay cord clamping 2-3 minutes after birth or until the cord has stopped
pulsating, whichever comes first
• Remove the first set of gloves (may use "double gloving" method) before
handling the umbilical cord, so the sterile underlying pair should be used
Properly- to handle and cut the cord
3 Timed Cord • Clamp the cord without milking it 2 cm from the base & put the second
Clamping clamp 5 cm from the base and cut the cord
• Benefits of properly timed cord clamping:
0 Decreases anemia in term and preterm babies
• Decreases need for blood transfusions in premature babies
• Decreases bleeding in the brain in premature babies
• Non-separation for 90 minutes after birth or after the first full breastfeed
• Leave the newborn between the mother's breasts in continuous skin-to-
skin contact.
Non-
• Monitor mother and baby regularly in the first 1-2 hours and assess baby:
Separation
4 • Breathing: listen for grunting, look for chest in-drawing and fast
of Mother
breathing
and Baby
0 Warmth: check to see iffeet are cold to touch if no thermometer

• Washing should be delayed until after 24 hours of life (body temperature

of the newborn is more stable after 24 hours)
170
II. OTHER EINC RECOMMENDATIONS

Recommends against • Foot printing

these traditional • Use of bigkis or abdominal binder
newborn care • Early bathing and removal of vernix
practices • Artificial milk substitutes
• Antenatal steroids for preterm labor
• Providing mothers with birth plan
• Allowing a companion of choice during labor
Recommended • Allowing mother to assume position of choice and mobility
obstetric practices during labor
• Use of partograph
• Upright position during delivery
• Active management of the 3rd stage of labor
• Perinea! shaving
Recommends against • Enema
routine practice of • NPO
the following • IV fluid administration
• Episiotomy

NEWBORN CARE

ASPECT MANAGEMENT
I
• For all infants, including those born by cesarean section
• To protect against gonococcal ophthalmia neonatorum
• Medications:
Eye prophylaxis
0Erythromycin ointment 0.5% or tetracycline ointment 1%
0Alternative is Crede's prophylaxis: 1% silver nitrate solution (can
lead to a transient chemical conjunctivitis in 10-20% of cases)
• Dose: 0.5-1.0 mg IMat anterolateral thigh
VitaminK
• To prevent hemorrhagic disease of the newbor
• Bacillus Calmette-Guerin (BCG):0.05 mL intradermally
• Hepatitis B vaccine (0.5 mL)
Vaccinations
• Hbig IM (0.5 mL) if mother is HBsAg-positive or if mother's HBsAg
status is unknown and birth weight is < 2 kg
Source:WHO.EarlyEssential
Newborn
CareClinicalPractice
Pocketguide.Geneva,Switzerland: WHO:2014
WHO.Newborncareuntil the firstweekof life. Geneva:WHO;2009

NEWBORN SCREENING (NBS)

• Mandated by RA #9288 "The Newborn Screening Act of 2004"
• Collection of blood is done by heel-prick method

I. TIMING OF NEWBORN SCREENING

• Should be done after 24 hours of life, but not later than 3 days of age (if blood was
collected <24 hours old, must repeat at 2 weeks old)
• For preterm infants: ideal time should be at 5-7 days old
• If newborn is placed in intensive care, test by 7 days old
• Can be done until 1 month old for really sick babies

II. FACTORSTHAT MIGHTAFFECT NBS RESULT

• Blood transfusions
• Dialysis
• Parenteral use of antibiotics
• Prematurity
• Patient not fed since birth

171
III. DISEASES SCREENED IN THE EXPANDED NEWBORN SCREENING
DISORDERS SPECIFIC DISEASES
• Congenital Hypothyroidism
Endocrine
• Congenital Adrenal Hyperplasia
• Homocystinuria
• Hypermethioninemia/Methionine
Amino acid • Adenosine Transferase Deficiency
disorders • Maple Syrup Urine Disease
• Phenylketonuria
• Tyrosinemia Type I, II, III
• Carnitine Palmioyltransferase I & II Deficiency
• Carnitine Uptake Deficiency Glutaric Acidemia Type II
Fatty acid
• Long Chain Hydroxyacyl-CoA Dehydrogenase Deficiency
disorders
• Medium and Very Long Chain-Acyl-CoA Dehydrogenase Deficiency
• Trifunctional Protein Deficiency
• 3-Methylcrotnyl CoA Carboxylase Deficiency
• Beta Ketothiolase Deficiency
• Glutaric Acidemia Type I
Organic Acids • lsovaleric Acidemia
• Methylmalonic Acidemia
• Multiple Carboxylase Deficiency
• Propionic Acidemia
• Citrullinemia
Urea cycle defects
• Argininosuccinic Aciduria
• Alpha & Beta Thalassemia
Hematologic • Hemoglobin C, Hemoglobin D, Hemoglobin E
• Sickle Cell Disease
• Galactosemia, Cystic Fibrosis Biotinidase Deficiency
Others
• Glucose-6-Phosphate Dehydrogenase Deficiency,

NEWBORN HEARING LOSS SCREENING

• Republic Act No. 9709: The Universal Newborn Screening and Intervention Act of2009
All infants shall undergo newborn hearing loss screening on or after 24 hours after
birth, before discharge
Infants who are not born in hospitals should be screened within the first three months
If hearing loss is detected, audiologic evaluation should be made before 6 months

I. RISK FACTORS FOR HEARING LOSS

• Family history of hearing loss
• TORCH infection
• Birth weight <1500 grams
Bacterial meningitis
• Hyperbilirubinemia needing exchange transfusion
• Low APGAR (0-6 at 1 min/Smin)
• Mechanical ventilation
• Neurodegenerative disorders
• Trauma

II. PROCESSOF SCREENING

• Measures electrical signals (electrophysiologic responses) from
Auditory Brainstem hearing pathways of brain in response to sounds
Response (ABR) • For screening high risk neonates from NICU who are at risk for
auditory neuropathy
Otoacoustic Emission • Detects physiologic response of hair cells in cochlea (sensory
(OAE) hearing impairment)
Source:Republicof ThePhilippines,
DOH.UniversalHearingScreeningandIntervention
act of 2009.DOH:2009

172
 SECTION THREE
N'EONATAL RESUSCITATION

NEONATAL RESUSCITATION
• Done to prevent the morbidity /mortality associated with hypoxic-ischemic tissue injury
and to reestablish adequate spontaneous respiration and cardiac output
• At the delivery of a newborn, the first step is immediate thorough drying and quick
assessment for thirty seconds
• The most important initial question to ask is "ls the baby apneic/gasping or limp"? - this
will differentiate a baby who will receive EINCand those that will be needing resuscitation
• Preparation is important:
Assessment of intrapartum and antepartum risk factors
Preparation of materials and personnel knowledgeable in newborn resuscitation
Teamwork and communication during resuscitation

Newborn Resuscitation Algorithm: Local guidelines of the Philippine Society of

Newborn Medicine. NRPH+ (Newborn Resuscitation Philippines Plus)

Birth
'-----'
U ~
I ...-:
I. Immediate
thorough
drying and
i quick
j
!
Apnea/
gasping or
limp
NO
Small baby?
I
I________________
'
assessment
:
YES

YES
j 1. Callfor help :
j 2. Changewet linen
: 3. Clamp and cut the cord
i 4. Transfertowarmer
! S. Position airway
: 6. Clear secretionsif needed

: 7.PPV ··-·-·-· f ...·-··---·-----:

: 8.Sp02 _________________________
;
Within 60 seconds

Stal
Labored breathing NO Post-resuscitation
Apnea / gasping or
or persistent care
HR<l00bpm
cyanosis Transport
Mask adjustment Team debriefing

Reposition Airway

Suction mouth and

nose

Open mouth

Pressureincrease

Airway alternative

lmin: 60-65%
2min: 65-70% HR<60bpm
3min; 70-75%
4min:
Smin:
75-80%
80-85%
·--~Esi
14. IV epinephrine
lOmin:85-05% 15. Consider hypovolemia
16. Consider pneumothorax

Resuscitation
shouldproceedrapidly.Onehasapproximately30 seconds(or60secondsif chestcompressions)
to achievea response
fromonestepbeforedecidingwhetheryouneedto moveon to thenext. Evaluation anddecision-making
arebasedpnmarilyon
respiralions,
heartrate,andoxygenation
(bypulseoximetryprimanly;colorin previousprotocols)

173
RESUSCITATIVE EFFORTS

• Maintain normal temperature and dry the infant

• Position the infant in a "sniffing" position to open the airway
Initial steps of
1 • Clear secretions if needed with a bulb syringe or suction catheter
stabilization
• Stimulate the infant by gently rubbing the back or by flicking the
soles of the feet
• The most important and effective action in neonatal resuscitation
is to ventilate (heart rate of newborns is dependent on
ventilation)
• Infants with secondary apnea will not respond to stimulation
and will require positive pressure ventilation (PPV), which will
improve/increase the heart rate.
• If despite adequate PPV the neonate is still apneic/gasping or
Ventilate and heart rate is< 100 bpm, ventilation corrective steps should be
2
oxygenate performed:
0Mask adjustment
0Repositioning of airway and suctioning of secretions
0Opening of the mouth to check for obstruction
0Pressure increase and alternative airway (e.g. intubation)
• Intubation is strongly recommended prior to beginning
chest compressions and can be considered at any step of the
resuscitative efforts
• Indicated when the HR remains <60 bpm after at least 30 seconds
of adequate PPV
• Administered for 60 seconds (as of January 1, 2017, this has been
extended to 60 seconds and the 2-finger technique has been
Chest dropped from the protocol)
3
compressions • Chest compressions are closely coordinated with ventilation, in
contrast to ACLSand PALS
• Compression rate is 90 compression per minute and the breathing
rate is 30 breaths per minute (total of 120 events per minute, with
a ratio of3:1)
• Indicated if the newborn's HR remains <60 bpm after at least 30
seconds of PPV and another 60 seconds of chest compressions
coordinated with PPV using 100% oxygen
Epinephrine • Dose:
0.1 to 0.3 mL/kg of 1:10,000 concentration via umbilical vein, or
0

0.5 to 1 mL/kg of 1:10,000 concentration via endotracheal route

0

4 for rapid administration

• Volume expansion during resuscitation is indicated when baby is
not responding despite resuscitative efforts and baby appears to
Volume be in shock, especially in the setting of fetal blood loss
expanders • Recommended fluids are plain NSS or type O Rh-negative blood
• Dose is 10 mL/kg via umbilical vein over 5-10 minutes
• Ringer's Lactate is no longer recommended
Source:WHO.Newborncareuntilthefirstweekof life.Geneva:WHO;2009
(6thEd).ElkGroveVillage;2011
AAP.Textbookof NeonatalResuscitation
J. et al.2015International
Perlman Consensus onCardiopulmonary 2015
Pediatrics:
Resuscitation.
HeartAssociation
WyckoffM, et al. 2015American GuidelinesUpdatefor Cardiopulmonary Pediatrics;2015
Resuscitation.

174
 SECTION FOUR
BREASTFEEDING

OVERVIEW OF BREASTFEEDING
WHO/UNICEF global strategy on infant and young child feeding (2002) recommends
exclusive breastfeeding (i.e., no other food or drink, even water) for the first 6 months
• Breastmilk is the most appropriate nutrition for the infant. It is uniquely adapted for the
infant's needs for growth and development

I. ADVANTAGESOF BREASTFEEDING
• Most appropriate food for the infant
• No risk of contamination
• Cost-effective
• Protection against colic, atopic dermatitis, GI bleeding and other diseases
• Decreases incidence of diarrhea, UTI, respiratory tract infection, CNS infections, middle
ear infections, necrotizing enterocolitis, sepsis and botulism

II. BREASTMILKCOMPOSITION

WITHIN A FEED REMARKS

I
• Released at start of feeding
Foremilk • Watery
• High lactose and high protein content
• Towards end of feeding
Hindmilk
• Creamy with high fat (Sx fat content of foremilk)

Ill. STAGESOF LACTATION

STAGE DESCRIPTION
• Colostrum is the earliest breast milk produced during
pregnancy and is secreted in the first 3 days after delivery
• Characteristics
0 37-84 ml/day produced in the first 2 days of life
0 80% water
First 0-7 days
0 Protein-rich
0 High concentration of secretory IgA & protective factors:
lactoferrin, lysozyme
° Contains vitamin A,E,K& growth factors
0 Low levels of fat & carbohydrates
• Between colostrum & mature milk
Transitional Milk
• Rising levels of macronutrients
• Day 10-14 oflife
Mature Milk
• Same as colostrum content+ high fat & lactose
• Produced when breastfeeding frequency decreases
• Reverts to being more like colostrum with a high concentration
Involutionary Milk
of immune factors
• Relatively low content of water; fat and lactose

175
IV. PHYSICALASPECTS
WITHIN A FEED REMARKS
• Curds are the semi-solid fraction which settles out when milk
is clotted, and whey is the clear fluid which remains. Curds are
made from the casein proteins.
Curds/whey • Human milk has low casein:whey ratio
10:90 in early milk
0

40:60 in mature milk

0

50:50 in late lactation

0

• Bound by membranes rich in:

Fat globules Phospholipids: substrates for cell growth and brain development
0

° Cholesterol: facilitate myelination of the CNS

V. COMPOSITIONOF BREAST MILKVS FORMULA

COMPONENT REMARKS BREASTMILK FORMULA

Bioactive Comp61!f;!lts
"'
} - ,.,,, .,,, .,~-. '"--"'
• Protection against specific
Maternal
secretory IgA antigens + -
Maternal WBC • For phagocytosis of pathogens
+ -
Lysozyme • Lysis of bacteria
+ -
• Inhibits binding of pathogens
Milk lipids to host cells and causes lysis
of enveloped pathogens
+ Less effective

Oligosaccharides • Inhibits pathogen binding

+ -
• Wide range of synergistic
protective properties (e.g.,
bactericidal & antiviral Some have
Lactoferrin
activity and enhances + bovine
lactoferrin
epithelial growth & recovery
of intestinal mucosa)
,-,Maturative .t:ompoftenti' 7,
•t )<'
,,.:, .
__,:
'" . .·,..,
'"'" - c-:·::t'
• ; i'i}'' "jc'r.}
,- ,_c,_
'ci:_
>

Epidermal growth
factor
• Promotes maturation of gut
+ -
• Mediators necessary to mount
Cytokines an antibody defense against
foreign organisms
+ -

176
 Bile salt
stimulated lipase
(BSSL)
• For fat digestion +
• Palmitic acid, Oleic acid,
Linolenic acid,
Docosahexaenoic acid (DHA), Complete
Essential fatty Fewer and less
Linoleic Acid, Arachidonic acid and highly
acids bioavailable
• Wide function of activity such bioavailable
as CNS development and
retina development

• Casein High solute

Easily digested, load, harder to
Proteins • Whey
less allergenic digest, more
• Mucins allergenic
• Nitrogen-containing
carbohydrate that promotes
Bifidus Factor
colonization of the gut
by Lactobacillus bifidus,
encourages growth of the
normal intestinal flora
+
I
• As a general rule, higher micronutrient contents in breastmilk than
formula.
Micronutrients • Exception: vitamin D, vitamin Kand iron is higher in formula
(however, for these micronutrients, the bioavailability is higher in
breastmilk)

VI. SIGNS OF ADEQUACY OF BREASTMILK SUPPLY

• Infant is satisfied after each nursing period
• Infant sleeps for 2-4 hours after
• Infant gains weight adequately: 25 g/day for the first 3 months
• Infant wets at least 6 diapers/day with pale yellow urine, 2-3 stools/day

VII. COMMON PROBLEMS DURING BREASTFEEDING

PROBLEM MANAGEMENT
Inverted nipples • This problem clears itself during pregnancy as breasts become larger
• Keep warm and dry
• Wash with water, not soap
Cracked nipples
• Apply lanolin
• Prevent engorgement through frequent feeding/ expression of milk
• Treat with antibiotics
Mastitis • Apply warm compress
• Continue frequent nursing on affected breast to prevent engorgement

VIII. CONTRAINDICATIONS TO BREASTFEEDING

• There are few true contraindications to breastfeeding
• Fever and minor maternal illness is NOT a contraindication (in fact, the infant will benefit
from the mother's developing immunity if breastfeeding is continued)
• Few medications are contraindicated in breastfeeding, alternative choices are available
for most cases

177
 • Refrain from breastfeeding and infant contact until treated
Tuberculosis
• No longer considered contagious after 2 weeks of treatment
Herpetic lesions on the • Avoid breastfeeding until active lesions have resolved
breast • Vaginal herpes is NOT a contraindication
• Express milk until lesions are crusted over. Administer varicella
Varicella
immunoglobulin to infant
• If safer alternatives are available, HIV infected mothers should not
breastfeed
Human
• WHO recommends breastfeeding in HIVinfection endemic areas
immunodeficiency
unless safe infant formula is readily available. The risks associated
virus (HIV)
with formula feeding in developing countries is greater than the risk
of acquiring HIVinfection with breastfeeding
Use of illicit drugs • Absolute contraindication
• Seek specialty consultation
Cancer chemotherapy
• Generally contraindicated for the duration of treatment
or radiation treatment
• Expressed breastmilk should be discarded during this time

Galactosemia • Absolute contraindication

Inborn errors of
metabolism (e.g.
• Specialty consultation regarding specific metabolic defects
phenylketonuria, maple
syrup urine disease)

IX. BREAST MILKEXPRESSIONAND STORAGE

• All milk should be dated before storing (e.g., first in, first out)
• Store milk in small amounts to reduce wastage
• Previously frozen milk that has been thawed may be kept in the refrigerator for up to 24 hours

A. Milk Storage Guidelines

STORAGE TEMPERATURE DURATION
Room temperature • 16 to 29°C • 4 hours
Insulated cooler bag • -15to4°C • 24 hours
• 4 days optimal
Refrigerator • 4°C
• 5 days acceptable
Freezer (1 door refrigerator) • -lS'C • 2 weeks
Freezer (2 door refrigerator) • -18°C • 3-6 months
Deep freezer • -20°C • 6-12 months

B. Options in Thawing Frozen Milk:

Thaw in refrigerator overnight
Run warm water over sealed container
0 Place frozen container in cup of warm water
Use waterless warmer
DO NOT boil or microwave

Source:
Andreas
N, et al. Humanbreastmilk:A reviewonitscomposition andbioactivity.
EarlyHumanDevelopment; 2015
WuX, et al. HumanMilkNutrientComposition in the UnitedStates:CurrentDevelopmentsIn Nutrition;2018
GleasonC, et al.Avery'sDiseasesof the Newborn(9thed.).Elsevier;2012
Academyof Breastteeding Medicine.HumanMilkStoragelnfomnalion. BreastteedingMedicine;2018
CDC.ProperHandlingandStorageof HumanMilk;2015and2017

178
 SECTION FIVE
APPROACH TO COMMON PROBLEMS OF THE NEWBORN

APPROACH TO APNEA
I. DEFINITION OF TERMS

TERM DEFINITION
• Normal respiratory pattern in neonates
Periodic breathing • Brief episodes of respiratory pauses 5-10 seconds followed by rapid
respirations 50-60 breaths/min lasting for 10-15 seconds
• Absence of the brainstem stimulus to breathe
Central apnea
• No respiratory effort

• Absence of airflow into lungs due to an obstruction by mucus or airway

Obstructive apnea
collapse

• Airway obstruction with inspiratory effmt precedes or follows central

Mixed apnea

Severe apnea
apnea

• Absence of breathing for >20 seconds or any duration if associated

with signs of cardio-respiratory compromise (e.g. oxygen desaturation,
cyanosis or bradycardia <100 bpm)
I
• This is due to physiologic immaturity ofrespiratory control
Apnea of • Presents on 2nd to 7th day oflife
prematurity • Inversely correlated with gestational age & birthweight (90% of infants
with birthweight of <1000 grams)
• Occurs as a result of oxygen deprivation in a fetus/neonate
Primary apnea
• Responds to stimulation and oxygen supplementation

• Occurs when oxygen deprivation/hypoxia continues

Secondary apnea • Cannot be reversed by stimulation
• Assisted ventilation is necessary

II. ETIOLOGYOF APNEA

SYSTEM EXAMPLES
• Asphyxia, hemorrhage, meningitis, increased intracranial pressure (ICP),
CNS
seizure, congenital neuropathies & malformations, encephalopathy
• Hypoxia, airway obstruction and malformation, lung disease,
Respiratory surfactant deficiency, pulmonary hemorrhage, hypercarbia

• Congestive heart failure, patent ductus arteriosus, congenital cyanotic

Cardiovascular heart disease, heart block, hypovolemia, hypotension, hypertension,
increased vagal tone

Gastrointestinal • Necrotizing enterocolitis, gastroesophageal reflux, feeding intolerance

Hematologic • Anemia, polycythemia

• Temperature instability, hyper /hypothermia

• Sepsis, metabolic/electrolyte imbalance, inborn errors of metabolism,
Other disorders
vagal reflex after orogastric tube insertion, feeding or suctioning, acute
pain, neck flexion, drug over-sedation, magnesium sulfate
Source:GomellaTL,et al. Neonatology 7thEdition.McGraw-Hill;2013
KliegmanR, et al. NelsonTextbook (21sted.).Elsevier;2020
of Pediatrics
2012
Care(7thed.).Philadelphia;
ClohertyJ., et al. Manualof Neonatal

179
III. DIAGNOSTICS
• ABGto rule out hypoxia and/or acidosis
• CBCwith differential count to check for infection, anemia, polycythemia
• Cultures
• Electrolytes: Na, Ca, Mg, glucose
• For inborn errors of metabolism: organic acid levels, amino acid profiles, ketones in urine
• Chest radiograph
• ECG
• Abdominal radiograph
• Ultrasonography of head if suspecting hypoxic ischemic encephalopathy

IV. MANAGEMENT
• See neonatal resuscitation
• Tactile stimulation with supplemental oxygen (1-2 LPM via
Emergency treatment nasal cannula)
• Bag-mask ventilation or intubation if necessary
• Once stabilized, work-up for suspected etiology
Determine cause • Rule out treatable causes before diagnosis and treatment of
of apnea & start
treatment if possible apnea of prematurity

• Keep patient thermoregulated

• Infant positioning: avoid neck flexion or extension to keep
Non-pharmacologic
treatment airway patent, prone position reduces apnea with head and neck
elevated 15 degrees
• Maintain nasal patency
• Methylxanthines (e.g., theophylline) or caffeine for idiopathic
apnea of prematurity
0Increases central respiratory drive by lowering threshold for
Pharmacologic hypercapnia
management 0Enhances diaphragmatic contractility
0Reduces diaphragmatic fatigue
• Caffeine is preferred because of its longer half life and lower
potential for side effects (feeding intolerance and tachycardia)
Source:GomellaTL,et al. Neonatology
7th Edition.McGraw-Hill;
2013
KliegmanR.et al. NelsonTextbook
of Pediatrics
(21sted.).Elsevier:2020

APPROACH TO BLOODY STOOL

• Bloody stools in neonates are often benign and self-limiting
• It is important to detect cases that have significant underlying pathology

• Dark colored stools as a result of swallowed maternal blood

• Apt test: positive if blood is due to gastrointestinal or pulmonary
Findings that suggest
benign causes bleeding from the neonate. Negative: suggests it is from
swallowed maternal blood
• Isolated microscopic occult blood
• Hematochezia, signs of bowel obstruction, signs of infection,
melena, constipation, hard stools with fissure, hematologic co-
Findings that suggest morbidities
pathologic causes • Formula intolerance/dietary protein intolerance (cow's milk or
soybean formula): mucoid bloody diarrhea on the 2nd to 3rd
week of life

Source:GomellaTL,et al. Neonatology

7thEdition.McGraw-Hill;2013
KliegmanR, et al. NelsonTextbook
of Pediatrics
(21sted.).Elsevier:2020
Aver;GB,et al.Aver;'sneonatology7thEdition.Philadelphia;
2016

180
APPROACH TO ABNORMAL STOOL PATTERNS
• 99% of term infants, 100% of post-term infants, and 76% of preterm infants should pass
stool in the first 24 hours of life
• Infants have a mean of 4 stools per day during the first week of life, which gradually
decreases to 1.7 per day at 2 years
• The first passage of meconium is a marker of normal gastrointestinal function
• Delayed meconium passage may occur due to gestational immaturity, severe illness, or
bowel obstruction (this may predispose the infant to bowel perforation)

Common Etiologies of Abnormal Stool Patterns

MANIFESTATIONS
1) Meconi um Plug Syndrome
• Transient obstruction
of the lower colon and
rectum due to impacted
meconium
• Associated with increased
incidence of Hirschsprung
DIAGNOSTICS
• Plain radiograph findings:
0 Distended bowel
0 No air fluid levels
0 Distal obstruction
with absence of air in
rectum
MANAGEMENT
• Verify by contrast enema
then do repeated water
soluble enemas every 4-6 hours
• If ineffective: acetylcystein
enema to break down
meconium plug & eventually

I
disease pass out meconium
• If abnormal stools recur, rule
out Hirschsprung disease
2) Small Left Colon Syndrome
• Due to transient • Plain radiograph findings: • Contrast enema is diagnostic
dysmotility in descending 0 Dilated intestinal loops and therapeutic
colon among term infants 0 Air-fluid levels • Surgery is indicated for
• 50% have mothers with recurrence or perforation
diabetes mellitus
3) Hirschsprung Disease
• Delayed passage of • Barium Enema • Surgical correction
meconium • Rectalbiopsy (gold standard)
4) lmperforate Anus
• Delayed passage of • Radiographic studies • Insert double-lumen NG
meconium oflumbosacral spine, tube for decompression
• Intestinal obstruction urinary tract • Rule out other congenital
• Spinal UTZ/MRIto rule out anomalies
tethered cord syndrome • Pediatric surgical consultation
5) Duodenal Atresia
• Bilious vomiting usually • Double bubble sign on • Decompression
noted on the 1st day of life plain radiograph • Surgical repair
• No abdominal distention
6) lieus
• Signs of infection • Septic/metabolic work up • Antibiotics
• Electrolyte derangement • Radiographs • Treat and correct causes
7) Other Causes of Abnormal Stool Pattern
• Prematurity /low birth weight: due to immature colon • Identify and treat causes
and delayed first feeding • Prematurity: conservative
• Electrolyte abnormalities: hypokalemia, hyponatremia, treatment for infants not
hypercalcemia and hypermagnesemia vomiting. Low osmolality
• Maternal medications: magnesium sulfate, opiates,
water-soluble contrast
neuroleptics, antidepressants
enema for passage of stool
• Drugs: theophylline, opiates, narcotic analgesic therapy
• Hypothyroidism

181
APPROACH TO RESPIRATORY DISTRESS
{details discussed in Pulmonology Chapter)

RESPIRATORY TRANSIENT PERSISTENT MECONIUM

DISTRESS TACHYPNEA OF PULMONARY ASPIRATION
SYNDROME THE NEWBORN HYPERTENSION SYNDROME
(RDS) (TTN) (PPHN) (MAS)
Definition

• Pulmonary
• Deficiency in • Disorder resulting
hypertension that • Aspiration of
surfactant, leading from delayed
causes hypoxemia meconium-stained
to respiratory clearance of fetal
from right to left amniotic fluid
distress alveolar fluid
shunting of blood
Profile and Manifestations

• Term or post term

• Preterm or term
• Term or post term infant
• Preterm • Delivered via
• Meconium aspirated • Meconium-stained
caesarian section
amniotic fluid
• Within first 12
• Respiratory
• Early onset hours of birth • Early onset
distress with
• Usually relieved • Cyanosis respiratory
prominent
with minimal 0 2 • Oxygen gradient distress with
grunting within
supplementation (preductal > asphyxia
minutes of birth
postductal)
Natural Course

• Progressive
worsening of
cyanosis & dyspnea • Recovers rapidly
• Unpredictable course
• Symptoms peak within 3 days
within three days
then improves
Chest Radiograph Findings

• Ground glass • May be normal

• Hyperaerated lungs • Widespread, coarse,
opacities (depends on
• Prominent asymmetric,
• Underaerated lungs comorbid
vascular markings patchy infiltrates
• Atelectasis conditions)
Overview of Management

• Surfactant
replacement • Oxygen
• Positive pressure supplementation,
• Treat underlying
ventilation ( e.g., • Supplemental intubation, and
cause
CPAP,mechanical oxygen support suctioning
• Supportive care
ventilation) • Supportive care
• Prevent with
antenatal steroids
Source:KliegmanR, et at NelsonTextbookof Pediatrics(21sted.).Philadelphia:
2020
ClohertyJ. et al. Manualof NeonatalCare(7thed.).Philadelphia:
2012

182
APPROACH TO ANURIAAND OLIGURIA
• All healthy preterm, full-term and post-term infants void by 24 hours of age
• Oliguria: urine output <l mL/kg/hour for 24 hours
• Anuria: absence of urine output by 48 hours of age

I. DIFFERENTIALS FOR ANURIA/OLIGURIA

POSSIBLE
CLINICAL CLUES
ETIOLOGY
Dehydration • Check for hydration status (e.g., BP,HR,physical exam), oliguria, renal function
Obstruction • Bladder distention
• Potter facies: low set ears, inner canthal crease, flattened face due to
severely decreased amniotic fluid secondary to renal disease
• Dysmorphic features suggestive of renal disease: single umbilical artery,
Renal failure hypospadia, anorectal abnormalities, vertebral anomalies, abnormal ears,
esophageal atresia
• Urinary ascites (rupture of bladder and renal calyces) with posterior
urethral valves
Maternal drug
intake during
pregnancy

Maternal diabetes
mellitus
• ACEinhibitors & NSAIDs interfere with fetal nephrogenesis and result in
renal tubular dysgenesis and acute kidney injury in neonates

• Increased risk for renal anomalies in the infant: renal agenesis,

hydronephrosis, ureteral duplication
I
II. DIAGNOSTIC APPROACH
A. General Examination
DIAGNOSTICS FINDINGS
Creatinine, BUN,and • Used to define acute renal failure/injury
electrolytes • For evaluation of renal function
• Abnormal in sepsis
CBCand platelet
• Thrombocytopenia or polycythemia seen in bilateral renal vein
count
thrombosis
• Normal in prerenal disease and urinary tract obstruction
• RSC, tubular cells, proteinuria suggest intrinsic renal disease
Urinalysis • Erythrocyte casts are seen in glomerulonephritis
• Protein in urine indicate glomerular disease
• Epithelial casts & brown granular casts in acute tubular necrosis
Arterial blood pH • Metabolic acidosis in hypovolemia and hypoperfusion states

8.lmaging
IMAGING FINDINGS
• Evaluate urinary tract obstruction, congenital disorders or
Renal
vascular abnormalities
ultrasonography with
• Doppler examination of renal blood flow: may suggest renal
doppler flow studies
vascular thrombosis
Abdominal radiographs • Can suggest ascites or possible masses

Voiding • If bladder outlet obstruction is suspected, can rule out

cystourethrography vesicoureteral reflux

Radionuclide renal
• Evaluate kidney function
scanning

183
Ill. MANAGEMENT

• Bladder catheterization
• Fluid challenge for diagnosis and initial management in infant
without evidence of heart failure or volume overload:
Initial evaluation 0 10-20 mL/kg NSS IV over 1-2 hours. Repeat once ifno response.
of suspected renal 0Increase in urine output 2:l ml/kg/hour indicates prerenal cause
failure • Discontinue or restrict potassium in IV fluids
• Evaluate medications, adjust dose if necessary, discontinue
nephrotoxic medications
• Strict I &O monitoring, weigh infant every 12 hours
Management • Depends on etiology of oliguria/anuria
Source:GomellaTL. et al. Neonatology
7th Edition.McGraw-Hill; 2013
AveryGB.et al.Avery'sneonatology 7th Edition.2016

APPROACH TO NEONATAL SEIZURES

I. SEIZURESVERSUSJITTERINESS
SEIZURES JITTERINESS
• Paroxysmal alterations in neurologic function • Stimulus sensitive, with tremors as the
(behavioral, motor, autonomic function) dominant movement
• Cannot be stopped by manipulation • Can be stopped by gentle flexion

II. FOURTYPES OF NEONATALSEIZURES

SEIZURE TYPE CHARACTERISTICS
• Common among preterms
• Consist of tonic horizontal eye deviation, with or without jerking,
Subtle Seizure
eyelid blinking, sucking, smacking or drooling, "swimming," "rowing"
or "pedaling" movements, and apneic spells
• Infant is conscious during and after the seizure
• Focal seizures: well localized, rhythmic, slow jerking movements of
Clonic
the face and upper and lower extremities on one side of the body
• Multifocal seizures: involve several body parts in a sequential fashion
• Focal seizures: sustained posturing of a limb, asymmetric posturing
of the trunk or neck
Tonic
• Generalized seizures: tonic extension of extremities or tonic flexion
of upper extremities and extension of lower extremities
Myoclonic • Single or multiple synchronous jerks

APPROACH TO CYANOSIS
CLUES POSSIBLE ETIOLOGY
Signs of respiratory distress • Pulmonary etiology
• Transposition of the great vessels
Cyanosis at birth
and tricuspid atresia
Cyanosis in the perinatal period • Truncus arteriosus, TOF
Cyanosis with feeding • Esophageal atresia
Cyanosis that disappears with crying • Choanal atresia
Cyanosis improved by crying • Pulmonary
Cyanosis worsened by crying • Cardiac
Cyanosis with normal pulse oximeter reading • Methemoglobinemia

184
III. COMMONETIOLOGIES
A. Perinatal Asphyxia
• Most common cause of neonatal seizures
• Occur within the first 24 hours of life
• Persistence leads to progressive hypoxemia and hypercapnea, and eventually to
hypoxic-ischemic encephalopathy (HIE)
• Commonly presents as generalized tonic clonic seizure in preterms and multifocal
clonic type in fullterms

1. Hypoxic-lschemic Encephalopathy (HIE) Severity

SARNAT SCALE CHARACTERISTICS

Stage 1 • Hyperalertness, normal muscle tone, weak suck, low threshold

(Mild) Moro, mydriasis, and absence of seizures
Stage Z • Lethargic or obtunded, mild hypotonia, weak or absent suck,
(Moderate) weak Moro, miosis, and focal or multifocal seizures
Stage 3 • Stuporous, flaccid muscle tone, intermittent decerebration,
(Severe) absent suck, absent Moro, and poor pupillary light response

2. Management
Prophylactic use of anticonvulsant therapy may reduce the occurrence of death and
I·:
neurodevelopmental disability
Phenobarbital (with therapeutic hypothermia, if indicated) to reduce clinical seizures

B. Other Causes of Neonatal Seizures

ETIOLOGY REMARKS
• Occurs as a result of hypoxic insult
• Primaty subarachnoid hemorrhage: seizures occur on 2nd postnatal day
• Periventricular or intraventricular hemorrhage arise from sub-
lntracranial
ependymal germinal matrix: present as subtle seizures, decerebrate
hemorrhage
posturing or generalized tonic seizures
• Subdural hemorrhage over cerebral convexities: present with focal
seizures and focal cerebral signs
• Electrolyte abnormalities (See Fluids and Electrolytes chapter):
hypoglycemia, hypocalcemia, hyponatremia, hypernatremia
Metabolic
• Pyridoxine dependency (seizures resistant to anticonvulsants)
disturbances
• Amino acid disorders: seizures commonly occur with
hyperammonemia and acidosis
Infections • See Infectious Disease chapter

Drug • Maternal drug intake of heroin, methadone, propoxyphene, sedative

withdrawal hypnotics, alcohol, antidepressants
• Inadvertent injection of anesthetics during delivery
Others
(paracervical, pudenda!, saddle block anesthesia)

IV, DIAGNOSIS
DIAGNOSTICS REMARKS
Complete blood count • Rule out infection and polycythemia
Serum chemistries • Glucose, calcium, sodium, BUN,magnesium, blood gases
CSFfluid analysis • As needed if suspecting infectious etiology of seizures
Imaging • Cranial ultrasonography, CT scan or MRI
• EEG
Others • Blood ammonia levels
• Amino acids

185
V. MANAGEMENT
MANAGEMENT REMARKS
• Initial drug of choice
Phenobarbital* • LD:15-20 mg/kg in single or divided doses (not to exceed 1 mg/kg/min)
• After 12-24 hours, begin maintenance: 3-4 mg/kg/day PO or IVonce daily
• LD: 15-20 mg/kg IVat a slow rate not to exceed 0.5 mg/kg/min
Phenytoin* • After 12 hours, begin maintenance: 4-8 mg/kg/day PO or IV divided
every 12 hours
• 0.2-0.5 mg/kg/dose IVas needed for seizures
Diazepam
• 0.3 mg/kg/hour as continuous infusion considered if seizures persist
• Correct underlying etiology
• Trial of pyridoxine (B6) if seizures still persist: 50-100 mg IV with
Others
EEGmonitoring
• Therapeutic hypothermia
therapy(phenobarbital
•Anticonvulsant loadingdosescontrol85%ofneonatalseizures)
and phenytoin
2013
71hEdition.McGraw-Hill;
Source:GomellaTl, el al. Neonalology
(21sted.).Elsevier;2020
of Pediatrics
KliegmanR. el al. NelsonTextbook
2016
71hEdition.Philadelphia;
AveryGB,el al.Avery'sneonalology
Engorn,8., & Flerlage,J. (2015).TheHarrie!lane Handbook Elsevier
(20lhed.).Philadelphia:

APPROACH TO JAUNDICE
I. OVERVIEW OF JAUNDICE
• Jaundice is the accumulation of yellow-orange pigment bilirubin in the skin, sclera & mucosa
• It is the most common transitional finding in the newborn period (60-70% oftenn &
80% of preterm infants)
• Becomes clinically apparent when serum bilirubin concentration is.: 5mg/dL
• Neonatal hyperbilirubinemia in infants ;,35 weeks gestational age is defined as total
serum or plasma bilirubin (TB) >95th percentile on the hour-specific Bhutani nomogram
40th Percentile
75th Percentile
97th Percentile

- -- ---
.--- ----
20 342

High ,
-
t11gnI ~termeu1;,te

::r
~
.S,10
15

,'/
, .-
, ,-
~
- ~
~
Lowi ~termedi Me
257

171
~
0
E
e
'1//
- -~ ~"

----
Ill
I-
C/)
Low
5 85

0 0
0 12 24 48 72 96 120 144

Postnatal age (hours)

Riskzonesare designatedaccordingto percentile:

High(TB;e95th)
Highintermediate (95th>TB;,?5th)
Lowintermediate (75th>TB;e40th)
Low(TB<40th)
significant
ofclinically
Infantswithvaluesinthe highriskzoneare at increasedriskforthe development hyperbiliru-
binemiaandrequireintervention. 1999
Source:BhulaniVK.el al. Pedialrics;

186
II. PATHOPHYSIOLOGY
• Bilirubin is the end product of heme catabolism derived primarily from the breakdown of
red blood cell hemoglobin
• Physiologic jaundice is the most common cause of jaundice in healthy infants
• There are two forms of circulating bilirubin: unconjugated bilirubin and conjugated bilirubin

FORM REMARKS
• Water-soluble (does not cross blood-brain barrier)
Conjugated • Elevated levels are always pathologic
Bilirubin • Conjugated bilirubin is considered elevated if it is:
(Direct) 0>l mg/dL for total bilirubin levels ~5 mg/dL, or
0>20% of the total bilirubin for total bilirubin levels >5 mg/dL
• Lipid soluble (may cross blood brain barrier) & predominantly bound
to albumin
Unconjugated • Kernicterus or bilirubin encephalopathy (clinical diagnosis):
Bilirubin 0 Most dreaded complication of unconjugated hyperbilirubinemia
(Indirect) ° Caused by the accumulation of unconjugated bilirubin in the basal
ganglia and brainstem nuclei
0 Rare in healthy children with bilirubin levels <20 mg/dL

Ill. PHYSIOLOGICVERSUS PATHOLOGICJAUNDICE

Source:Kliegman
R, et al. NelsonTextbook
AAPSubcommittee
of Pediatrics
(21sted.).Elsevier;
onHyperbilirubinemia.
2020
Pediatrics;
2004
I
FORM PHYSIOLOGIC JAUNDICE* PATHOLOGIC JAUNDICE
• Visible on the 2nd to 3rd day • Presents in the 1st 24-36 hours
Onset
of life of life
Increase in total • Does not increase by >5 mg/ • Increases by >0.2 mg/dL/hr or
bilirubin dL/day >5 mg/dL/day
• TB increases to >12 mg/dL in
Peak total
• TB peaks at around 12 mg/dL full-terms, or 10-14 mg/dL in
bilirubin (TB)
preterms
Direct bilirubin • < 2 mg/dL • >2 mg/dL
• Does not persist beyond
10-14 days • Persists beyond 10-14 days
Duration
• Usually decreases to< 2 mg/dL after birth
at the 5th to 7th day of life
'Physiologicjaundiceis a diagnosisof exclusion(i.e.onlyonecriteriaof pathologic
jaundiceneedslo be fulfilledlo labelthe patientas
havingpathologicjaundice)

Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Elsevier;2020

187
IV. DIFFERENTIAL DIAGNOSIS FOR JAUNDICE

Hyperbilirubinemia

Direct Indirect

.J
CholestaticJaundice

COOMB'S Negative COOMB's Positive

"
INCREASEDHb Normal/Low Hb

...........
i...........
.
Polycythemia
Infant of diabetic mother
Increased Reticulocytes Normal Reticulocytes
SGA
Delayed cord clamping
Twin transfusion/
Characteristic RBC
Maternal-fetal transfusion
Morphology

.........
J
Spherocytosis
Elliptocytosis

Source:Taeusch
HW,et al. SchafferandAvery'sdiseasesof thenewborn.
Philadelphia;
1991

A. Breastfeeding and Breastmilk Jaundice

BREASTFEEDING- BREASTMILK JAUNDICE

ASSOCIATED JAUNDICE SYNDROME
• Jaundice resulting from an • Associated with one or more
Definition
insufficient intake of milk abnormalities in the maternal milk
• 3rd to 4th day oflife • Onset after 7th day of life
Onset • Occurs in 13% of breastfed infants • Extension of physiologic jaundice
• Lactation failure jaundice beyond 1st week oflife
• Decreased milk intake with
• Presence of glucuronidase in
Factors dehydration
some breastmilk
• Reduced caloric intake
Duration • Few days • 3 weeks to 3 months
• No therapy needed if serum
• Frequent breastfeeding
bilirubin remains< 270 umol/L
(>10/24 hrs)
in healthy fullterm infants
Prevention • Rooming-in with night feeding
• Temporary interruption of
• Discouraging 5% dextrose or
breasfeeding may be indicated if
water supplementation
serum bilirubin >270 umol/L
Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21s1ed.).Elsevier;2020

188
 B. Hemolytic Anemia from ABO and Rh Incompatibility
ABO INCOMPATIBILITY
• Most common cause of hemolytic
disease of the newborn
Description
• Mother is type O and baby is
either A or B
• Most cases are mild
• Jaundice usually during first 24
Presentation
hours of life
• Mild hepatosplenomegaly
• Presumptive diagnosis in the
presence of:
0 ABO incompatibility
Weakly to moderately positive
Rh INCOMPATIBILITY
• Occurs when Rh(+) blood is
infused to a Rh(-) women
• Rarely occurs in first pregnancy
because transfusion of Rh(+)
fetal blood occurs near the time
of delive1y which does not give
enough time for sensitization
• Majority is triggered by the D-antigen
• Mild hemolysis (15%) to
severe anemia
• Jaundice is usually evident on the
1st day of life
• Direct Coombs test positive
• Anemia is generally present
• Increased reticulocyte count with

I
0
polychromasia
Diagnostic direct Coombs test
• Definitive diagnosis:
findings 0 Spherocytes in blood smear
demonstration of blood
• Hemoglobin is usually normal
group incompatibility and
• Increased reticulocyte count
corresponding antibody bound to
• Extensivepolychromasia& increased
the infant's RBCs
numbers of nucleated RBCs
Management • See management ofunconjugated hyperbilirubinemia below

V. DIAGNOSIS
DIAGNOSTICS
Bilirubin levels
(Total, Direct, Indirect)
Blood type and Rh status
of mother and infant
Direct Coombs test
CBCand blood smear
Reticulocytes
Serum albumin
REMARKS
• Total bilirubin level is plotted in the Bhutani chart
• Rule out ABO and Rh incompatibility
• Cord blood can be sent for routine blood typing of the newborn
• Detects antibodies bound to the surface of RBCs
• Positive in hemolytic disease as a result of isoimmunization
• Anemia suggests hemolytic process
• Polycythemia increases risk for jaundice
• RBC morphology: spherocytes suggest ABO incompatibility
• Elevation suggests hemolytic disease, occult or ove,t hemorrhage
• Assess fraction ofbilirubin unbound to albumin in the circulation
• Useful in determination of exchange transfusion

189
VI. MANAGEMENTOF UNCONCONJUGATED
HYPERBILIRUBINEMIA
• The goal of management is to prevent acute bilirubin encephalopathy
• The mainstay in management is phototherapy

MANAGEMENT POSSIBLE
REMARKS
COMPLICATIONS
• Phototherapy converts bilirubin • Bronze baby syndrome
into byproducts that are less toxic (bronze color of urine & skin)
& more easily excreted (process is with photodestruction of
Phototherapy
called photoisomerization) copper porphyrins, causing
• Recommended distance is 20 cm retinal degeneration ( eye
between infant and light source shields must be used)
• Metabolic acidosis
• Electrolyte derangement
• A double volume exchange • Hypoglycemia
replaces 85% of circulating RBC • Hypocalcemia
• Thrombocytopenia
Exchange and decreases bilirubin to half
• Volume overload
transfusion the pre-exchange value
• Arrhythmia
• See discussion below for • NEC
indications • Infection
• Graft-vs-host disease
• Death

Intravenous • Adjunctive treatment for

• Anaphylaxis
immune globulin isoimmune hemolytic disease
• Headache, flushing, chills,
(0.5-1.0 g/kg/dose, • Reducesneed for exchangetransfusion
repeat in 12 hours) myalgia
in isoimmune hemolytic disease
Source:Khegman
R, et al. NelsonTextbook of Ped1atncs
(21sted.).Elsevier:2020
Cloherty, J., et al. Manualof NeonatalCare.Philadelphia:
2012
MP Subcommittee
on Hyperbilirubinemia.
Pediatrics:
2004

A. Phototherapy
Should be initiated when the Serum Total Bilirubin (STB) exceeds the cut-off line for
each risk category in relation to the age of the infant at the time of blood extraction

- Infantsat higherrisk (35-376/7 wk + risk Factors)

- - Infantsat mediumrisk(> or= 38 weeks+ risk factorsor 35-37 6/7 weeksand well)
· · Infantsat lowerrisk> 38 weeksandwell

22
. . .....
342

16.5 257
:r ..I
~ ---
0
171 E
Cl
.§.11 e
tll 0
1-
(/) ~

5.5 V° 85

0 0
Birth 12 24 48 72 96 120 144

Postnatalage (hours}
Source:AAPSubcommittee
on Hyperbilirubinemia.
Pediatrics:
2004

190
 8. Exchange Transfusion
Should be done when the STB exceeds the cut-off line for each risk category in
relation to the age of the infant at the time of blood extraction.
Should be done in:
Evidence of ongoing hemolysis and TB fails to decline despite 4-6 hours of
intensive phototherapy
Rate of increase in TB indicates that the level will reach 25 mg/dL within 48 hours
Early signs ofbilirubin encephalopathy (e.g., hypertonia, arching, torticollis,
opisthotonus, feve1; high-pitched cry)
There is hemolysis causing anemia and hydrops fetalis
- Infants at higher risk (35-37 6/7 wk + risk Factors)
- - Infants at medium risk(> or= 38 weeks + risk factors or 35-37 sn weeks and well)
· · Infants at lower risk ::-38 weeks and well)

30 513

26 445

14./ 291

10 171
Birth 24 48 72 96 120 144

Postnatal age (hours)

on Hyperbilirubinemia.
AAPSubcommittee
Source: 2004
Pediatrics;

APPROACH TO EYE DISCHARGE

I. NEONATALCONJUNCTIVITIS(OPHTHALMIA NEONATORUM)
• Refers to inflammation of the eye presenting as hyperemia and eye discharge during the
first 4 weeks of life
Most common ocular disease in neonates

II. COMMONETIOLOGIESOF OPHTHALMIA NEONATORUM

ETIOPATHOGENESIS/
MANAGEMENT
MANIFESTATIONS
1) Chemical/Inflammatory Conjunctitivitis
• Nonpurulent watery discharge, • Observe and monitor patient
conjunctiva! injection and swelling • Clears by the 4th day of life
within hours of instilling the
medication (e.g.,silver nitrate ocular
drops or other prophylactic antibiotics)
2) Congenital Lacrimal Duct Obstruction (Dacryocystitis)
• Blockage of the lacrimal duct system • Most clear spontaneously without treatment
due to delay in the normal • Massage inside corner of the eye over lacrimal
development and opening of the tear duct sac to establish patency
• Persistent tearing and mucoid • If problem persists after 6-7 months, refer to
discharge in the inner corner of the eye ophthalmologist for probing of the duct
• Obstruction is at the nasal end of • If with dacryocystitis: topical or systemic
the duct antibiotics depending on severity of infection
• Often unilateral
191
3) Chlamydia trachomatis (2-40%)
• Associated pneumonia in 10-20%
4) Neisseriagonorrhea (<1%)
• Causes severe, bilateral, hyperacute
conjunctivitis with thick discharge
and chemosis
• Corneal ulcer and perforation within
hours if untreated
• Request for blood and CSF cultures
5) Other Bacterial Etiologies (30-50%)
• Staphylococcus
• S. pneumoniae, Strep viridans
• H. influenzae
• Group A and 8 Streptococci
• Enterobacter, Acinetobacter
• M. catarrhalis
• S. marcescens
• Corynebocterium
• Stenotrophomonas maltophilia
• £. coli, K. pneumoniae, P.aeruginosa
6) Herpes Simplex (<1%)
• Suspect if not responding to
antibiotics
General Management principles
•Treatment mustbe initiatedas soonas possiblebecausecomplications can be severe(perforation/blindness)
• Practicehandhygiene:drainageis contagiousfor24-48hoursafterbeginningtreatment
• Irrigateeye withsterileisotonicsalineto removeaccumulatedpurulentdischarge
• Systemictreatmentis requiredforgonococcal,staphylococcal, chlamydia,pseudomonas& herpeticconjunctivitis
• Evaluateforsignsof systemicdisease becauseinfantswithconjunctivitisare at riskforsecondaryinfectionssuch
as sepsis,meningitisand pneumonia
• Erythromycin base or ethylsuccinate SO mg/kg/
day orally in 4 divided doses for 14 days OR
• Azithromycin20 mg/kg once daily for 3 days
• May recur in 20% of cases
• Risk of infantile hypertrophic pyloric stenosis in
infants <6 weeks treated with erythromycin
• Isolate the infant during the first 24 hours of
parenteral antibiotics
• Evaluate for concomitant infections: Chlamydia
trachamatis, congenital syphilis, HIV
• Treat also for chlamydia due to high rate of
co-infection
• Gonococcal conjunctivitis without
dissemination:
, Ceftriaxone single dose: 25-50 mg/kg IV or IM
, Cefotaxime single dose: 100 mg/kg IV or IM
• Gonococcal conjunctivitis with dissemination:
, Ceftriaxone 25-50 mg/kg IV or IM once daily
for 7 days (10-14 days if with meningitis)
• Healthy infants without conjunctivitis born to
mothers with gonococcal infection
, Ceftriaxone single dose: 25-50 mg/kg IV or IM
• Gram-positive organisms: erythromycin or
bacitracin ointment
• Gram-negative organisms: gentamicin or
tobramycin or ciprofloxacin ointment
• Apply topical antibiotics as 0.5 - 1cm ribbon of
ointment every 6 hours for 7 days (preferred
over eye drops because of reduced washout effect)
• Isolate patient
• Obtain viral cultures: blood, CSF,eyes, stool,
urine, mouth, nasopharynx
• Topical ophthalmic therapy: 3% vidarabine
ointment, 1% trifluridine, or 0.1%
iododeoxyuridine every 2 hours for 14 days
• Acyclovir 60 mg/kg/day IVdivided into 3 doses for
14 days (21 days if disseminated/CNS infection)
• Consult with ophthalmologist
Source:GomellaTL,el al. Neonalology
71hEdition.McGraw-Hill;2013
KliegmanR,el al. NelsonTextbookof Pediatrics
(21sted.).Elsevier;2020
AveryGB,et al.Avery'sneonalology7th Edition.Philadelphia;
2016
192
 SECTION SEVEN
OTHER ISSUES IN THE NEWBORN

RETINOPATHY OF PREMATURITY (ROP)

• Disorder of the developing retinal vasculature among premature infants
• Early vasoconstriction and obliteration of the capillary network in response to high
oxygen concentrations, followed by progressive neovascularization, retinal edema, retinal
hemorrhages, fibrosis and traction, and eventual detachment of the retina
• Risk factors for ROP include: transient hyperoxemia, extreme prematurity, apnea, sepsis,
hyper/hypocapnia, intraventricular hemorrhage, anemia, exchange transfusion, hypoxia,
lactic acidosis
I. STAGESOF ROP
STAGE DESCRIPTION
Stage I • A flat white line that demarcates the vascular and avascular retina
• A ridge of fibrous tissue protrudes into the vitreous in the region between
Stage II

I
vascular and avascular retina
Stage Ill • New blood vessels grow along the ridge & often extend into the vitreous
• Partial retinal detachment:
Stage IV 0 IV-A:excludes the macula
0 IV-8: includes the macula
Stage V • Total retinal detachment

II. DIAGNOSIS
• Examination by binocular indirect ophthalmoscopy confirms the diagnosis
• Infants weighing <1500 g or those <30 weeks AOG should have dilated eye examinations
at 4-6 weeks of age or 31-33 weeks postmenstrual age, and continued examination every
2-3 weeks until retinal maturity
• Infants with ROP or very immature vessels should be examined every 1-2 weeks

III. MANAGEMENT& PROGNOSIS

• Laser photocoagulation: treatment of choice
• Prognosis:
90% of Stage I & II disease regress spontaneously
50% of Stage III+ disease may still regress spontaneously
0 Sequelae of regressed disease: myopia, strabismus, amblyopia, glaucoma and late
detachment require regular follow up
Source:International
Committee
for Classification
of Retinopathy
of Prematurity.
ArchOphthalmol:
2005

MULTIPLE GESTATION
I. ETIOPATHOGENESIS
A. Placental Classification
° Classified according to the placental disk (single, fused or separate), number of
chorions (mono or dichorionic) and number of amnions (mono or diamniotic)
Heterosexual (dizygotic) twins always have a dichorionic placenta
0 Monochorionic twins are always of the same sex and monozygotic

B. Placental Complications:
0 Increased frequency of anomalies of the placenta and adnexa (i.e. single umbilical artery)
0 Umbilical cord is more susceptible to thrombosis when compression or twisting
occurs, resulting in intrapartum fetal distress

C. Perinatal Complications
0 Prematurity and uteroplacental insufficiency are major contributors to perinatal complications
0 Intrauterine growth restriction (incidence of LBW among twins at 50-60%)
° Congenital anomalies
193
II. TWIN-TWIN TRANSFUSION SYNDROME
• Vascular anastomoses seen among monochorionic placentas, wherein the donor fetus
pumps blood into the recipient's circulation)
• Twins have a hemoglobin difference of >5 g/dL and weight discordance >20%
MANIFESTATIONS MANAGEMENT
• Pale/anemic
• Low birth weight • Requires volume expansion and/
Donor twin
• Oligohydramnios or RBCtransfusion
• Hypoglycemia
• Plethoric
• High birth weight
• Polyhydramnios • May require partial exchange
Recipient twin
• Polycythemia transfusion
• Hyperviscosity / hypervolemia
• Hyperbilirubinemia

Ill. APPROACH TO MULTIPLE GESTATION

• Delivery should be conducted at high risk perinatal centers with experienced delivery
teams in attendance
• Infants should be examined for evidence of IUGR,congenital anomalies, twin-twin transfusion
• Central hematocrit should be obtained from both infants
Source:GomellaTL.el al. Neonalology 7thEdition.McGraw-Hill;2013
KliegmanR, el al. NelsonTextbook of Pediatrics(21s1ed.).Elsevier;2020
ClohertyJ, el al. Manualof NeonatalCare(7thed.).Philadelphia;
2012

INTRAUTERINE GROWTH RESTRICTION (IUGR)

I. ETIOPATHOGENESIS
A. Definition ofTerms (to differentiate from SGA)
• Intrauterine growth retardation or restriction
Intrauterine Growth • Reduction in the expected fetal growth of an infant due to
Restriction genetic and environmental factors
(IUGR) • Key feature: appearance of a malnourished infant at birth,
regardless of their size
• Infant whose weight is lower than population norms
• Birthweight <10th percentile for gestational age or >2
Small for Gestational
standard deviations below the mean for gestational age
Age (SGA)
• Definition does not distinguish infants who are
constitutionally small from those who are growth restricted

B. Etio ogy o IUGR

• Genetic determinants (greatest impact in early gestation)
Fetal factors • Others: chromosomal anomalies, congenital malformations,
congenital infections, inborn error of metabolism
• Reduced uteroplacental blood flow, malnutrition, multiple
pregnancies, maternal substance abuse, teratogen exposure,
Maternal factors maternal hypoxemia
• Others: short stature, young maternal age, low socioeconomic
class, grand multiparous, low prepregnancy weight
• Placental insufficiency
• Placenta structural abnormalities: infarcts, aberrant cord
Placental factors
insertions, umbilical vascular thrombosis, premature
placental separation
'Historyof riskfactorsshouldalertobstetrician
of likelihoodof fetalgrowthrestriction.
Ultrasonography
shouldconfirmdiagnosis

194
II. MANIFESTATIONSAND DIAGNOSIS
A. General Features of IUGR
° Fetus responds to compromised nutrient supply by reducing its overall size,
preserving brain growth, accelerating lung maturation, and increasing RBC production
Wasted appearance of the infant (because total body fat, lean mass, and bone mineral
content are reduced)
Risk of mortality and morbidity is increased in IUGR

B. Classification of IUGR
ASYMMETRIC IUGR SYMMETRIC IUGR
• Weight affected more than • Weight, length and head
Presentation
length circumference equally affected
• Poor maternal nutrition or • Genetic and metabolic
Predisposing
exacerbation of maternal conditions that affect fetal cell
condition
vascular disease number
Timing of • Fetus affected late in the • Fetus affected early in
insult gestation gestation, usually <18 weeks

Prognosis

C. Signs of IUGR
• Good catch-up growth (brain
growth is spared)

Reduced birthweight for gestational age

• High mortality and morbidity
• Poorer outcome
I
0 Thin, loose peeling skin due to loss of subcutaneous tissue, scaphoid abdomen

III. MANAGEMENT
ASPECT MANAGEMENT
• Monitoring of fetal wellbeing
• IUGRinfants have worse outcomes when delivered before
Delivery and
28-30 weeks
resuscitation
• Cesarean section delivery when lungs are mature or when
there is fetal distress
• Prevent hypothermia
• Monitor blood glucose levels & treat hypoglycemia promptly
Supportive
• Hematocrit readings to detect polycythemia
management
• Screen for congenital infections
• Screen for genetic anomalies

IV. PROGNOSIS
• Neurodevelopmental morbidities is seen 5-lOx more often in IUGR
• Smaller head circumference is associated with cognitive, psychomotor and behavioral delays
• Prete rm IUGR: higher incidence of abnormalities than the general population having been
subjected to both the risks of prematurity and IUGR

195
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1. Kliegman, R., ST GEME 111,)., Blum, N., Shah, S., Taske1; R., Wilson, K.. & Behrman, R. (2020). Nelson
Textbook of Pediatrics (21st ed.). Philadelphia: Elsevier.
3. Gleason, C.,& Juul, S. (2017). Ave1y's diseases of the newborn (10th ed.). Philadelphia: Elsevier.
4. Apgar, V.(1953). A Proposal for a New Method of Evaluation of the Newborn Infant. Anesthesia & Analgesia, 32(1).
5. The Apgar Score, American Academy of Pediatric, Committee on Fetus and Newborn, American College
of Obstetricians and Gynecologists and Committee on Obstetric Practice. Pediatrics 2006; 117; 1444
6. Ballard ]L, Khou1y JC, Wedig K, et al: New Ballard Score, expanded to include extremely
premature infants.] Pediatrics 1991; 119:417-423.
7. Lowdermilk, D.L. and Perry, S.E.: Maternity & women's health care [9th ed.]. St. Louis, 2007, Elsevier
Mosby; modified from Lubchenco, L., Hansman, C., Boyd, E.: Intrauterine growth in length and head
circumference as estimated from live births at gestational ages from 26 to 42 weeks. Journal of
Pediatrics, 37:403, 1966; Battaglia, F. and Lubchenco, L.: A practical classification of newborn infants by
weight and gestational age. Journal of Pediatrics, 71 [2]:159-163, 1967.
8. Fanaroff, A., Martin, R., & Walsh, M. (2015). Fanaroff and Martin's neonatal-perinatal medicine
(! 0th ed.). Philadelphia: Elsevier/Saunders.
9. World Health Organization. (2014). Early Essential Newborn Care Clinical Practice Pocket
guide. Geneva, Switzerland: WHO.
10. World Health Organization. (2009). Newborn care until the first week of life. Geneva: WHO.
11.American Academy of Pediatrics (AAP): Textbook of Neonatal Resuscitation; 6th Ed. Elk
Grove Village, IL; 2011
12.Perlman, ]., Wyllie,)., Kattwinkel. ]., Wyckoff, M.. Aziz, K., & Guinsburg, R. et al. (2015). Part 7: Neonatal
Resuscitation: 2015 International Consensus on Cardiopulmonary Resuscitation and Emergency
Cardiovascular Care Science With Treatment Recommendations [Reprint). Pediatrics 136[Supplement),
S120-S!66. doi: 10.1542/peds.2015-3373d
13. Wyckoff, M., Aziz, K., Escobedo, M., Kapadia, V., Kattwinkel, ).. & Perlman, ]. et al. (2015).
Part 13: Neonatal Resuscitation: 2015 American Heart Association Guidelines Update for
Cardiopulmonary Resuscitation and Emergency Cardiovascular Care (Reprint).
PEDIATRICS,136(Supplement), Sl 96-S218. doi: 10.1542/peds.2015-3373g
14.Republic of The Philippines, Department of Health. (2009). Universal Hearing Screening
and Intervention act of 2009.
15.Andreas, N., Kampmann, 8., & Mehring Le-Doare, K. (2015). Human breast milk: A review
on its composition and bioactivity. Early Human Development, 91 (11), 629-635. doi:
l 0.1016/j.earlhumdev.2015.08.013
16. Wu, X., Jackson, R., Khan, S., Ahuja, )., & Pehrsson, P. (2018). Human Milk Nutrient
Composition in the United States: Current Knowledge, Challenges, and Research Needs.
Current Developments In Nutrition, 2(7). doi: 10.1093/cdn/nzy025
17.Academy of Breastfeeding Medicine. (2017) Clinical Protocol Number #8: Human Milk
Storage Information for Home Use for Healthy Full Term Infants. Breastfeeding Medicine,
12(7), 390-395.
18.Centers for Disease Control and Prevention (CDC). (2015). Breastfeeding: Frequently
Asked Questions. Retrieved from https://www.cdc.gov/breastfeeding/faq/index.htm
19.Centers for Disease Control and Prevention (CDC). (2017). Proper Handling and Storage of
Human Milk. Retrieved from https://www.cdc.gov/breastfeeding/recommendations/
handling_breastmilk.htm
20.Gomella TL, et al. Neonatology 7th Edition. McGraw-Hill; 2013
21.Cloherty, ]., Eichenwald, E., Hansen, A., & Stark, A. (2012). Manual of Neonatal Care (7th
ed.). Philadelphia: Lippincott Williams & Wilkins.
22.Sharma, V., Berkelhamer, S., & Lakshminrusimha, S. (2015). Persistent pulmonary
hypertension of the newborn. Maternal Health, Neonatology And Perinatology, 1(1).
doi: 10.1186/s40748-015-0015-4 Modified from 2015 Red Book Report of the Committee
on Infectious Diseases, 30th ed
23.Ave,y GB, et al. Avery's neonatology 7th Edition. Philadelphia; 2016
24. Engorn, 8., & Flerlage, ]. (2015). The Harriet Lane Handbook (20th ed.). Philadelphia: Elsevier
25.Bhutani VK, Johnson L, Sivieri EM: Predictive ability of a predischarge hour-specific
serum bilirubin for subsequent significant hyperbilirubinemia in healthy term and near-
term newborns, Pediatrics 103:6-14,
26.0ski FA: Differential diagnosis of jaundice. In Taeusch HW, Ballard RA. Avery MA. editors:
Schaffer and Avery's diseases of the newborn, ed 6, Philadelphia, 1991. Saunders
27.American Academy of Pediatrics Subcommittee on Hyperbilirubinemia: Management of
hyperbilirubinemia in the newborn infant 35 or more weeks of gestation, Pediatrics
114:297-316, 2004
28.International Committee for Classification of Retinopathy of Prematurity. The International
Classification of Retinopathy of Prematurity revisited. Arch Ophthalmol 2005; 123: 991

196
 DISEASES
INFECTIOUS
 SECTION ONE
APPROACH TO FEVER

OVERVIEW OF FEVER
I. DEFINITION OF FEVER
• Fever is a normal response to a variety of conditions, usually from an infection
• Controlled increase in body temperature of c,l °C above normal values at the following sites:

SITE TEMPERATURE
Rectal "38.0 °C
Oral ;,, 37.6 °C
Axilla ;,, 37.4 °C
Tympanic "37.6 °C
Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia;
2020

II, MECHANISMOF FEVER

• Due to elevation of the thermoregulatory set point of the hypothalamic center during
condition of increased heat production or when the mechanisms of heat loss are defective
• Leads to an increased risk of febrile seizures especially among ages 6 months to 6 years
I
• _·

III. FEVER PATTERNS

PATTERN DESCRIPTION EXAMPLES

Sustained/ • Persistently elevated temperature but does • Enteric fever
Continuous
fever not vary by >0.5°C over 24 hours • Lobar pneumonia

• Elevated temperature observed over 24

Remittent
hours, varies by >0.5°C, and does not return • Endocarditis
fever
to normal levels
• Malaria
Intermittent • Elevated temperature observed over 24 hours
• Acute pyelonephritis
fever varies with intervals of normal temperature
• Local boils, furuncles

Undulant • Gradual increase in temperature that remains high for a few days then
fever gradually decreases to normal temperature levels
Septic/ • Large differences between peak and trough temperatures
Hectic fever
Source:KliegmanR.et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia;
2020

199
GENERAL CLASSIFICATION OF FEVER
I. FEVER WITH LOCALIZING SIGNS
An acute illness with the focus of infection readily identified
• Management is directed toward the underlying cause

II. FEVER WITHOUT A FOCUS

• Refers to a rectal temperature of ;,38°C as the sole presenting feature
• Subcategories include the following:
° Fever Without Localizing Signs (FWLS)
° Fever of Unknown Origin (FUO)

A. Fever Without Localizing Signs (FWLS)

Fever of recent onset (<l week) with no adequate explanation determined by history
or examination, without any apparent site of infection

AGE AND COMMONCAUSES MANAGEMENT

TEMPERATURE
• Sepsis & meningitis from group-B • Hospitalize
Infant <1 month Streptococcus, Escherichia coli, • Empiric parenteral
Listeria monocytogenes antibiotics
T ;,38°C • Workup for infectious
• Neonatal herpes simplex virus
infection, enteroviruses, parechovirus etiology
• No need to hospitalize
Infant 1-3 mos & no antibiotics
T;,38°C • Serious bacterial disease in 5-15% • Daily follow-up until
Low risk* • UT! (most common serious bacterial blood, urine, and CSF
infection) cultures are final
• E. coli most common pathogen
Infant 1-3 mos
• Enterovirus, parechovirus, influenza • Hospitalize
T ;,38°C
• Parenteral antibiotics
High risk**

Child 3-36 mos • No need to hospitalize

T 38-39°C • Majority are viral in etiology & no antibiotics
• Bacterial etiology for occult
bacteremia: S. pneumoniae, H. • Hospitalize
Child 3-36 mos influenzae type b, N. meningitidis, • Empiric antibiotics
Salmonella, S. aureus. S. pyogenes • Workup for infectious
T>39°C
etiology
'A lowriskinfanthas an uncomplicatedmedicalhistory,normalPE, laboratorystudies:
• Urine:negativeleukocyteesterase,negativenitriteand <10WBC/HPF
• Peripheralblood:5,000-15,000WBC/mm3; <1,500bands;band:totalneutrophil
ratio<0.2
• Stoolstudiesifwithdiarrhea:no RSCand <5WBC/HPF
• CSFcellcount<8WBC/µLand negativeGramstain
• Chestradiographwithoutinfiltrates
"A highriskifinfantdoes notfulfillall lowriskcriteria

Source:KliegmanR,et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia:

2020
MandellGL.el al. Mandell.DouglasandBennett'sPrinciplesandPracticeof InfectiousDiseases:Philadelphia,
2010

200
B. Fever of Unknown Origin (FUO)
0 Documented fever for which the cause could not be identified after 3 weeks of
evaluation as outpatient or after 1 week of evaluation in the hospital

HEALTHCARE- IMMUNE-
HIV-RELATED
FEATURE CLASSIC FUO ASSOCIATED DEFICIENT
FUO
FUO FUO

Temperature ~38.0°C (or 100.4°F)

• >3 weeks as
Duration of outpatient
evaluation & • >l week • >l week
fever • >3 weeks as • >l week as
outpatient inpatient
• >2 visits
• >l week as
inpatient
• Not present • Negative
Other • HIV infection
or incubating cultures after
Aspects confirmed
on admission 48hrs

• HIV primary

I
infection
• Typical and
atypical
• Healthcare- • Majority are Mycabacteria
• Cancer
associated caused by • CMV
• Infections
infections infections, • Lymphoma
Causes • Inflammation
• Postoperative documented • Toxoplasmosis
• Hyperthermia
complications only in • Cyptococcosis
• Undiagnosed
• Drug fever 40-60% • Immune
reconstitution
inflammatory
syndrome
(IRIS)

Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia;

2020
MandellGL.et al. Mandell.DouglasandBennett'sPrinciplesandPracticeof InfectiousDiseases;Philadelphia,
2010

201
 SECTION TWO
INFECTIONS IN THE NEONATE

NEONATAL SEPSIS
• Clinical syndrome of systemic illness accompanied by bacteremia occurring in the first
month of life

I. ETIOPATHOGENESIS
EARLY-ONSET SEPSIS LATE-ONSET SEPSIS
Onset • Birth to 7th day of life • 8th to 30th day of life
• Transplacental • Vertical transmission: acquired
• From the maternal genital after birth from the maternal
tract during the antepartum or genital tract
How intrapartum period • Horizontal transmission or
organism is
acquired • Ascent of pathogens after rupture from contact with contaminated
of amniotic membrane leading to equipment, environmental
chmioamnionitis& fetalcolonization/ sources, or healthcare workers
aspiration of amniotic fluid • Hematogenous route
Most common • GBS • GBS
bacterial
agents • E. coli • S. aureus

• Prematurity (single most significant risk factor)

• Prolonged rupture of membranes (>18 hours)
• Maternal peripartum infection
• Fetal and intrapartum distress (meconium staining, traumatic delivery)
Risk Factors
• Invasive procedures
• Metabolic factors (hypoxia, acidosis, inherited metabolic disorders)
• Previous delivery of a neonate with GBSdisease
• Multiple gestation
Source:EdwardsMS,et al. Principlesand Practiceof PediatricInfectiousDisease.Philadelphia;
2012

II. MANIFESTATIONS
REMARKS
Temperature
instability • Hypothermia/hyperthermia

Neurologic • Lethargy, irritability, seizures

• Poor peripheral perfusion, cyanosis, mottling, pallor, petechiae,
Skin
rashes, sclerema, jaundice
Feeding problems • Vomiting ,diarrhea, abdominal distention
• Tachypnea, respiratory distress, apnea
Cardiopulmonary
• Tachycardia, hypotension
Metabolic • Hypoglycemia, hyperglycemia, metabolic acidosis
• Cellulitis, impetigo, soft tissue abscesses, omphalitis, conjunctivitis,
Focal infections
meningitis, osteomyelitis

202
III. DIAGNOSIS
DIAGNOSTICS REMARKS
• Neutropenia (better specificity than leukocytosis)
CBC
• Decreased platelet count: late sign of sepsis and very nonspecific
Acute phase
• Increased CRP
reactants
Chest
• Done if with respiratory manifestation
radiograph
Blood culture • Required for all neonates with suspected sepsis
Urine culture • Not part of routine workup
Urinary tract • Ultrasound, renal scan, voiding cystourethrogram
imaging • If sepsis is accompanied by UT!
• Indications include:
Lumbar tap for 0 Positive blood culture
CSFstudies 0 Suspected meningitis
0 Non-responsive to antibiotics

IV. MANAGEMENT
ASPECT

Prevention
MANAGEMENT
• GBS prophylaxis with penicillin/ampicillin/cefazolin during labor or
rupture of membranes among those screened at 35-37 weeks AOGto be:
I
0Positive for vaginal and rectal GBScolonization
0With GBS isolated in the urine (>10,000 cfu/ml)
Empiric • Early-onset: Ampicillin + Aminoglycoside or 3"' generation Cephalosporin
treatment • Late-onset: Vancomycin + Aminoglycoside or 3"' generation Cephalosporin

Algorithm for Secondary Prevention of Early-Onset GBSDisease among Newborns

Full diagnostic evaluation
Signs of neonatal sepsis?
Antibiotic therapy
No

Limited evaluation
Maternal Chorioamnionitis?
Antibiotic therapy
No

GBS Prophylaxis indicated for mother? Routine clinical care

No

Mother received o!::4

hours of penicillin,
ampicillin, or cefazolin JV?
No Observation for a:48 hours
a:37 weeks AND duration of membrane
rupture <18 hours?
No

Either <37 weeks OR duration of Limited evaluation

membrane rupture ?!18 hours? Observation for ?:48 hours

Source:KliegmanR,et al. NelsonTextbookof Pediatrics

(21sted.).Philadelphia;
2020
GomellaTL,et al. Neonatology7thEdition.McGraw-Hill;
2013.
Edwards,
M.ClinicalFeatures,
Evalualion,
Diagnosis of SepsisinTenmandLalePretenm Infants.UpToDate2019

203
NECROTIZING ENTEROCOLITIS
• lschemic and inflammatory necrosis of the bowel after initiation of enteral feeding
• Most common life threatening emergency of the GIT in neonates

I. ETIOPATHOGENESIS
A. Pathophysiology
0 Multi factorial cause: prematurity, formula milk, ischemia and bacterial colonization
0 Triad of NEC: Intestinal ischemia (injury), enteral nutrition (metabolic substrate),
pathogenic organisms (bacterial translocation)
Increase in incidence & case fatality rates in decreasing birth weight and gestational age
0 Most commonly involved area: distal ileum & proximal colon
° Characteristic histologic finding: coagulation necrosis
8. Risk Factors
0 Prematurity (most important risk factor due to immature mucosa! barrier)
• Microbial colonization
0 Enteral feeding
• Circulatory instability (blood is diverted away from splanchnic circulation)
0 Others: maternal cigarette smoking, congenital heart disease (hypoxemia from cardiac
failure), and polycythemia & hyperviscosity syndromes

II. MANIFESTATIONS
• May be insidious or acute
• Usually occurs in the z,•
or 3•• week of life to as late as 3 months old for the very low
birth weight (VLBW) infants
Age of onset is inversely proportional to gestational age

GASTROINTESTINAL SIGNS SYSTEMIC SIGNS

• Abdominal distention/ tenderness* • Lethargy
• Feeding intolerance* • Apnea / respiratory distress
• Delayed gastric emptying • Temperature instability
• Vomiting • Metabolic/respiratory acidosis
• Occult or gross bloody stool* • Glucose instability
• Change in stool patterns • Poor perfusion / shock
• Abdominal mass • DIC
• Erythema in abdominal wall • Positive results of blood cultures
'Clinicaldiagnosismaybe madeifthe triadof feedingintolerance,abdominaldistensionand grosslybloodstoolare present
"Complicationsincludebowelperforation, responsesyndrome(SIRS),shock,death
peritonitis,systemicinflammatory

III. DIAGNOSIS
DIAGNOSTIC REMARKS
• CBC with differential count and platelets
• CRP
Laboratory
• Blood & stool culture
Tests
• Electrolytes, ABG
• Coagulation studies

Imaging • Plain abdominal radiograph

204
Modified Bell Staging Criteria for NECin Neonates (for evaluation of disease severity)
STAGE& RADIOGRAPHIC
SYSTEMIC SIGNS ABDOMINAL SIGNS
CLASSIFICATION SIGNS

• Temperature • Gastric retention • Normal or

instability • Abdominal distention mild intestinal
IA Suspected
• Apnea, lethargy • Emesis dilation
• Bradycardia • Heme-positive stool • Mild ileus
1B Suspected • Same as above • Grossly bloody stool • Same as above
• Same as above, plus • Intestinal
• Absent bowel dilation
Definite, mildly
IIA • Same as above sounds, with or • lieus
ill
without abdominal • Pneumatosis
tenderness intestinalis
• Same as above, plus
• Same as above,
• Absent bowel
plus
Definite, sounds, definite • Same as II A, plus
II B moderately ill
• Mild metabolic
tenderness, with or • Ascites
acidosis and
without abdominal
thrombocytopenia
cellulitisor RLQmass
• Same as II B, plus

IIIA
Advanced,
severely ill,
• Hypotension,
bradycardia,
severe apnea,
combined
• Same as above, plus
• Signs of peritonitis,
marked tenderness,
and abdominal
• Same as above,
plus
• Ascites
I
intact bowel respiratory
distention
and metabolic
acidosis, DIC,
neutropenia

Advanced, • Same as above,

severely ill, plus
IIIB • Same as III A • Same as IIIA
perforated • Pneumo-
bowel peritoneum
Source:NeuJ. NecrotI21ng
enterocohtIs.
Ped1atr
ChnNorthAm;1996
IV. MANAGEMENT
ASPECT MANAGEMENT

• Exclusive breastfeeding
Prevention • Minimal enteral feeds followed by judicious volume advancement
• Probiotics
• Systemic antibiotics (with gram positive, gram negative, and
Antibiotics anaerobic coverage) after blood is drawn for cultures
• Usually given for 10-14 days
• Cessation of feeding
• Bowel decompression
Supportive Care
• IV fluids and monitor fluid intake/output
• Monitor hemodynamics, coagulation profile, electrolytes, acid-base status
• Evidence of perforation on abdominal radiograph
• Abdominal paracentesis shows stool or organism on Gram stain
Indications for • Failure of medical management
surgery • Single fixed bowel loop on X-ray
• Abdominal wall erythema
• Palpable mass
Source:Khegman
R, et al. NelsonTextbookof Ped,atncs
(21sted.).Ph1ladelph1a;
2020
GomellaTL,et al. Neonatology7thEdition.McGraw-Hill;
2013

205
 TRANSPLACENTAL INFECTIONS
PATHOGENESIS
1) Toxoplasmosis (Toxoplasma gondii)
• Transmission through cat feces or
ingestion of undercooked meat
• Transmission greatest when acquired
during the 3rd trimester
2) Rubella
N
0
• Spread by respiratory secretions,
stool, urine and cervical secretions
"' • Earlier infection (1-12 weeks AOG)
carries higher incidence of congenital
defects and greater effects on the fetus
3) Cytomegalovirus
• Most common congenital infection
• Transmitted through secretions
• Penetrates placental and blood brain
barrier
• Teratogenic potential in the fetus
during the first trimester
MANIFESTATION
• Most are asymptomatic at birth
• Triad: chorioretinitis, intracranial
(cerebral) calcilkacions, hydrocephalus
• Late manifestation: visual impairment,
learning disabilities, mental
impairment
• IUGR
• Mental retardation,
• "Blueberry muffin" rash
• Cataracts
• Microphthalmos
• Retinopathy
• Congenital heart disease (e.g., PDA)
• Sensorineural deafness
• Speech and language delay
• Pregnant women develop
mononucleosis-like illness
• Triad of congenital CMV:
chorioretinitis, microcephaly,
periventricular calcifications
• Other findings:hepatosplenomegaly,
petechial rashes, jaundice
DIAGNOSIS MANAGEMENT
• Direct isolation of organism from body • Treatable but not curable
nuids or tissue • Medications cannot kill the encysted
• Serologic testing with toxoplasma bradyzoites:
specific lgG and lgM 0 Pyrimethamine & sulfonamide for 6
• Perinatal diagnosis by PCR of amniotic months
Ouid • Folinic acid for 12 months
• CSF examination: PCR and lgM 0 Prednisone for active chorioretinitis
• Cultures may be done up to 1 year
of illness (nasopharyngeal swabs,
conjunctiva! scrapings, urine, CSF)
• No specific treatment
• Serologic studies: rubella specific lgM
• Prevent with maternal rubella vaccination
in serum or oral fluid taken before 3
prior to pregnancy
months of age
• Rubella virus PCR
• CSF examination: encephalitis with
increased protein and cell count
• Prenatal ultrasonography
• Ganciclovir for infants with symptomatic
• Gold standard: urine or saliva Clllture
disease
obtained before 3 weeks of age
• Symptomatic infants have a mortality rate
• PCR for CMV DNA of blood
of 20-30% (sepsis like illness)
• Serology: lgM detection
• Leading cause of deafness
• CBC, liver function tests, CSF analysis
• Late complications: intellectual or
and culture
developmental impairment, IUGR,
• Cranial UTZ or CT scan demonstrate
microcephaly
periventricular calcification
 PATHOGENESIS MANIFESTATION
4) Herpes Simplex
• Three patterns of disease:
0 Disease localized to the skin, eyes,
• Most are due to HSV-2
or mouth
• Acquired intrauterine,
0 Encephalitis with or without skin,
intrapartum or postnatal
eyes, and mouth disease
• 85% are ascending infection
0 Disseminated infection involving
multiple organs
N
0
5) Varicella (Fetal Varicella Syndrome)
___,
• Cicatricial scars and skin loss
• CNS:microcephaly, seizures, encephalitis,
cortical & spinal cord atrophy, mental
• Develops when mother is exposed
retardation, cerebral calcifications
during first half of pregnancy
• Ocular: microphthalmia,
(8th-20th week)
chorioretinitis, cataracts, optic atrophy,
• Virus spreads to all fetal organs
nystagmus, Horner syndrome (ptosis,
hematogenously
miosis, enophthalmos)
• Limb hypoplasia
• Prematurity and IUGR
DIAGNOSIS MANAGEMENT
• Viral cultures of skin or
• For mothers with genital lesions, do surface
mucocutancous membrane lesions/
cultures of infant at 12-24 hours of age and
surface
treat if symptoms develop or if cultures are
• PCR of CSF and blood
positive
• Immunologic assays: HSV antigen
• Acyclovir IV 60 mg/kg/day every 8 hours
• Imaging: CT or MRI of brain
0 14 days for skin, eyes and mouth disease
reveals parenchyrnal brain edema,
0 21 days for disseminated or CNS disease
hemorrhage and destructive lesions in
• Acyclovir PO 300 mg/m' /dose thrice a day for 6
the temporal lobe
months after treatment as suppressive therapy
• EEG for infants with CNS involvement
• Mother: VZIG or !VIG given within 72-96 hours
of exposure
• Maternal varicella
• Acyclovir if chicken pox is diagnosed during
• Presence of congenital skin lesions in
pregnancy
dermatomal distribution
• Infant:
• Proof of intrauterine VZVinfection by:
0 Supportive care because of profound
0 Detection of viral DNA in infant by
neurologic impairment
PCR (fetal blood and amniotic fluid)
0 Acyclovir to stop progression of eye disease
0 VZVspecific IgM
or to treat shingles, which is common during
0 VZV specific IgG beyond 7 months
the first 2 years of life
0 Ultrasonography for typical VZV
• 30% die in the first 4 months
anomalies
• High risk of developing zoster in the first 2
years
-
 PATHOGENESIS
6) Congenital Varicella Infection
• Occurs when mother suffers from
infection during the last 3 weeks of
pregnancy or within first few days
postpartum (when rash appears 5 days
before delivery & 2 days post partum)
7) Chlamydia trachomatis
• Most common cause of sexually
transmitted genital infections
• Causes conjunctivitis and pneumonia
N in infants
0 with the development of infantile
00 • Neonate acquires infection through
vaginal delivery (infected cervix)
8) Neisseria gonorrhea
• Transmitted via contact with cervical
canal during delivery or with
contaminated amniotic fluid
• Co-infection with Chlamydia is
common
MANIFESTATION
• Centripetal rash that spares the
extremities
• Macules that progress to vesicles and
encrustation
• Staphylococcal and streptococcal
superinfectionare the main complicatons
• May develop varicella pneumonia
• Conjunctivitis: discharge, redness,
swelling, corneal opacification, chemosis,
pseudomernbrane formation
• Trachoma: chronicfollirularkeratoconjunctivitis
whichcauses scaningandneovdSrulaiii.ationof
the cornea resulting in blindness
• Pneumonia:presentsat 3-11 weeks of
life, majority are afebrile
• Paroxysmal, staccato cough that
interferes with sleepingand eating
• Ophthalrnianeonatorum
• Gonococcalarthritis (common in knees
& ankles)
• Amniotic nuid syndrome when there
is premature rupture of membranes,
inflammation of the placenta and
umbilical cord
• Sepsisand meningitis
• Scalpabscess
DIAGNOSIS
• VZV DNA PCR of vesicular fluid or scabs,
biopsies, amniotic nuid
• Viral culture and direct fluorescent
antibody assay
• Serum testing ofVZV antibody: lgM
detected as soon as 3 days after
appearance of symptoms
• Gold standard: culture of the organism
from tarsal conjunctivae or from the
nasopharyngealaspiration
• NucleicAcid Amplification tests
• Antigen detection tests:direct fluorescent
antibody and enzyme immunoassay
• Serum anti-chlamydia! antibody (lgM)
concentration
• Pneumonia:WBC is normal but with
eosinophilia in 70%
• Gram stain of any exudate
• Culture of eye discharge, nasopharynx,
orogastricor anorectalarea
• Blood r.:ulture
• Lumbar puncture with spinal fluid
studies
• Culture for concomitantChlamydia
trachomatis
Source:KliegmanR.et al. NelsonTextbookof Ped,atncs(21sted.). Phrladelphra;
MANAGEMENT
• IVIG400mg/kg as soon as possible, not later
than 10 days
• Acyclovir: lSmg/kg/dose every 8 hours for
7 days for post exposure prophylaxis and as
treatment of symptomatic neonates
• Erythromycin SO mg/kg/day divided in 4
doses for 14 days OR Azithromycin 20 mg/kg
single daily dose for 3 doses
• Note: reported associationof erythromycin
hypertrophic pyloric stenosis (IHPS) in infants
< 6weeks
• Non-disseminatedinfections (including
ophthalmia neonatorum):
Ceftriaxoneor Ceoftaxime single dose
• Arthritis and septicemia:
Ceftriaxoneor Cefotc1xi111e for 7 days
• Meningitis:
Ceftriaxone or Cefotaxime for 10-14 days
• All infants with gonococcalinfection:contact
isolation until effective parenteral antibiotic
therapy given for 24 hours
2020
GomellaTL,et al. Neonatology7th Edition.McGraw-Hill;2013
NeuN,et al. Clinicsin Perinatology.
Elsevier;2015
 SECTION THREE
VIRAL INFECTIONS

INFLUENZA
I. ETIOPATHOGENESIS
A. Influenza Virus (Types A, B, and C)
RNA virus, orthomyxovirus
, Antigenic drift: minor antigenic variations may occur within types A & B that result in
new strains leading to seasonal epidemics
0 Antigenic shift: major antigenic changes in influenza A viruses resulting in new HA or
NA which can result in pandemics

• Causes epidemics
• Has an animal host
Influenza Type A
• Subtypes (based on surface antigens Hemagglutinin and
Neuraminidase): H & N
• Causes epidemics
Influenza Type B
• Has no animal host
Influenza Type C • Causes sporadic mild influenza-like illness

B. Transmission

• Respiratory droplets
• Contact with respiratory tract droplet-contaminated surfaces
I
Mode of
followed by autoinoculation
Transmission
• Viral shedding in nasal secretions peak on first 3 days of illness
and cease within 7 days
Incubation Period • 1-4 days

II. MANIFESTATIONS
• Abrupt onset of high grade fever, coryza, conjunctivitis, pharyngitis, dry cough, myalgia,
malaise & headache
• Majority recover fully after 3-7 days
• Complications include:
Otitis media
0 Pneumonia
° Febrile seizures to encephalopathy /encephalitis
Reye syndrome with intake of aspirin
0 Myocarditis
Invasive secondary bacterial infections

Ill. DIAGNOSIS
• May be confirmed serologically by hemagglutination inhibition test (during acute and
convalescent stages), viral culture, reverse transcriptase-PCR

IV. MANAGEMENT

Non- • Supportive therapy

Pharmacologic • Do not give aspirin
• Oseltamivir taken within 48 hours may decrease the severity & duration
Pharmacologic • Amantadine & Rimantadine as prophylaxis & treatment of type A
outbreaks
Source:KliegmanR.et al. NelsonTextbook
of Pediatrics
(21sled.).Philadelphia;
2020
Redbook.Reportof theCommiltee on InfectiousDiseases.31sted; 2018

209
HERPES SIMPLEX VIRUS (HSV)
I. ETIOPATHOGENESIS
A. Etiologic Agent
HSVTYPE 1 HSVTYPE2
• Affects the face & skin above • Affects the genitalia & skin
Infection
the waist below the waist
Incubation Period • 2-12 days • 2-12 days
• Direct contact with virus
Mode of • Direct contact with infected
shed from genital lesions or
Transmission secretions or orolabial lesions
secretions duting sexual activity

Primary Infection • HSVseronegative (subclinical)

First infection, • Infection caused by a 2nd type of HSV in a person with
Non primary immunity to 1 HSVtype (less severe)
• Reactivation of a latent infection in an immune host
Recurrent Infection
• Follows stimuli like cold, stress, fever

II. MANIFESTATIONS
MANIFESTATION DESCRIPTION
• Most commonly affects 6 months to 5 years old
• Drooling, high-grade fever, refusal to eat or drink, and painful
Herpetic
ulcers on the tongue, gums, lips
gingivostomatitis
• Hallmark: skin vesicles and shallow ulcers that are more
extensively distributed than herpangina

Herpes labialis or • Most common manifestation of recurrent HSV-1infection

fever blisters or cold • Most common site is at the vermilion border of the lip
sores • Burning pain, or itching sensation before appearance of lesions
• Common in sexually experienced adolescents and young adults
• Classic primary genital herpes: local burning and tenderness
Genital herpes before vesicles appear
• Vesicles become shallow ulcers with yellowish gray exudates,
then become crusts
• Due to skin trauma and exposure to infectious secretions,
commonly in contact sports (wrestling)
Herpes gladiatorum
• Multiple vesicles affect a larger surface area and systemic
symptoms are uncommon
Herpes whitlow • HSVinfection of the finger or toes, strictly involvingthe paronychia
Conjunctivitis/ • Usually unilateral, with blepharitis & preauricular lymphadenopathy
keratoconjunctivitis • Vesicles on lid margins and periorbital skin with fever
Perinatal / neonatal
• See transplacental infections
infection
• Acute necrotizing infection affecting the frontal lobe and/or
temporal lobe and limbic system
• Manifestations: fever; headache, neck rigidity, nausea, vomiting,
HSV encephalitis
seizure, change in sensorium, anosmia, memory loss, expressive
aphasia, hallucinations, focal seizures
• Untreated infections progress to coma & death in 7S% of cases
210
III. DIAGNOSTICS
• Viral culture: gold standard in diagnosing HSYinfections
• PCR to detect HSYDNA:test of choice in examination of the CSF for herpes encephalitis
• EEGand cranial MRI beyond the neonatal period to check for temporal lobe abnormalities

IV. MANAGEMENT
ANTI-VIRAL AGENT INDICATIONS
• Severe mucocutaneous and disseminated infections in
immunocompromised patients
IV acyclovir
• CNS infection
• Perinatal infection
• Gingivostomatitis
Oral acyclovir or
• Herpes labialis
valacyclovir
• Genital herpes
Source:KliegmanR.et al. NelsonTextbook
of Pediatrics
(21sted.).Philadelphia;
2020
Redbook.Reportof theCommittee on InfectiousDiseases,31sted;2018

MUMPS
I. ETIOPATHOGENESIS
• Due to RNAvirus of the family Paramyxoviridae and the genus
Etiology

Mode of Transmission
Period of
Communicability
Rubulavirus
• Respiratory droplets from person to person

• 1-2 days before to 5 days after onset ofparotid swelling

I
Incubation Period • Average of16-18 days (12-25 days)

II, MANIFESTATIONS
• Presents with 1-2 days prodrome of fever, headache, vomiting then parotitis appears
• Parotid swelling and tenderness, peaks in 3 days then gradually subsides over 7 days,
occurs bilaterally in 70% of cases
• Angleof the jaw is obscured as swelling ensues and the earlobe may be lifted upward and outward
• Edema over the sternum may occur due to lymphatic obstruction

III. DIAGNOSIS
• Enzyme immunoassay for mumps lgM
• Serum lgG from acute and convalescent serum specimens
• Virus isolated from upper respiratory tract secretions, urine, or CSF during the acute
illness through cell culture, immunofluorescence, reverse transcriptase PCR

IV. MANAGEMENT
• Supportive: antipyretics, pain relief, adequate hydration

V. COMPLICATIONS

Meningitis with or • Most commonly manifests 5 days after the parotitis

without encephalitis • Symptoms resolve in 7-10 days
• Begins within days afteianset of parotitis
Orchitis • High fever, chills, exquisite testicular pain and swelling
(30-40% of post-
• May be bilateral in 30% of cases
pubertal males)
• Sterility is rare even with bilateral testicular involvement
• Pancreatitis, myocarditis, pneumonia, optic neuritis,
Less common
conjunctivitis, nephritis, thrombocytopenia
Source:Khegman
R,et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia,
2020
Redbook.Reportof theCommittee on InfectiousDiseases,31sted;2018
211
 VIRAL EXANTHEMS
ETIOLOGY & TRANSMISSION
INCUBATION MANIFESTATION &PERIOD OF DIAGNOSIS MANAGEMENT COMPLICATIONS
PERIOD (IP) COMMUNICABILITY
1) Measles (Rubeola)

• High-grade revcr with cough, conjunctivitis and colds • Most common: otitis media
• Airborne • Most common cause of
(3-5 days) and photophobia
• Viable virus may he mortJlity: pneumonia
• Enanthcm: Kuplik spots (grayish white dots with red
found up to 1 hour in • Warthin- • Subacu1·c sdcrosing
border opposite lower molars) appear before the rash • Supportive
air and up to 2 hours Finkeldey panencephalitis (ratal
• Paramyxovirus • Rashes usually appear at the height of fever • Vitamin A oral OD
on contnct surfaces giant cells degenerative disease from
• 1P8-12days • Cranioan1d.ildissemination:1naculopapularrashbeginsin face lor2 days
• Communicability: 4 (rusion or persistent infection 7-l Oyears
• Rash fades downward in the same sequence in which it
days before to 4 days infected cells) after natural measles infection)
dcsqunmation), disappears i117-10 days
appeared (b1c11111y • Final common pathway to a
after onset or rash
• If uncomplicated, symptoms subside rapidly & fover fatal outcome: bronchiolitis
abates as rashes appe;,1rs on the legs & feet obliterans

N
2) German Measles (Rubella or 3-day Measles)
......
N
• Most characteristic sign: retroauricular, posterior
• Thrombocytopenia 2 weeks
cervical & postoccipital symmetrical lymphadenopathy • Droplet or
• fourfold after rash
(begins 24 hours before the rash & remains for 1 week) transplacenta\ • Arthritis of the snrnll joints
• Enanthem: Forschcimcr spots fdiscrete rose spots on increase in
• Togavirus • Communicability: 7 of the hands (adult women)
• IP 14-21 days . the soft palate) appear at the same time as rash
No photophobia
• Feverwitl1decrease in temperature on 3"14 th day as rashes appear
clays before to 6-7 clays
after onset of rash
lgM and lgG
antibody is
• Supportive
within 1 week of rash
• Encephalitis (postinfoctious
diagnostic syndrome or a rare progressive
• Maculopapular rash begins at face and neck and
panencephalitis)
spreads centrifugally as discrete maculcs

3) Roseola lnfantum
. Most often among <3 years old, peak at 6-15 mos old
• Fever for 3-5 days with fussiness • Probably acquired from
• HHV-6
• Excellent with no obvious
serology, l'CR,
• Human Herpes • Rash appears within 12-24 hours of fever resolution: the saliva or healthy sequelae
virus culture
virus (l·IHV) discrete, small pink lesions on the trunk, spreads in a persons, enters host • Hl·IV-6can suppress all cellular
• LowWllC • Supportive
6&7 centripetal pattern, fades in 1-3 days through mucosa lineages within the bone
count,
• 11'9-l0days • Ulcers in uvulopalatoglossal junction (Nagayama spots) • Communicability: marrow
neutrophils,
• Bulging of anterior fontanelle unknown
• Convulsions lymphocytes
 ETIOLOGY & TRANSMISSION
INCUBATION MANIFESTATION & PERIOD OF DIAGNOSIS MANAGEMENT COMPLICATIONS
PERIOD (IP) COMMUNICABILITY
4) Chickenpox (Varicella)
• Varicella zoster • IV Acyclovir: for
• Fever then pruritic exanthem • Direct contact or
antibody titers severe disease and • Secondary bacterial
• All stages of rash are present simultaneously airborne spread
• Leukopenia immunocompromised infection
• Varicclla·Zoster • Rash often appear first on the scalp, face, or trunk, then • Communicability: 1-2
during the 1st • Oral acyclovir best • Encephalitis or meningitis
Virus (VZV) spread to other parts of the body days before the onset
3 days after started on JS1 day • Pneumonia
• IP 10-21 days • Intensely pruritic red macules, become pa pules, then of the rash until all the
onset of rash, of illness to reduce • Reye syndrome
vesicles (rapid progression) lesions have crusted
followed by number of lesions & • Glomerular nephritis
• Ulcers also common in oropharynx and vagina
lymphocytosis shorten course

5) Erythema Infectiosum (Fifth Disease)

• Clinically apparent infection prevalent in 5-15 years old
• Mild fever & systemic symptoms precedes facial rash • Large droplet spread
• Hallmark sign: rash with e1ythematous facial flushing from nasopharyngeal
N • Severe anemia
>--' ("slapped-cheek") & spreads rapidly to trunk & proximal shedding & by blood/
w • Serum lgM • Infection in pregnant
• Parvovirus 819 extremities as a diffuse macular erythema blood-borne products
antibodies patients may cause
and bocavirus • Atypical skin eruption: papular-purpuric "gloves & • Communicability: before • Supportive
• PCR miscarriage, intrauterine
• IP4-14days socks" syndrome (PPGGS), then fever; pnrritus, painful the onset of the rash
death, hydrops fetal is
edema & erythema localized to distal extremities, until after the onset of
followed by acral petechiae & oral lesions the rash
• Resolves within a few weeks, without desquamation,
but can wax & wane over 1-3 weeks

6) Hand-Foot-Mouth Disease
• Commonly affects <l Oyears of age • Fecal-oral and
• Viral culture • More severe disease
• 1-2 days fever then enanthem (vesicles & ulcerations in respiratory routes
[gold standard) with enterovirus 71:
• Coxsackie A 16 the oral cavity) followed by exanthem • Communicability:
• Serotype neurologic disease,
• Enterovirus 71 • Exanthem: tender vesicular skin lesions with Viral shedding from the • Supportive
identification encephalomyelitis,
• IP 4-6 days surrounding erythema on the hands (more than feet), respiratorytrnct1-3weeks
for outbreak pulmonary edema,
buttocks, groin & fecalshedclingupto 7-11
investigation hemorrhage
• Vesicles resolve by absorption of nuid in ~1 week weeks post-infection
Source:KhegmanR, et al. NelsonTextbook
of Ped,atncs(21sted.).Ph1ladelphra;
2020
Redbook.Reportofthe Committee on Infectious
Diseases,31sted; 2018

Ill
POLIOMYELITIS
I. ETIOPATHOGENESIS
• Polio virus is an Enterovirus from Picornaviridae family
Etiology (coxsackie, echovirus, polio) with 3 antigenically distinct
serotypes (1. 2, and 3)
Mode of Transmission • Fecal-oral
• Shortly before & after the onset of clinical illness
Period of
• Virus persists in the throat until a week after onset & excreted
Communicability
in the feces for 3-4 weeks
Incubation Period • 7-21 days

II. DIAGNOSIS
DIAGNOSTIC REMARKS
• Blood (end of 1st week)
• Throat (end of 2nd week)
Isolation of virus
• Feces (end of 3rd week)
• CSF
• Pleocytosis with early predominance of PMNs followed by a
CSF analysis shift to mononuclear cells
• Normal glucose, increased protein

III. MANIFESTATIONS AND MANAGEMENT

TYPE MANIFESTATIONS MANAGEMENT
Inapparent • 90-95% asymptomatic • N/A
• Analgesics
• Sedatives
Abortive • Non-specific febrile illness • Bed rest for 2 weeks
• Neurological and musculoskeletal
re-assessment after 2 months
• Analgesics with hot packs for 15-30
minutes every 2-4 hours
Nonparalytic • Also called aseptic meningitis • Footboard or splint should be used to
keep the feet at a right angle to the legs
• Gentle physical therapy
• Suitable body alignment (feet at a
right angle, knees slightly flexed, hips
• Weakness of one or more
& spine straight)
Paralytic muscle groups, either skeletal
• Bethanecol 5-10 mg PO for bladder
or cranial
paralysis
• Tracheostomy for respiratory distress

IV. OTHER INDICATIONS OF POLIOVIRUS VACCINES

ORAL POLIOVIRUS VACCINE (OPV) INACTIVATEDPOLIOVIRUSVACCINE(IPV)
• Unimmunized persons at imminent • HIV positive
(within 4 weeks) risk of exposure to polio • Household contacts of an immunodeficient patient
• Contributes to herd immunity • Unimmunized persons at future risk of exposure

Source:KliegmanR. et at NelsonTextbook
of Pediatrics
(21sted.).Philadelphia:
2020
Redbook.Reportof the Committeeon InfectiousDiseases.31sted: 2018
214
DENGUE FEVER
I. ETIOPATHOGENESIS
A. Dengue Virus
0 Refers to four serotypes of RNAviruses from the genus Flaviridae (DENV-1, 2, 3, and 4)
0 Transmitted to humans through the bite of infected Aedes aegypti mosquito
0 Infection with one DENVtype produces life-long immunity against that type and a very
short period of protection against the other three serotypes - thereafte1; infection with
a different strain may predispose to more severe disease

8. Transmission

Incubation Period •1-7days

Period of • In mosquito: DENV replication for 8-12 days and remains

Communicability infectious for life
• Virus replication leads to viremia and formation of Ag-Ab
complex which triggers the complement cascade & activation
Pathophysiology
of Hageman factor
• This leads to increased vascular permeability and/or shock

II. MANIFESTATIONS
A. Characteristic Rash
0 Transient, macula,; generalized rash that blanches under pressure seen during the
first 24-48 hours of fever
1-2 days after defervescence, a generalized maculopapular rash appears which spares
the palms & soles; this disappears in 1-5 days and may desquamate (Hermann rash)
I
B. Phases of Dengue Infection

Course of dengue illness Febrile Critical Recovery

Days of illness 2 3 4 5. 6 7 8 9 10

Temperature

40'

Potential clinical issues

Laboratory changes
Dehydration

-
Organ impairment

' ......................
.
Reabsorption
Fluid overload

Platelet

Hematocrit
Serology and virology

Viraemia

215
 PHASE MANIFESTATIONS COMPLICATIONS
• Sudden high-grade fever &
• Anorexia, nausea, and vomiting
• Facial flushing, skin erythema, generalized rash • Dehydration
• Generalized body ache, myalgia, arthralgia • High fever may
Febrile Phase ("back-break fever" or "breakbone fever") cause neurologic
(3-7 days) • Retroorbital eye pain, photophobia, headache disturbances and
• Petechiae, mucosa) membrane bleeding, easy febrile seizures in
bruising, and bleeding may be present young children
• Enlarged and tender liver
• Leukopenia on CBC
• Not all pass through this phase
• Warning signs mark the onset of this phase as
a result of plasma leakage
• Weak pulses, cold clammy extremities, and
Critical Phase prolonged capillary refill time • Shock from plasma
(24-48 hours • Easy bruising and bleeding leakage
after fever • Shock may result in metabolic acidosis, • Severe hemorrhage
defervesence) progressive organ impairment, and • Organ impairment
disseminated intravascular coagulopathy (DIC)
• Progressive leukopenia followed by
thrombocytopenia & increasing hematocrit
(hemoconcentration)
• Gradual reabsorption of extravascular
compartment fluid • Hypervolemia and
• May have bradycardia, stabilization of acute pulmonary
Recovery hematocrit, or hemodilution edema (if IV fluid
Phase • Improvement of well-being & return of appetite therapy was
• Hemodynamic status stabilized & diuresis ensues excessive or extended
• Hermann's rash ("isles of white in a sea of into this period)
red") & may have generalized pruritus

IJI. 2009 WHO CLASSIFICATIONOF DENGUEFEVER

WHO CLASS CRITERIA

• Live in/travel to endemic areas

• Fever and two of the following criteria:
0Nausea, vomiting
Probable Dengue
0Rash
0Aches & pains
0Tourniquet test positive
0Leukopenia
0Any warning signs
Laboratory-
• Important when no signs of plasma leakage is present
Confirmed Dengue
• Warning signs include:
0 Abdominal pain or tenderness
0 Persistent vomiting
Dengue with ° Clinical fluid accumulation
Warning Signs 0 Mucosa) bleed
0 Lethargy, restlessness
0 Liver enlargement >2 cm
0 Increase in hematocrit with decrease in platelet
• Severe plasma leakage: shock, fluid accumulation with respiratory distress
• Severe bleeding (as evaluated by clinician)
Severe Dengue • Organ involvement
0 Liver: AST or ALT>1,000
° CNS:impaired consciousness
0 Heart and other organs
treatment,preventionandcontrol.WHO;2009
Source:WHO.Dengue:guidelinesfor d1agnos1s,
YipWCL.Denguehaemorrhagic
fever:currentapproachesto management. MedicalProgress;1980
216
IV. 2011 WHO CLASSIFICATION OF DENGUE INFECTIONS & GRADING OF SEVERITY
LABORATORY
CLASSIFICATION* MANIFESTATIONS
FINDINGS
• Fever with two of the following:
0Headache • Leukopenia (WBC
0Retro-orbital pain ,,; 5000 cells/mm')
0Myalgia • Thrombocytopenia
Dengue Fever Arthralgia
0
<150,000 cells/mm'
0Rash • Rising hematocrit
0Hemorrhagic manifestation** (5-10%)
• No evidence of plasma leakage

• Fever and hemorrhagic manifestation and

DHFI
evidence of plasma leakage

• Grade I plus spontaneous bleeding • Thrombocytopenia

DHF II
( e.g. epistaxis, palatal petichae) <100,000 cells/mm'
• Grade I or II plus circulatory failure • Hematocrit rise
DHF III*** (weak pulse, narrow pulse pressure, 2'20%
hypotension, restlessness)

• Grade Ill plus profound shock with

DHF IV***
undetectable BP and pulse
'Both 2012and 2011classificationcan be used (dependingon institutionalpreference)
"Hemorrhagic manifestationsincludepositivetourniquettest (2e10spots/ square inch),petechiae, purpura,ecchy-
mosis, epistaxis,gum bleeding,hematemesis and/or melena
... DHFIlland IVare considered Dengue Shock Syndrome(DSS)
I
Source:WHO.Comprehensive
guidelinesfor dengue.WHO:2011

V. DIAGNOSIS
A. Diagnostics
DIAGNOSTIC REMARKS
Dengue NS-1 Antigen • Useful from day 1 until day 3 of the illness

• Method of choice at the end of the acute phase of infection

• Dengue lgM: samples should be collected not earlier than
5 days nor later than 6 weeks after onset
Serology • Primary infection: dengue lgG is detectable in low titers at
(Dengue IgM/lgG) the end of 1st week of illness then increases slowly after
(lgG detectable after several months)
• Secondary infection: lgG detected even in the acute phase &
persists from 10 months to life

CBC • Leukopenia, then thrombocytopenia, or with hemoconcentration

• Prolonged bleeding time

PT/PTT
• Moderately decreased prothrombin level

• Elevation of serum transaminase

• Metabolic acidosis with hyponatremia
Blood chemistry
• Hypochloremia, elevated BUN, and hypoalbuminemia
(occasionally)

Chest radiograph • Pleural effusion may be present

217
 B.The "ABCS"of Dengue
0 Suggested laboratory investigations for patients who do not respond well with
adequate volume replacement (both shock and non-shock)
INVESTIGATION REMARKS
• Seen in profound shock
• Blood gas
A: Acidosis • Work up for organ involvement: liver
(venous or capillary)
function, BUN,Crea
• An increase in hematocrit suggests
hemoconcentration
• A decrease in hematocrit with improving
B: Bleeding • Hematocrit clinical condition suggests that volume
resuscitation is working
• A drop in hematocrit with worsening clinical
condition suggests occult bleeding
• Hypocalcemia in almost all cases of DHF
C: Calcium • Electrolye (Ca2 ·J
• Usually asymptomatic
• Factors that contribute to hypoglycemia:
S: Blood sugar • Blood sugar
decreased intake, vomiting, hepatic impairment

VI. MANAGEMENT
• Not all cases should be admitted
• Supportive therapy is the main management of dengue fever
Anti-pyretic: paracetamol (ibuprofen and NSAIDsare generally avoided)
0

Increase in fluid intake (oral rehydration solution or ORS)

0

Monitoring ofhematocrit and platelet count

0
General
Monitoring for complications
0

• Those with warning signs and complications (e.g., severe dengue, DHF
and DSS) should be admitted
• Maintenance of the cardiovascular and circulatory status is a priority
especially during the critical phase
• Fluid therapy is critical in the management of dengue because the main
pathophysiology is plasma leakage
• Isotonic fluids are preferred (e.g.,PNSS,PLR,IES-isotonic electrolyte solution)
• Fluids are adjusted based on:
0Hematocrit determination
Fluid
° Clinicalstatus: BP (watch out for narrowed pulse pressure & hypotension),
Therapy
sensorium, capillary refill time, extremities (cold & clammy), pulses
0Urine output (maintain a minimum of 1 mL/kg/hr in children and
0.5 mL/kg/hr in adolescents)
• After the critical phase, fluids should be tapered off or withheld to
prevent fluid overload (for specific guidelines, see algorithm below)
• Prophylactic transfusion of blood products, especially platelets and
plasma (FFP and cryoprecipitate) is not recommended (no proven
benefit & may contribute to fluid overload and adverse reactions)
• Whole blood or PRBCtransfusion is indicated for profuse bleeding or
Blood clinical deterioration that does not respond to fluid resuscitation
Transfusion • FFP and cryoprecipitate are indicated for bleeding that does not respond
to whole blood or prbc transfusion OR in cases of DIC
• Platelet transfusion is indicated if:
0Platelet :,:10,000/mm 3 with bleeding, or
0Prolonged shock with bleeding not responsive to red cell & plasma products
• Afebrile for at least 24-48 hours
Criteria for • Good well being
discharge • Stable hematocrit without !VF and increasing platelet trend
• At least 2-3 days from the last episode of shock

218
A. Fluid therapy for Patients Who are NOT Admitted or Patients NOT in Shock

• Use Ludan's method to approximate fluid

(see Fluid and Electrolyte chapter)
Admitted Patients NOT in Shock
• Use Holliday Segar (see Fluid and Electrolyte
chapter)
Without • Constant fluid adjustment depending on
warning signs clinical status and laboratory parameters
(DHFI-11) • Maximum recommended fluid in 24 hours
is 3 liters
• Isotonic fluid • Use ideal body weight for obese patients
• Start with 5-7 mL/kg/hour of IV fluid for 1-2
hours then
With warning • Reduce to 3-5 mL/kg/hour for 2-4 hours then
signs • Reduce to 2-3 mL/kg/hour or less
• Reduced based on clinical status and
laboratory parameters

B. Fluid Therapy for Patients in Shock

1. Compensated Shock
BP maintained but with signs of reduced perfusion: e.g., narrow pulse
pressure, weak pulses, cold and clammy extremities, prolonged CRT
I
STEP MANAGEMENT
• Obtain baseline hematocrit (HCT)
• Give crystalloid 10-15 mL/kg over 1 hour
• Give 02 support
A
• If with improvement, go to STEP B
• If without improvement, go to STEP C
• Reduce fluids as follows (as long as clinical and laboratory status are stable)
05-7 mL/kg/hour for 2-4 hours
B 03-5 mL/kg/hour for 2-4 hours
02-3 mL/kg/hour for 2-4 hours
• Second bolus of fluid (crystalloid/colloid) 10-20 mL/kg/hour in 1 hour
0If patient improves and HCT decreases proceed to STEP B
If patient does not improve, give 3rd bolus offluid (crystalloid/colloid)
C
0

10-20 mL/kg/hour in 1 hour

. If patient improves, proceed to STEP B
• If patient does not improve, consider inotropes & refer to tertiary center

Ifwithovert/occultbleeding(at any time, transfuse fresh whole blood20 ml/kg or PRBC10 ml/kg
• If patient improves,proceed to STEPB
• If patient does not improve,consider inotropes and refer to tertiarycenter

219
2. Hypotensive Shock
Defined as hypotension for age
• In BOLDtext are the key differences from compensated shock

STEP MANAGEMENT
• Obtain baseline hematocrit (HCT)
• Give isotonic crystalloid or colloid 20 ml/kg over 15 minutes via
"push-pull" method
A
• If with improvement, go to STEP B
• If without improvement, go to STEP C
• Reduce fluids as follows (as long as clinical and laboratory status are stable)
0 10 ml/kg/hour for 1 hour
B 0 5-7 mL/kg/hour

0 3-5 mL/kg/hour
for 2-4 hours
for 2-4 hours
0 2-3 mL/kg/hour for 2-4 hours
• Second bolus offluid (colloid) 10-20 ml/kg/hour in½ to 1 hour
0 If patient improves and HCT decreases, reduce IVF to 7-10 ml/kg/hour
for 1-2 hours and if patient remains stable proceed to STEP B
C 0 If patient does not improve, give 3rd bolus offluid (crystalloid/colloid)
10-20 mL/kg/hour in 1 hour
If patient improves, proceed to STEP B
• If patient does not improve, consider inotropes and refer to tertiary center

If withovert/occultbleeding (at anytime, transfuse fresh whole blood 20 mUkg or PRBC10 mUkg
• If patient improves, proceed to STEPB
• If patient does not improve,consider inotropes and refer to tertiarycenter

3. Other Key Recommendations

If patient is stable and HCT increases by 10%, assess the need to increase fluid
If patient is unstable and HCT increases, assess accordingly and manage as either
compensated or hypotensive shock
If patient is unstable and HCT suddenly drops, look for signs of bleeding and
consider transfusion
Patients who are stable for 48 hours should be tapered off IVF and given oral fluids
For obese patients, ideal body weight is used (common clinical practice is to use
a maximum of 50 kg for computation of fluids in patients whose weight is> 50 kg)

Source:WHO.Guidelinesforprevention
andcontrolofdengueanddenguehaemorrhagic fever.WHO:2011
WHO.Dengue:guidelines
fordiagnosis,
treatment.
preventionandcontrol
-- Newedition.Geneva:WHO:2009
Administrative
OrderNo. 2012-0006.DOHRevisedDengueClinicalCaseManagement Guidelines2011

220
RABIES
I. ETIOPATHOGENESIS
A. Transmission

Etiology • Rabies virus is an RNAvirus of the Rhabdoviridae family

• Bite of rabid animal (dogs, cats, bats, and cattle) or by licking
the mucosa or open wound
Mode of
• Probable aerosol exposure in laboratory
Transmission
• Possible airborne exposure in caves inhabited by bats
• Person to person transmission by bite: not documented
• Extremely variable, ranging from 4 days to 7 years but
Incubation Period
generally 20-90 days

B. Pathophysiology
, Rabies virus inoculation begins with an animal bite and local tissue replication in the
muscle and skin
, The virus attaches to nicotinic acetylcholine receptors and gains access into the
peripheral nervous system. It then moves centripetally eventually reaching the spinal
cord and the brain, salivary, and lacrimal glands.
Virus causes neuronal destruction in the brainstem & medulla
Negri bodies (pathognomonic): rabies specific eosinophilic inclusions within the

I
neuronal cytoplasm

II. MANIFESTATIONS
A. Prodrome (2-10 days)
Pain, pruritus, paresthesia, myoclonic jerking at the site of the wound/inoculation
' Fever, malaise, anorexia, dysphagia, changes in behavior, sleep patterns and emotions

B.Acute Neurologic Phase

• Excessive restlessness, uncontrollable excitement, agitation,
Furious type
confusion, hallucination, combativeness, focal paralysis,
(99%)
hydrophobia, aerophobia, hypersalivation
• Ascending symmetric paralysis with flaccidity & decreased
Paralytic phase
DTRs, respiratory paralysis, coma

III. DIAGNOSIS
• CBCreveals polymorphonucleosis
• CSF revelas mononuclear cells with increased protein
• Isolation of virus from saliva or CSF
• Detection of antibody in serum & CSF
• Postmortem diagnosis: fluorescent microscopy of brain & salivary glands
• Animal brain: Negri bodies

IV. POST-EXPOSURE PROPHYLAXIS (Management after an animal bite)

• See preventive pediatrics chapter

V. PROGNOSIS
• Rabies is generally fatal
• Neither rabies immunoglobulin nor rabies vaccine provides benefit once symptomatic

Source:KliegmanR,et al. NelsonTextbook

of Pediatrics
(21sted.).Philadelphia:
2020
Redbook. Reportof theCommitteeon Infectious
Diseases, 31sted:2018

221
HUMAN IMMUNODEFICIENCY VIRUS (HIV) INFECTION
I. ETIOPATHOGENESIS
A. The Human Immunodeficiency Virus (from Retroviridae family)

• Most prevalent pathogenic species

HIV 1 (99%) • Associated with reported AIDS (Acquired Immunodeficiency
Syndrome, the advanced stage of HIV infection)
• Less pathogenic
HIV2
• Limited to West Africa

B.Transmission
• Sexual contact
• Percutaneous blood exposure from contaminated needles and sharp
Modes of instruments
Transmission • Transfusion of contaminated blood products
• Vertical transmission can occur before (intrauterine), during
(intrapartum), or after delivery (through breastfeeding)
Incubation • 12-18 months for perinatally infected infants
Period • Some children are asymptomatic for >S years

C. Pathogenesis of HIV/AIDS
0 Depletion of CD4+ T cells leads to immunodeficiency
0 HIV infection ultimately leads to the impairment of every arm of the immune system

II. MANIFESTATIONS
A. Progression of Untreated HIV through 4 Clinical Phases

PHASE DESCRIPTION
Acute retroviral
• Flu-like syndrome
syndrome
• HIV DNA integrates into CD4+ cells
Latent infection
• Declining CD4+ counts
• Inability of immune system to contain viral replication
• Manifestations may include:
0 Generalized lymphadenopathy
0 Hepatosplenomegaly
° Failure to thrive
Symptomatic HIV ° Chronic or recurrent diarrhea
0 Interstitial pneumonia
0 Oral thrush
0 Recurrent bacterial infections
0 Lymphocytic interstitial pneumonitis
0 Early onset of progressive neurologic deterioration
• CD4 count <200 cel!s/uL
AIDS • Patient succumbs to opportunistic infection within 2 years
of developing AIDS

222
 B. HIVClassification
CATEGORY REMARKS
• At least 2 mild symptoms: lymphadenopathy, parotitis,
A Mild Symptoms hepatosplenomegaly, dermatitis, persistent or
recurrent sinusitis or otitis media
• Any of the following: oropharyngeal thrush persisting
for >2 months, recurrent or chronic diarrhea, persistent
Moderate
B fever for> 1 month, hepatitis, recurrent herpetic
Symptoms
gingivostomatitis, pneumonitis, disseminated varicella
with visceral involvement, cardiomegaly
• Two of the following serious bacterial infections:
sepsis, meningitis, pneumonia in a 2-year period,
C Severe Symptoms lower respiratory tract candidiasis, cryptococcosis,
encephalopathy, malignancies, disseminated
mycobacterial infection, pneumocystis carinii pneumonia

III. DIAGNOSIS
• Demonstration of lgG to HIV by a repeatedly reactive enzyme
immunoassay & confirmatory test (Western immunoblot or

I
IF assay) establishes the diagnosis of HIV infection
• Some viral detection assays:
Any child >18 months
HIV DNA or RNA by PCR
0

HIV culture
0

HIV p24Ag
0

Immune complex-dissociated p24 Ag

0

• Viral diagnostic testing should be done within the first 2 days

For infants born to of life, at 1-2 months old & at 4-6 months old
HIV-infected mothers • CD4+ & CDS+ lymphocyte counts done at 1 & 3 months old &
repeated every 3 months starting at 6 months of age

IV. MANAGEMENT
• Initiating antiretroviral treatment for pediatric HIV-infected patients are based on:
Magnitude of viral replication
° CD4+ count & percentage
° Clinical condition
• HIV-infected children with symptoms (clinical category A, B, C) or with evidence of
immune dysfunction should be treated with antiretrovirals regardless of age or viral load
• In general, the best single prognostic indicator is the plasma viral load

ANTIRETROVIRAL REMARKS
THERAPY
• Either nucleoside (NRTI) or non-nucleoside reverse
Reverse transcriptase inhibitors (NNRTI)
transcriptase 0 NRTI: Didanosine, Abacavi1; Stavudine, Lamivudine,
inhibitors Zidovudine
0 NNRTI: Delaviridine, Efavirenz, Nevirapine
• Prevent packaging of infectious virions before they leave the
infected cells
Protease inhibitors
• Examples include Indinavi1; Amprenavir, Nelfinavil; Ritonavir,
Saquinavir

223
 SECTION FOUR
BACTERIAL INFE'CT.IONS

STREPTOCOCCAL INFECTIONS

I. STREPTOCOCCUS PNEUMONIAE (PNEUMOCOCCUS)

• Gram-positive, lancet-shaped polysaccharide encapsulated diplococcus
• Encapsulated strains cause most serious disease in humans
• Important cause of secondary bacterial pneumonia in patients with influenza
• Major cause of life-threatening pneumonia, meningitis, and septicemia

Transmission • Spread from person to person by droplet transmission

• Signs and symptoms are related to the anatomic site of
disease
• Common clinical syndromes: otitis media, sinusitis,
pneumonia, and sepsis
Manifestations • Local complications of infection may occur leading to
empyema, mastoiditis, pericarditis, epidural abscess,
periorbital cellulitis, or meningitis
• Pneumococcal meningitis may result in sensorineural
hearing Joss in 20-30%
• Culture: identification of the organism from the site of
Diagnosis
infection, blood, or sterile body fluid
• In patients > 1 month old:
0 Vancomycin IV 60 mg/kg/day every 6 hours, and
Meningitis
0 High-dose Cefotaxime IV 300 mg/kg/day every 8 hours
OR Ceftriaxone IV 100 mg/kg/day every 12 hours
Management Pneumonia • See Pulmonology chapter
Otitis media • High doses of Amoxicillin 80-100 mg/kg/day
Those allergic • Macrolide
to penicillin • Cotrimoxazole
Control measures • Use of PCV 13 and PCV23 vaccines

II. GROUP B STREPTOCOCCAL (GBS) INFECTIONS

• Streptococccus agalactiae: gram-positive aerobic diplococci that account for 95% of
Streptoocccal infections among infants
• Major cause of neonatal bacterial sepsis (see discussion on neonatal sepsis)

224
III. GROUP-ASTREPTOCOCCALINFECTIONS(GAS)
• Streptococcus pyogenes is the most common & only clinically significant GASorganism
which infects children & adolescents (it causes a wide range of infections)
• Most common site of infection is the pharynx (followed by skin infections such as
pyoderma, impetigo)
Non-suppurative complications include:
0 Acute rheumatic fever (sequela of pharyngitis, NOT skin infections)
0 Acute glomerulonephritis (sequela of pharyngitis OR skin infections)
A.Acute Pharyngitis
• Contact with respiratory tract secretions
Transmission • Communicability highest during acute infection
• Children become non-contagious 24 hours after antibiotic initiation
Incubation • 2-5 days
• Feve1;sore throat, tonsillar inflammation ("beefy red tonsils") with
exudate, tender cervical lymphadenopathy
Manifestations
• Most often associated with scarlet fever (e.g., strawberry tongue,
erythematous sandpaper-like rash due to an erythrogenic exotoxin)
• Throat swab culture: 95% sensitivity to detect GAS
Diagnosis • Rapid antigen detection test: more expensive but yields fast results
(a negative rapid test does not exclude GAS)

Management
• Penicillin or amoxicillin is the drug of choice given for 10 days
, Oral Penicillin-V (Phenoxymethylpenicillin):

0
250 mg/dose 2-3 times a day if< 60 lbs
500 mg/dose 2-3x a day if >60 lbs
Amoxicillin 50 mg/kg/day in divided doses x 10 days
I
• For penicillin-allergic patients: oral clindamycin or oral macrolide
(e.g., erythromycin, clarithromycin, or azithromycin)

B. Impetigo (Pyoderma)
Incubation • 7-10 days
• Nonbullous impetigo (more common, 70%)
0 Staphylococcus aureus is the predominant cause ofnonbullous
impetigo
, Superficial infection: discrete papulovesicular lesion with
localized erythema that become purulent & covered with a thick,
confluent, amber-colored crust
0 Most common on the face and extremities

Manifestations 0 Usually start from insect bites, abrasions, chickenpox, scabies

0 Regional lymphadenitis is common (90% of cases) with
occasional pruritus
• Bullous impetigo
0 Less common and often seen in neonates and young infants
° Flaccid and transparent bullae <3 cm on previously
untraumatized skin (face, trunk, buttocks, perineum)
• Culture of the lesion to distinguish nonbullous impetigo due to
Diagnosis Staphylococcus aureus from GAS (not routinely indicated)

• Mupirocin 2% ointment or Retapamulin 1 % 2-3x/day for 10-14 days

limit person to person spread of GASand eradicates localized disease
• Oral antibiotic active against both GASand S.aureus:
Management
, Cefalexin 25-50 mg/kg/day in 3-4 divided doses for 7-10 days
, If no response in 7 days, do a culture and give clindamycin,
doxycycline,or cotrimoxazole for 7 days especially if considering MRSA

225
STAPHYLOCOCCAL INFECTIONS
• Gram-positive aerobic bacteria that grow in pairs and clusters
• Coagulase positive: Staphylococcus aureus (most common cause ofpyogenic infection of
the skin and soft tissues)
• Coagulase negative: S. epidermidis, S. saprophyticus, S. haemolyticus
• Clinical spectrum: osteomyelitis, suppurative arthritis, abscesses, pyomyositis, empyema, IE
• Toxin-mediated: scarlet fever, food poisoning, scalded skin syndrome, toxic shock syndrome

I. STAPHYLOCOCCAL SCALDED SKIN SYNDROME rssss)

• S. aureus of phage group II elaborates a staphylococcal exfoliatin A that
Pathogenesis acts on the cell surface of the epidermal granular cells
• Injury results in intraepidermal separation of cells within granular layer
• Spectrum: from bullous impetigo to generalized involvement
• Faint red eruption in face, neck, axilla, and groin
Manifestations • Skin rapidly becomes tender with crusting around eyes, mouth, & neck
• Mild rubbing of the skin results in epidermal separation leaving a
shiny, moist, red surface (Nikolsky sign)
• Oxacillin IV for methicillin sensitive staph aureus (MSSA)
Management • Clindamycin or Vancomycin for MRSA
• Fluid and electrolyte correction

II. TOXIC SHOCK SYNDROME [TSS)

• Due to TSST-1-producing strains of Staphylococcus aureus
Etiology and
• TSST-1 act as superantigens which trigger cytokine release leading to
Pathogenesis
massive fluid loss and end-organ cellular injury
• Affects women, men, and children
• Highest rates in menstruating women 15-25 years old
Epidemiology • Non-menstrual TSS is associated with infected nasal packing and
wounds, sinusitis, pneumonia, tracheitis, empyema, burns, abscesses,
osteomyelitis
• Acute onset of fever, diarrhea, vomiting, headache, myalgia
• Diffuse red macular rash appears within 24 hours
Manifestations
• Recovery occurs within 7-10 days with desquamation especially of
palms and soles

Major Criteria • Abrupt onset of fever with temp of >38.8°C

(all are required) • Hypotension (shock, orthostatic)
• Inflamed mucous membranes (oropharyngeal,
conjunctiva!, vaginal, strawberry tongue)
• Vomiting, diarrhea
• Liver abnormalities (bilirubin or transaminase >2x
the upper limit of normal)
Minor Criteria • Renal abnormalities (BUN or creatinine >2x the
Diagnosis (at least 3) upper limit of normal)
• Muscle abnormalities (myalgia or CPK >2x the
upper limit of normal)
• CNS abnormalities ( change in sensorium without
focal neurologic signs)
• Thrombocytopenia (<100,000/mm')
• Absence of another explanation
Exclusionary
• Negative blood cultures (except occasionally for S.
Criteri~
aureus)
• Antistaphylococcal antibiotic (oxacillin or nafcillin)
Management + clindamycin to decrease toxin production
• Consider Vancomycin where MRSArates are very high

226
ESCHERICHIA COLI (E. coli)
I. ETIOPATHOGENESIS
• Person to person transmission
Mode of Transmission
• Fecal-oral route (contaminated food & water)
Incubation Period • 10 hours to 6 days
Infectious period • As long as the person excretes the bacteria (1-4 weeks)

II. MANIFESTATIONS
• Most fecal E.coli are nonpathogens
• Six major groups of pathogenic£. coli:
AGE GROUP TYPE OF OTHER
PATHOGENESIS MANIFESTATIONS
AT RISK DIARRHEA
I

1) Enterotoxicogenic E. coli (ETEC)

• Most common • Adherence factors • Watery diarrhea • Abdominal pain
cause of • Heat-labile and heat • Nausea, vomiting,
travelers' stable enterotoxins little or no fever
diarrhea) • Usually self-limited

2) Enteroinvasive E. coli (EIEC)

• >l year old • Adherence
• Invasion and
innammation of large
intestine
3) Enteropathogenic E. coli (EPEC)
• <2 years old • Adherence
• Effacement of intestines
4) Enterohemorrhagic (EHEC)or Shiga Toxin-Producing or Verotoxin Producing E. coli
• 6 months- • Shiga toxin production,
10 years old large bowel attachment
• Elderly • E.coli O157:H7 is the
• Poorly cooked most virulent serotype
hamburger is a and most frequently
common cause of associated with HUS
outbreaks
5) Enteroaggregative E. coli (EAEC)
• < 2 years old • Small and large
• HIV patients intestine adherence
• Travelers • Enterotoxin and
cytotoxin production
6) Diffusely Adherent E. coli (DAEC)
• >l year old • Adherence factors
• Travelers • Toxin production
• Watery diarrhea or
dysentery
• Feve1~systemic
toxicity
• Crampy abdominal
pain, tenesmus
• Often with postive
I
fecal leukocytes
• Watery, non bloody • Vomiting
diarrhea with mucus • Low grade fever
• Initially watery but • Fever is not
becomes bloody commonly present
• 5-10% develop
systemic
complications such
as HUS
• Watery, mucoid • Low grade fever
diarrhea • Little to no vomiting
• Some patients may
have bloody stools
• Watery diarrhea • Abdominal pain
• Fever

Ill. DIAGNOSIS
• Clinical features of illness are seldom distinctive
• Routine laboratory tests are of very limited value
• Serotype O157:H?: suggested by isolation of£. coli that fails to ferment sorbitol on
MacConkey sobitol medium
• For the others: tissue cultures or identification of specific virulence factors of the bacteria

227
IV. MANAGEMENT
ASPECT MANAGEMENT
• Fluid and electrolyte therapy
Supportive
• Early refeeding (within 6-8 hours of initiation of rehydration)
• Specific antimicrobial therapy is problematic due to difficulty of
making a diagnosis & unpredictability of antibiotic susceptibilities
• Antibiotics should not be given for STEC or EHEC infection due to
Antimicrobials
increased risk of HUS
• Antibiotics may be useful for severe watery diarrhea in a traveler
coming from a developing country (ETEC responds to co-trimoxazole)
Zinc supplements • For malnourished children to hasten recovery
Prevention • Proper food and water handling procedures, handwashing
Source:KliegmanR. et al. NelsonTextbookof Pediatrics(21sted.). Philadelphia:2020
KimberlinDW,et al.AmericanAcademyof Pediatrics.Redbook:2015

TYPHOID FEVER
I. ETIOPATHOGENESIS
• Salmonella enterica serovar Typhi
Etiology
• Salmonella Paratyphi A, Band C (causes less severe disease)
• Most common: ingestion of food/water contaminated from human feces
• Shellfish and vegetable grown in sewage contaminated water
Transmission
• Communicable as long as the infected person excretes S. typhi (after
the 1" week of illness up to convalescence)
Incubation • 7-14 days, range of3-30 days

II. MANIFESTATIONS
A. Symptoms/Signs
• High-grade fevet; malaise, myalgia, abdominal pain, anorexia,
hepatosplenomegaly, diarrhea/ constipation
• Temperature pattern: rises in small increments then reaches 40°C
Symptoms
by the end of the 1" week
• Fever is remittent and rises to peak every afternoon
• Diarrhea (greenish pea soup) follows fever on the 2"d week
• Maculopapular rashes (rose spots): visible on day 7-10 of illness on
the lower chest or abdomen and lasts 2-3 days (seen in 25% only)
Signs
• Relative bradycardia, neurologic manifestations, and GI bleeding
are rare in children, but relatively common in adults

B. WHO Standard Case Definition

CASE
DESCRIPTION
DEFINITION

• Persistent fever (38 °C) lasting "'3 days, with a positive

serodiagnosis or antigen detection test but no S. typhi isolation
Probable Case
• A clinical compatible case that is epidemiologically linked to a
confirmed case or outbreak
• Persistent fever (38 °C) lasting "'3 days with laboratory confirmed
Confirmed Case S. typhi organisms (blood, bone marrow, bowel fluid)
• A clinical compatible case that is laboratory confirmed
• Excretion of S. typhi in the urine or stool for> 1 year after the onset
Chronic Carrier of typhoid fever
• The risk of becoming a chronic carrier is 2-5%

228
III. DIAGNOSIS
DIAGNOSTIC REMARKS
• Mainstay of diagnosis: blood culture (positive in 40-60% of
patients early in the disease course)
Cultures
• Stool and urine cultures become positive after the 1st week
• Bone marrow cultures
• Widal test (become positive in 7-10 days)
Typhoid Tests
• Typhidot lgM and lgG
• Leukopenia (leukocytosis in younger children), thrombocytopenia
Other Tests
• Deranged liver function tests

IV. MANAGEMENT (Based on Local Sensitivity Pattern)

SENSITIVITY ANTIBIOTIC
PATTERN

• Uncomplicated typhoid fever

• First line: Chloramphenicol OR Amoxicillin OR Ampicillin OR
Cotrimoxazole
Fully susceptible
• Second line: Cefixime OR Azithromycin OR Ciprofloxacin
• Complicated typhoid fever

Multidrug-
Resistant
• Ceftriaxone OR Ciprofloxacin OR Azithromycin

• Ciprofloxacin
Guidelines2017
Source::NationalAnt1b1ot1c
I
V. PREVENTION
• Central chlorination and domestic water purification
• Avoid consumption of street food
• Typhoid vaccine for high risk populations Source:CDCandPrevention. Salmonella.
Veeraraghavan
8, et al.Typhoidfever;FutureSciOA;2018
WHOGuidelines forTyphoidFever.2011.

MENINGOCOCCEMIA
I. ETIOPATHOGENESIS
A Etiology
Etiologic agent • Neisseria meningitidis
Incubation
• 3-4 days (range of2-10 days)
period
• Close contact through aerosol droplets or contact with respiratory
Transmission secretions
• Humans are the only natural reservoir

B. Epidemiology
Sero groups A, B, C, W, Y: responsible for almost all cases of disease
Highest rate of disease occurs in infants <l year old
• Carriage of bacteria peaks during adolescence and young adulthood

C. Risk Factors
Respiratory viral infections (especially influenza)
Male gender
• Smoking, marijuana use, binge drinking
• Crowded living conditions (living in dormitories)
Underlying chronic diseases
• Low socioeconomic status
CDCandPrevention.Meningococcal Disease
HarrisonLH,et al. PediatrInfectDisJ; 2018
229
II. MANIFESTATIONS
• Most deaths occur within 48 hours of hospitalization
• Case fatality rate for invasive meningococcal disease is 5-10%
A. Different Presentations
PRESENTATION ANTIBIOTIC

• Most common presentation of invasive meningococcal infection

Meningococcal • Fever, headache, photophobia, nuchal rigidity, bulging fontanels
meningitis • Seizures & focal neurologic signs occur less frequently in
(30-50%) meningococcal meningitis compared to meningitis from
Streptococcus pneumoniae or Haemophilus influenzae type b
• Nonspecific symptoms (fever, irritability, lethargy, respiratory
Acute symptoms, vomiting, sore throat, diarrhea), followed by cold
meningococcal
septicemia hands/feet, abnormal skin color, prolonged capillary refill time,
non-blanching petechial rash
Fulminant • Rapid progression from nonspecific symptoms to septic shock
meningococcal
septicemia • Prominent petechiae and purpura (purpura fuhninans) within hours

Waterhouse- • May be see in fulminant cases

Friderichsen
syndrome • Diffuse adrenal hemorrhage leading to adrenal failure

Occult
meningococcal • Fever with or without associated symptoms
bacteremia
• Rare
Chronic
• Fever, arthralgia, headache, splenomegaly, maculopapular or
meningococcemia
petechial rash, lasting for 6-8 weeks

· Mostcases have asymptomaticcarriageinthe nasopharynx

B p .
POOR PROGNOSTIC FACTORS POORER PROGNOSTIC FACTORS

• Hypothermia or hyperpyrexia
• Presence ofpetechiae <12 hrs before
• Hypotension or shock, seizures
admission
• Purpura fulminans
• Absence of meningitis
• Leukopenia, thrombocytopenia, acidosis
• Low or normal ESR
• High levels of endotoxin and TNF-alpha

Ill. DIAGNOSIS
• Identification of meningococci from blood, CSF,joint fluid or skin lesions

IV. MANAGEMENT
• Empiric antimicrobial therapy: third-generation cephalosporin
• Drug of choice for meningococcemia:
0 Penicillin G 300,000 units/kg/day IV in 4-6 divided doses
° Ceftriaxone 100 mg/kg/day IM or IV 1-2x/day
° Cefotaxime 200-300 mg/kg/day IM or IV every 6 or 8 hours
• Treatment duration: 5-7 days

V. PREVENTION
• Household, school or day care contacts during the 7 days before onset of illness should
receive antibiotic prophylaxis
• Prophylaxis not routinely recommended for medical personnel except those with
intimate exposure (intubation, suctioning, mouth to-mouth resuscitation):
0 Rifampicin 10 mg/kg PO every 12 hrs for a total of 4 doses (max 600 mg/dose);
5 mg/kg/dose for <l month old
° Ceftriaxone 125 mg single dose IM for <15 yrs old, 250 mg single dose IM for 2:15 years old
° Ciprofloxacin 500 mg PO as a single dose 2: 18 years old
• Meningococcal vaccination for high risk population
230
PERTUSSIS
I. ETIOPATHOGENESIS
• Bordetella pertussis
Etiologic agent
• Bordete/la parapertussis

Incubation
• 7-10 days (range of4-21 days)
period
• Aerosol droplets
Transmission • Extremely congatious [attack rates as high as 100% in susceptible
individuals)

II. MANIFESTATIONS
STAGES* REMARKS
• Non-specific symptoms of low-grade feve1; colds, congestion, sneezing,
Catarrhal stage (1-2
lacrimation, conjunctiva! suffusion
weeks)
• Patients are most infectious during this period
• Coughing marks the onset (starts as dry, intermittent and irritative
hack)
• Cough evolves into paroxysms of 5-10 rapid coughs ending in a high-
pitched whoop (forceful inspiratory gasp), described as "whooping
Paroxysmal stage
(2-6 weeks)
cough"
• Cyanosis during paroxysmal coughing, posttussive vomiting and
exhaustion, conjunctiva! hemorrhage, and petechiae on upper body is
common
• In between paroxyms, child may look well
I
Convalescent stage
• Number, severity, and duration of cough episodes diminish
(>2 weeks)

'Infants <3 months old may not manifestwiththe classic 3 stages (apnea with or withoutcough may be the
presenting symptom)

III. DIAGNOSIS
Clinical case
• Cough lasting ;,14 days, with paroxysmal cough, inspiratory whoop or vomiting
definition
Laboratory • Culture or PCR (specimen obtained through posterior nasopharyngeal swab)
confirmation • Paired serology

IV. MANAGEMENT
• Macrolides: drug of choice
• Infants <l month old should be treated with azithromycin due to higher risk of
developing infantile hypertrophic pyloric stenosis with erythromycin & clarithromycin
ANTIBIOTIC REMARKS

• <6 months: 10 mg/kg/day for 5 days

Azithromycin • ;,6 months: 10 mg/kg (max 500 mg/dose) on day 1 then 5 mg/kg/day
[max 250 mg/dose) on days 2-5
Erythromycin • SO mg/kg/day 4x daily for 14 days (max 1-2 g/day)
Clarithromycin • 15 mg/kg/day twice daily for 7 days [max 1 g/day)
CDCandPrevention.
RecommendedAntimicrobialAgentsfor Pertussis:2005CDCGuidelines;2005
Redbook.Reportof theCommitteeon InfectiousDiseases,31sted; 2018

V. PREVENTION
• Household contacts should be treated with macro!ides (same dose as in management)
• Ensure complete immunization of children, adolescents and pregnant women (refer to
preventive pediatrics chapter)
231
TETANUS
• Acute spastic paralytic illness due to tetanus toxin (tetanospasmin)
• Favorable prognosis: long incubation period, absence of fever, localized disease
• Unfavorable prognosis: onset oftrismus <7 days after injury, generalized tetanic spasms
occuring <3 days after onset oftrismus, cephalic tetanus
I. ETIOPATHOGENESIS
Etiologic agent • Clostridium tetani
Incubation • 2-14 days, but may occur months after an injury
• Associated with traumatic injury(penetrating wound from a dirty object)
Transmission
• Introduced spores germinate, and produce tetanus toxin

II. MANIFESTATIONS
TYPES REMARKS
Neonatal or • Occurs within 3-12 days of birth
umbilical
tetanus (most
• Progressive difficulty in feeding
common form) • Hunger, crying, paralysis, stiffness and rigidity to touch, spasms

• Trismus (masseter muscle spasm or lockjaw) is common

• Headache, restlessness, irritability, fever, followed by stiffness,
difficulty chewing, dysphagia, neck muscle spasm
• Risus sardonicus (sardonic smile of tetanus)
Generalized • Opisthotonus (arched posture of extreme hyperextension)
tetanus • Laryngeal & respiratory muscle spasm can lead to airway obstruction
• Seizures: sudden tonic contractions of the muscles
• Smallest disturbance by sight, sound, or touch may trigger a tetanic spasm
• Dysuria and urinary retention from bladder sphincter spasm
• Autonomic symptoms: tachycardia, arrhythmia, labile hypertension
• Painful spasms of muscles adjacent to wound site
Localized • May precede generalized tetanus
tetanus • Cephalic tetanus: rare form of localized tetanus involving bulbar
(less common) musculature, results in retracted eyelids, deviated gaze, trismus, risus
sardonicus, spastic paralysis of tongue and pharyngeal membrane

III. MANAGEMENT
• Human tetanus immunoglobulin 500 U single IM dose neutralizes
tetanus toxin
Vaccination
• lfTIG unavailable: human !Vig,equine or bovine-derived tetanus antitoxin
• Upon discharge, give tetanus toxoid

Antibiotics
• Metronidazole PO/IV is the drug of choice
• Penicillin G (erythromycin and tetracycline if penicillin-allergic)
• Wound excision & debridement
• For generalized tetanus: muscle relaxants and neuromuscular blockers
Supportive
• Intubation to prevent aspiration, early tracheostomy in severe cases
• Quiet, dark, secluded setting

IV. PREVENTION
• Vaccination with DTaP,Tdap and Td
• Pregnant women should receive 1 dose of Tdap during each pregnancy, at 27-36 weeks AOG
• Tetanus prophylaxis in wound management:
° Clean minor wounds and <3 doses ofTd: Tdap or Td
• Clean minor wounds and ,;:3 doses ofTd within the past 10 years: No need for toxoid
All other wounds and <3 doses ofTd: Tdap or Td and TIG
All other wounds and ;;;3doses ofTd within the past 5 years: No need for toxoid/TIG
Source:CDCandPrevention.
Tetanus;
2019
232
TUBERCULOSIS (TB)
I. ETIOPATHOGENESIS
Etiologic agent • Mycobacterium tuberculosis
• Inhalation of airborne mucus droplet nuclei
Mode of
• Factors increasing transmission:·extensive upper lobe infiltrate or cavity,
Transmission
copious sputum production, severe and forceful cough, poor air circulation
Incubation • 3 months to 2 years
• Aerosol droplets
Transmission
• Extremely congatious

II. MANIFESTATIONSOF TUBERCULOSIS(Pulmonary TB is discussed in Chapter 12)

Scrofula (TB of • Most common form of extra pulmonary TB
the lymph nodes) • Enlarging nontende1; often unilateral lymph nodes

Tuberculous • Usually unilateral

pleural • Fever, dyspnea, chest pain on deep inspiration
effusion • Diminished breath sounds on the ipsilateral side
CNSTB • Most serious complication in children (see Neurology chapte11
Bone and joints • Most likely to involve the vertebrae (Pott disease)
Cardiac TB

Gastrointestinal
TB
• Most common form is pericarditis (e.g., chest pain, friction rub)
• May manifest as painless ulcer in the oral mucosa, TB peritonitis
(abdominal pain, ascites, anorexia, fever), or TB enteritis (abdominal
pain, diarrhea or constipation, weight loss, fever)
I
Renal TB {Rare) • Sterile pyuria, hematuria, dysuria, flank or abdominal pain
• Congenital transmission via hematogenous or transplacental spread
from an infected mother or during the postnatal period via inhalation
of airborne droplet nuclei
• Any infant with TB lesions & 1 or more of the following (Cantwell
Congenital
Tuberculosis Criteria, 1994):
0 Present within the first week of life
0 A primary hepatic complex or caseating hepatic granuloma
0 TB infection of placenta or endometrial TB in mother; or exclusion of the
possibility of postnatal transmission by excluding TB in other contacts

Ill. DIAGNOSIS
DIAGNOSTIC REMARKS

Direct sputum • Primary diagnostic method in the Philippines because simple,

smear microscopy* economical, and provides definitive diagnosis of active TB
Xpert MTB/RIF* • PCR assay that detect M. tuberculosis and rifampicin resistance
TB culture* • Most specific
• Basic screening tool for children
• Indicates TB infection, not necessarily active disease
Tuberculin skin • Interpretation:
test (TST) / 0 Positive if ;,10 mm induration
purified protein
Positive if;,5 mm induration in the presence of any of the following:
.
0
derivative (PPD)
History of close contact with a known or suspected case of TB
test/
Mantoux test
. Clinical findings suggestive of TB
Chest X-ray findings suggestive of TB
. lmmunocompromised condition (e.g.,malnourished patient, HIV)
'The ideal specimen for the above tests is sputum for patients who can expectorate. For young children
who are unable to expectorate, gastric aspirates may be obtained as long as early morninggastric acid is
obtained before the childhas arisen and peristalsis has emptied the stomach of the pooled secretions.

233
IV. MANAGEMENT

Category I • 2-HRZE / 4-HR

Category la • 2-HRZE / 10-HR
Category II • 2-HRZES/ 1-HRZE / 5-HRE
Category Ila • 2-HRZES/1-HRZE/9-HRE

B. Classification of Tuberculosis based on Drug-Susceptibility Testing

Monoresistant TB • Resistance to 1 first-line anti-TB drug
• Resistance to > 1 first-line anti-TB drug (other than
Polydrug resistant TB
isoniazid and rifampicin)

Multidrug resistant TB • Resistance to at least both isoniazid and rifampicin

• Resistance to isoniazid and rifampicin + any

Extensively drug
fluoroquinolone + at least 1 of the 3 second-line
resistant TB
injectable drugs
• Resistance to rifampicin (whether monoresistant,
Rifampicin resistant TB
multidrug, polydrug, or extensive drug resistant)
Source:BehrMA,et at Revisitingthetimetableof tuberculosis.
BMJ;2018
KliegmanRM,et at NelsonTextbookof Pediatrics(20thed.).Elsevier;2016
DOH.NTCPManualof Procedures. (5thed.).Manila:Department of Health;2014
CantwellM, et al. NewEnglandJournalof Medicine;1994

LEPTOSPIROSIS

I. ETIOPATHOGENESIS
Etiology • Nine pathogenic species of spirochetes from the genus leptospira
• Zoonotic disease: infection mostly results from exposure to water or
soil contaminated with rat urine
Transmission
• Other animal reservoirs: dogs, cats, livestock, wild mammals
• Incubation period: 7-12 days
• Leptospires enter humans through abraded skin, mucous membranes,
or ingestion
Pathogenesis • Leptospires circulate in the bloodstream and cause damage to the
endothelial lining of small blood vessels, resulting in ischemic damage
to the liver, kidneys, meninges & muscles

234
II. MANIFESTATIONS
• Lasts 2-7 days
• Abrupt onset of fever, chills, severe headache, malaise, nausea
• Severe myalgia most prominent in the lower extremities,
Initial or lumbosacral spine and abdomen
septicemic
phase • Conjunctiva! suffusion with photophobia & orbital pain,
without chemosis & purulent exudate
Anicteric • Hepatosplenomegaly, generalized lymphadenopathy
Leptospirosis • Transient e1ythematous maculopapular rash
• Lasts several weeks
Second or • Follows a brief asymptomatic interlude with recurrence of
immune fever (biphasic fever)
phase • Aseptic meningitis: CNS symptoms usually resolve
spontaneously within 1 week
• Severe form affecting < 10% of cases
• Initial or septicemic phase: similar to anicteric leptospirosis
• Immune phase:
Icteric Leptospirosis • Jaundice, RUQ pain, hepatomegaly, increased liver
(Weil's syndrome) enzymes, hyperbilirubinemia
• Renal failure (principal cause of death)
• Thrombocytopenia

III. DIAGNOSIS
• Hemorrhage & cardiovascular collapse

• Considered in acute febrile illness with history of direct contact

I
Presumptive with animals, or contact with urine-contaminated soil or water,
Leptospirosis especially when there is abrupt onset of chills & fever, severe
myalgia, conjunctiva! suffusion, vomiting, headache

Microscopic • ;;,4-fold increase in antibody titers in paired sera obtained

agglutination test ;;elOdays apart

• Culture of leptospires from blood or CSF during the first 10 days of

illness and from urine after the second week of illness
Other tests • Enzyme-linked immunosorbent assay methods
• Latex agglutination,immunochromatography
• PCR

IV. MANAGEMENT
ASPECTS MANAGEMENT
• Penicillin G 6-8 million U/m2/day every 4 hours for 7 days**
Antibiotics* • Alternative: doxycycline (not for children <8 years old),
azithromycin
• Doxycycline 4mg/kg single dose (max 200mg): in children
Prophylaxis <8 years, dose and duration unlikely to cause dental staining
• Alternatives: Azithromycin OR Amoxicillin
• Rodent control measures
Prevention • Use protective clothing to avoid contaminated water and soil
• Immunization of livestock and domestic dogs

'Initiationof treatmentbeforethe 7th day may shorten the clinicalcourse and decrease the severityof infection
"Jarisch-Herxheimer reaction may develop after initiationof penicillintherapy
Sources:KliegmanRM,etal. NelsonTextbook
of Pediatrics
(21sted.).Elsevier;
2020
ChirathawomC,et al.AsianPacJ TropBiomed; 2014
Kimberlin
OW,et al.American
Academy of Pediatrics.
Redbook;2015
TheLeptospirosis
TaskForce(PSMID, PSN,PCP)Philippine CPG;2010
235
COMMON BACTERIAL SKIN INFECTIONS

ETIOLOGY MANIFESTATIONS MANAGEMENT

1) Impetigo (most common skin infection in children)

• Nonbullous impetigo (70%)
• Preceded by insect bite, • Topical muprocin or retapumilin
• Staphylococcus
abrasion, or laceration 2-3 times a day for 10-14 days
aureus
• Tiny vesicle or pustule, rapidly • Systemic therapy for lesions near
(predominant)
developing into honey-colored the mouth, or widespread or deep
• Group A beta-
crusted plaque involvement (e.g., cephalexin
hemolytic
25-50 mg/kg/day in 3-4 divided
streptococci • Bullous impetigo doses for 7-10 days)
(GABHS) • Flaccid, transparent bullae
developing on intact skin

2) Cellulitis
• Penicilinase·resistant penicillin
• Infection of loose connective tissue
[cloxacillin) or first generation
with limited dermal involvement
cephalosporin (cephalexin)
and sparing of the epidermis
• Parenteral therapy should be
• Staphylococcus • Area of edema, warmth, erythema,
initiated if (e.g., oxacillin):
aureus and tenderness with indistinct
• Disease progresses significantly
• Streptococcus margins
within 1-2 days of antibiotics
pyogenes • Cellulitis from S. aureus: more 0 No improvement noted on oral
localized
antibiotics
• Cellulitis from S. pyogenes: more
• With feve1; lymphadenopathy or
rapid and cause lymphangitis
constitutional signs

3) Orbital and Preorbital Cellulitis

• 5. aureus . Preseptal cellulitis • Cotrimoxazole or clindamycin,
• Streptococcus • Infection of soft tissues anterior plus
pyogenes to orbital septum • Amoxicillin or coamoxiclav
• Streptococcus c Warm, tendec erythematous
pneumoniae lid swelling, mucoid discharge,
• Haemophilus conjunctiva\ swelling
influenzae
• Orbital cellulitis • Va neomycin+ ceftriaxone or
• Infection poste1ior to orbital septum cefotaxime
• Proptosis, ophthalmoplegia,
change in visual acuity, ocular
pain, chemosis
• Wann, tende1; erythematous
lid swelling, mucoid discharge,
conjunctiva! swelling
4) Furuncle, Carbuncle, Abscess
• Furuncule: tender erythematous
nodule
• Bathing with antimicrobial
• Carbuncle: confluence of several
soaps ( chlorhexidine ), hot moist
adjacent areas of furuncles
compress to facilitate drainage,
producing a tender red tumor with
• Staphylococcus incision to drain large lesions
multiple drainage points
aureus • Systemic antibiotics based on
• Abscess: long-standing carbuncle
culture results for carbuncles,
that becomes soft and fluctuant
large or numerous furuncles 1
• Sites of predilection: hair-bearing
abscesses
areas on face, neck. axilla, buttocks
and groin

Source:KliegmanRM,et al. NelsonTextbook of Pediatrics

(21sted.).Elsevier;2020
GappyC, et al. OrbilalCellulitis.hllps:/lwww.uptodate.com/conlentslorbital-cellulitis;
2019
GappyC. el al. Preseptal
Cellulilis.https:/11w1w.uplodale.comlconlentslpreseptal-cellulitis;
2019

236
 OTHER BACTERIAL INFECTIONS

ETIOLOGY MANIFESTATION TRANSMISSION DIAGNOSTICS MANAGEMENT CONTROL MEASURES

1) Haemophilus lnfluenzae

• Amoxicillin PO for • Hib conjugate vaccine

• Humans are the
otitis media and • Rifamipicin for all household
only natural
• Nontypable H. influenzae: most • Gramstain uncomplicated sinusitis members, including the index
hosts
common cause of otitis media • Blood culture • IV antibiotics for patient, if the group includes
• May be part
• Meningitis • Serotyping is preseptal cellulitis, a child <48 months of age not
of normal
• Cellulitis done by slide orbital cellulitis, fully immunized
respiratory • Respiratory droplets
• Preseptal cellulitis agglutination epiglottitis, septic • Rifampicin (OD for 4 days with
flora in healthy
• Orbital cellulitis with type- arthritis max 600 mg/dose):
children
• Suppurative arthritis specific antisera • Meningitis: Cefotaxime, • 10 mg/kg/dose for
• Invasive disease
• Epiglotittis, sinusitis, pneumonia Ceftriaxone, or Ampicillin sl month old
in children
for 14 days for • 20 mg/kg/dose for
<5 years old
uncomplicated cases >1 month old
N
w
-.J
2) Nontyphoidal Salmonellosis (Salmonella enterica serotype Enteritidis, Salmonella enterica serotype Typhimurium)
• Treat dehydration
• Most common: acute enteritis (nausea,
• Antibiotics not
vomiting, feve1; crampy abdominal
recommended,
• Incidence peaks pain, watery or bloody diarrhea)
but may be given
at extremes • Bacteremia typically follows
• Food borne ( eggs are if <3 months old, • Control of infection in animal
of age gastroenteritis. Presents with fever,
the most commonly • Stool culture immunocompromised, reservoir
• Incubation chills and septic shock
implicated food) disseminated infection: • Avoid contaminated food
period: 6-72 • Extraintestinal focal infections:
• Cefotaxi me
hours osteomyelitis, meningitis, arthritis
• Ceftriaxone
• Most healthy children recover fully
• Ampicillin
• Prolonged carrier state is rare
• Cefixime

Source:KliegmanR, et al. NelsonTextbookof Pediatrics

(21sted.).Philadelphia;
2020

-
Kimberlin
OW,et al.AmericanAcademyof Pediatrics.Redbook.American Academyof Pediatrics;
2015
GomellaTL,et al. Neonalology 7thEdition.McGraw-Hill;
201
 ETIOLOGY MANIFESTATION TRANSMISSION DIAGNOSTICS MANAGEMENT CONTROL MEASURES
3) Shigellosis (Shigella dysenteriae)
• For bloody diarrhea,
• Bacillary dysentery: bloody diarrhea with feve1; • Presumptive regardless of age:
abdominal cramps, rectal pain and mucoicl stools
• Most common • Personto person diagnosis: fecal Ciprofloxacin 20-30
• Severe abdominal pain, cmesis, anorexia,
in 2-3 years transmission leukocytes, mg/kg/day in 2 • Proper handwashing
generalized toxicity, painful defecation (tenesmus)
old (fecal-oral route) fecal blood and divided doses techniques when preparing
• Abdominal distention & tenderness, hyperactive
• Incubation • Requires very low leukocytosis • Other antibiotics: food, using the toilet, or
bowel sounds, tender rectum on digital exam
period: 12 hrs inocula to cause • Definitive ° Cefixime changing diapers
• Neurologic manifestations (40%) are the most
to 7 days illness diagnosis: cultu,·e ° Ceftriaxone
common extraintestinal manifestation
0 Azithromycin
• Dehydration is the most common complication of stooljrectal swab
• Duration: 5 days

4) Cholera (Vibrio cholerae)

• Acute onset of profuse, painless, watery diarrhea • Fluid & electrolytes
• In endemic • Ingestion of water • Primarily clinical
with rice-water consistency and fishy odor • Antibiotics if with
N areas, highest contaminated with • Rapid test: • Chlorination or boiling of
w • Vomiting is usually present at the onset of illness severe dehydration
(X) incidence stools of patients darkfield drinking wate1; thoroughly
• Cholera gravis: most severe form, purging rates to shorten illness
among <2 or carriers, microscopy (wet cooking shellfish
of 500-1,000 mL/hour duration:
years old undercooked mount of stool • Oral cholera vaccines (not
• Complications • Tetracycline
• Incubation shellfish examined for available in the Philippines)
u Renal failure fro111
llehyclralion/hypotension • Erythromycin
period: 1-3 • Nephropathy, arrhythmias • Requires high "darting motility"
• Azithromycin
clays Seizures and coma from hypoglycemia inocula organisms)
0
• Doxycycline

5) Diphtheria (Corynebacterium diphtheria)

• Leather-like gray-brown adherent pseudomembrane • Antitoxin:single IVclose • Two follow-up cultures
that may extend beyond the foucialarea of equine antitoxin at should be obtained after
• Humans • Culture of
• May affect nasal, pharyngeal, laryngeal, • Direct contact 20,000-100,000 units completing antibiotic
are the sole specimens
cutaneous, ear, eye, genital tract areas via respiratory • Antibiotics: treatment to ensure detection
reservoir obtained from the
• Complications include: droplets or • E1ythromycin of relapse
• Incubation nose or throat or
• Toxic cardiomyopathy: occurs in 10-25% of discharges from PO/IV • Vaccine should be given
period: 2-4 any mucosa! or
patients during the 2nd-3rd week of illness skin lesions • Penicillin G IV during convalescence because
clays Toxic ncuropathy: occurs 2-3 weeks after skin lesion
0
• Penicillin G disease does not necessarily
onset; symmetric polyncuropathy procaine IM confer immunity
 SECTION FIVE
FUNGAL AND PARASITIC INFECTIONS

COMMON FUNGAL AND PARASITIC SKIN INFECTIONS

ETIOLOGY MANIFESTATIONS MANAGEMENT
1) Candidiasis
Oral candidiasis
• Usually among infants or in older
• Oral nystatin four times a
children treated with antibiotics
day for 5 days
• White plaques on a red base in buccal
mucosa, tongue or palate

Diaper dermatitis
• Most common infection caused by
Candida
Candida
albicans
• Confluent erythematous rash with • Topical nystatin
satellite pustules • Miconazole
Vu!vovaginitis • Clotrimazole
• Pubertal & postpube1tal females

2) Tinea Versicolor
• Pain or itching, dysuria, vulvar or
vaginal erythema, opaque white or
cheesy exudate I
• Reddish brown macules (in fair-
• Topical selenium sulfide
skined children) or hypopigmented/
2.5% shampoo 10 minutes
hyperpigmented macules (in dark-
• Malassezia daily for 2 weeks, then once a
skinned children)
globose week (for several months) to
• Macules are covered with fine scales,
• Malassezia prevent relapse
beginning in a perifollicular location
sympodialis • Other topical agents:
and merging to form patches, with
• Malassezia ketoconazole shampoo,
little to no pruritus
restricta terbinafine, clotrimazole
• Most commonly found on neck, upper
• Malassezia • For severe/recurrent
chest, back & upper arms
furfur disease or if topical therapy
• Potassium hydroxide [KOH) scrapings
failed: oral ketoconazole,
show short curved hyphae & circular
fluconazole, or itraconazole
spores ("spaghetti & meatballs")

3)Scabies

• Intense pruritus particularly at night • Pennethrin 5% cream

• First noticeable sign: 1-2 mm red applied to the entire body
papules, with excoriation, crusting (neck down) for 8-12 hours.
• Sarcoptes
or scaling Reapply after 1 week for
scabiei
• Classic lesion: threadlike burrows another 8-12 hours. All
(burrowing
• Frequent on palms, soles & scalp household members should
into stratum
• For older children, preferred sites be treated at the same time.
comeum &
are interdigital spaces, wrist flexors, • Alternative: lindane lotion
release of
anterior axillary fold, ankles, or cream, sulfur ointment,
antigenic
buttocks, umbilicus and belt line, crotamiton lotion or cream
substances by groin, and areola • Itching may persist for 7-10
female mite) • Clinical clue: highly contagious; ask days after successful therapy
if other household members have and may be alleviated by
similar lesions topical cortocosteroids
Sources:KliegmanRM,et al. NelsonTextbook of Pediatrics
(21sted.).Elsevier;2020
KimbertinOW,et al.AmencanAcademyof Pediatncs; 2015
Redbook:2015reportof theCommittee
on InfectiousDiseases(30thed.).ElkGroveVillage.IL:AmericanAcademyof Pediatncs.

239
 SECTION SIX
CLINICAL SYNDROMES

HEAD AND NECK INFECTIONS

INFECTION ETIOLOGY EMPIRIC ANTIMICROBIAL
1) Conjunctivitis
• Ophthalmic antibiotic drops
Bacterial • S. pneumoniae, Moraxella, HiB or ointment (quinolones,
erythromycin, aminoglycosides)

Gonococcal • N. menin9itidis • Ceftriaxone

• C. trachomatis, Streptococcus., • Tetracycline or Erythromycin

Neonatal Hemophi/lus, P.aeru9inosa, or Sulfonamide ophthalmic
Moraxella, N. menin9itidis, E. coli ointment

Z) Eyelids
Blepharitis • S. aureus, S. epidermidis • Etythromycin or Bacitracin ointment
Hordeolum • S. aureus • Warm compress

• S. aureus, Streptococcus, Klebsiella, • Ceftriaxone or Cefotaxime, plus

3) Orbital cel/ulitis
Pseudomonas, Enterococcus • Metronidazole +/- Clindamycin
4) Ear Infections (Acute)
• S. aureus, P.aeru9inosa, P.
Otitis Externa • Quinolone otic drops
mirabilis, E. coli
• Amoxicillin or Cefuroxime
Otitis Media • H. influenzae, S. pneumoniae
• Co-amoxiclav or Ceftriaxone

INFECTIONS OF THE SKIN AND SOFT TISSUES

1) Cellulitis

• Clindamycin, plus
Neonates • S. aureus, GroupB streptococci,HiB
• Cefotaxime or ceftriaxone

• S. aureus, Group A Beta hemolytic

Others • Clindamycin
streptococcus
• Pasteurella multocida, S. aureus, • Amoxicillin-clavulanate or
Z) Dog/ Cat Bite
Streptococcus, anaerobes Ampicillin-sulbactam
• Streptobacillus moni/liformis,
3) Rat Bite • Amoxicillin-clavulanate
S. aureus

• Streptococci, Eikenella corrodens, • Amoxicillin-clavulanate or

4) Human Bite
S. viridans, S. aureus Ampicillin-sulbactam

5) Ecthyma • Ceftazidime, plus

Gangrenosum • P.aeruginosa
• Aminoglycoside
6) Erysipelas • Group A Beta hemolytic strep • Penicillin
7) Carbuncles,
Fol/iculitis, • S. aureus • Cloxacillin
Furunculosis
• Group A Beta hemolytic strep,
8) Impetigo • Cloxacillin
S. aureus

9) Necrotizing • S. pyo9enes, S. aureus, • Penicillin or Clindamycin, plus

Fasciitis Gram negative, bacilli • Aminoglycoside

240
INFECTIONS OF THE PULMONARY SYSTEM
I. UPPER RESPIRATORY TRACT INFECTIONS
INFECTION ETIOLOGY EMPIRIC ANTIMICROBIAL
• S. a11re11s,
Streptococcus, Gram (-) • Oxacillin or Clindamycin, plus
1) Parotitis
organisms, anaerobes aminoglycoside

• H. influenzae, S. pyogenes, S.
2) Epigfottitis • Ceftriaxone or Cefotaxime
aureus, S. pneumoniae

3) Tonsillopharyngitis • Group A,C,GStreptococci • Penicillin V

(Ex11dative)
4) To11sillophary11gitis • Benzyl penicillin G
(Membranous) • C. diphtheriae
• Give antitoxin
5) Peritonsillar,
Retropharyngeal, • Group A Streptococci, MSSA,
or Parapharyngea/ • Clindamycin
anaerobes
abscess
• H. influenzae, S. pneumoniae, M.
6) Sinusitis • Amoxicillin
catarrha/is, S. aureus, S. pyogenes

II LOWER RESPIRATORYTRACT INFECTIONS

1) Pneumonia

Neonate
• Group B streptococcus,
Gram-negative bacilli

• S. p11e11mo11iae,
H. i11f/11e11zae,
• Penicillin G or Ampicillin, plus
• Aminoglycoside
• Mild illness: Amoxicillin PO
• Moderate or serious illness: 2 nd
I
Infant to 5 yrs old
S. aureus or 3"1 generation cephalosporins
or Ampicillin-sulbactam

>5 years old to • M. pneumonioe, C. pneumoniae, • Erythromycin or Azithromycin

Adolescent S. pneumoniae or Clarithromycin
• Mild illness: Cefazolin,Cefuroxime
• Moderate illness: Clindamycin +
2)Empyema • S. aureus,S. pneumoniae,S. pyogenes (Cefuroxime or Ceftriaxone)
• Severe illness: Vancomycin +
(Cefotaxime or Ceftriaxone)

3) Aspiration • S. aureus, S. pneumoniae,

• Clindamycin or Ampicillin-
Pneumonia or anaerobes of the upper
lung Abscess sulbactam
respiratory tract

INFECTIONS OF THE CARDIOVASCULAR SYSTEM

1) Infective Endocarditis (IE)
Acute IE • S.aureus,S.pyogenes,S. pnewnoniae • Oxacillin +/- Gentamicin
• Viridans streptococci,
Subacute IE • Penicillin G + Gentamicin
Enterococci, Gram(-) organisms
2) Bacterial • S. aureus, S. pneumoniae, HiB, N. • Oxacillin, plus
Pericarditis meningitidis • Cefotaxime or Ceftriaxone
3) Rheumatic Fever • Grp A Beta hemolytic Streptococci • Benzathine Penicillin

INFECTIONS OF THE URINARY TRACT

1) Cystitis or • Enteobacteriaceae,S.saprophyticus, • Cotrimoxazole or Co-Amoxiclav
Pyelonephritis Enterococci • Cefuroxime or Cefixime

• S. aureus, Streptococcus,Gram • Cefotaxime or Ceftriaxone or

2) Renal Abscess
negative baci/t,:enterococci,
anaerobes Ceftazidime, plus Aminoglycoside

241
GENITOURINARY TRACT
INFECTION ETIOLOGY EMPIRIC ANTIMICROBIAL
1) Bacterial Vaginosis • Gardnerella,anaerobes • Metronidazole PO or vaginal gel

• S. aureus, E. coli, Chlamydia, • Ceftriaxone IV

2) Epididymitis
N.gonorrhea • Add Doxycycline for >8 years old

3) Gonococcal Infections
• Children <45 kg: Ceftriaxone IM
Uncomplicated
cervicitis, urethritis, • Adolescents: Ceftriaxone or Cefixime
vaginitis
• N. gonorrhea + regimen effective for Chlamydia

Bacteremia, • Ceftriaxone IV+ regimen effective

Arthritis, Meningitis for Chlamydia

4) Granulomatous / Ulcerative lesions

Chancroid • H. ducreyi • Azithromycin or Ceftriaxone IM

Granuloma • Klebsiellagranulomatis • Doxycycline

inguinale

Lymphogranuloma • Erythromycin or Doxycycline

• C. trachomatis
venereum (for >8 years old)

5) Pelvic Inflammatory • C. trachomatis, N. • Cefoxitin, plus

Disease gonorrhea, anaerobes • Doxycycline

• Gram negative enteric • 3"' gen cephalosporin, plus

6) Prostatitis
organisms • Aminoglycoside

7) Syphilis
Congenital • Aqueous Penicillin G

Primary, Secondary, • Benzathine Penicillin G IM x ldose

Early Latent
• T pallidum
Late Latent or • Benzathine Penicillin G IM
Unknown duration weekly x 3 doses

Neurosyphilis • Crystalline Penicillin G IV

• S.saprophyticus,Streptacoccus,
• Azithromycin or Doxycycline
Enterobacteriaceae,G.vaginalis
8) Non-Gonococcal • <8 years old: Erythromycin,
Urethritis Azithromycin
• Chlamydia trachomatis
• >8 years old: Azithromycin or
Doxycycline

MUSCULOSKELETAL INFECTIONS
INFECTION ETIOLOGY EMPIRIC ANTIMICROBIAL
1) Osteomyelitis
• S. aureus,gram negativebacilli, • Oxacillin, plus
Infants <4 months
Group B streptococcus • Ceftazidime

>4 months & above • S. aureus, Group A streptococws • Oxacillin

Puncture wound • P.aeruginosa, S.aureus, • Oxacillin, plus

osteomyelitis Streptococcus, anaerobes • Amikacin or Ceftazidime

• S. aureus, H. influenzae, • Infants & young children: Cefuroxime

2) Septic Arthritis
Streptococcus • Children > 5 years: Oxacillin

242
GASTROINTESTINAL TRACT

INFECTION ETIOLOGY EMPIRIC ANTIMICROBIAL

1) Gastroenteritis
Neonatal • E. coli, Salmonella • Ampicillin + Aminoglycoside
Bloody diarrhea • Shigella • Ciprofloxacin

• Giardia lamblia, E. histolytica,

Diarrhea >14 days • Treat specific infection
Shigella, EAEC

Severe diarrhea • V.cholera • Tetracycline

Traveler's • Enterotoxigenic E. coli, Shigella, C.

• Azithromycin
diarrhea jejuni, Salmonella spp.

• Ampicillin, plus
2}Acute • Gram negative enteric aero bes • Metronidazole or Clindamycin,
Appendicitis and anaerobes plus
• Aminoglycoside
• 3 rd generation cephalosporin
3) Acute Cholangitis • E. coli, Klebsiel/a, Enterococcus, (except Ceftriaxone), plus
Cholecystitis Bacteroides • Metronidazole, plus

4) Peritonitis

Primary
• S. pneumoniae, S.pyogenes,
S. aureus, E. coli
• Aminoglycoside

• Penicillin G
I
• Ampicillin, plus
• Enteric gram negative aerobes,
Secondary • Metronidazole or Clindamycin,
anaerobes (8. fragilis)
plus Aminoglycoside
• S. aureus, Streptococcus, Gram • Clindamycin or
SJ Pyogenic Liver negative aerobic and anaerobic Oxacillin+Metronidazole, plus
Abscess
organisms • Aminoglycoside

CENTRAL NERVOUS SYSTEM

INFECTION ETIOLOGY EMPIRIC ANTIMICROBIAL

1) Brain Abscess/ Subdural Empyema
• Viridans streptococci, anaerobic • Penicillin OR Cefotaxime Or
From Sinusitis/ Streptococci, Hemophilus, Ceftriaxone, Plus
Dental infection
Fusobacterium, Bacteroides • Metronidazole
• Oxacillin or Vancomycin, Plus
From Endocarditis • S. aureus, Viridans streptococci • Cefotaxime or Ceftriaxone, Plus
• Metronidazole

From Congenital • Viridans streptococcus, • Cefotaxime or Ceftriaxone, Plus

Heart Disease Hemophilis • Metronidazole

• H. influenza, S.pneumoniae • Ampicillin or Chloramphenicol

2) Meningitis
• N. memingitidis • Ceftriaxone or Cefotaxime

Souce:Handbook
of PediatricInfectious
Diseases,2014
NationalAntibioticGuidelines
2017

243
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1. American Academy of Ped1atncs Kimberlin OW, Brady MT, Jackson MA, Long SS, eds Red
Book: 2015 Report of the Committee on Infectious Diseases 30th ed Elk G1ove Village, IL·
American Academy of Pediatrics; 2015.
2. American Academy of Pediatrics. Kimberlin OW, Brady MT, Jackson MA, Long SS, eds.
RedBook: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca ,IL: American
Academy of Pediatrics 2018
3. Behr MA, et al. Revisiting the timetable of tuberculosis. BM) 2018;362:k2738.
4. Cantwell, M., Shehab, Z., Costello, A., Sands, L., Green, W., & Ewing, E. et al. (1994). Congenital
Tuberculosis. New England Journal Of Medicine, 330(15), 1051-1054
5. Centers for Disease Control and P1·evention. Meningococcal Disease. https://www.cdc.gov/
vaccines/pubs/pinkbook/mening.html. Accessed February 25, 2019.
6. Centers for Disease Control and Prevention. Recommended Antimicrobial Agents for the
Treatment and Postexposure Prophylaxis of Pertussis: 2005 CDC Guidelines. MMWR,
54(RR14), 1-16.
7. Centers for Disease Control and Prevention. Salmonella. https://www.cdc.gov/salmonella/
general/technical.ht111l. Accessed February 25, 2019.
8. Centers for Disease Control and Prevention. Tetanus. https://www.cdc.gov/tetanus/
clinicians.ht111l. Accessed February 25, 2019.
9. Department of Health. National Tuberculosis Control Program Manual of Procedures. (5th
ed.). Manila: Depart111ent of Health; 2014
10.Edwards, M. Clinical Features, Evaluation, Diagnosis of Sepsis in Term and Late Preterm
Infants. UpToDate20l 9.http://www.uptpdate.com. Accessed March 21, 2019
11.Edwards, M. Management and Outcome of Sepsis in Term and Late Preterm Infants.
UpToDate20l 9.http://www.uptodate.com Accessed March 21, 2019
12.French P. Syphilis. BM) 2007 334(7585), 143-147
13.Gappy C, et al. Orbital Cellulitis. https://www.uptodate.com/contents/orbital-cellulitis; 2019
14.Gappy C, et al. P1-eseptal Cellulitis. https://www.uptodate.com/contents/preseptal-
cellulitis; 2019
15.Gomella TL, et al. Neonatolgy 7th Edition. McGraw-Hill; 2013.
16.Harrison LH, Kreiner CJ, Shutt KA, Messonnier NE, O'Leary M, Stefonek KR, et al. (2008).
Risk factors for meningococcal disease in students in grades 9-12. Pediatr Infect Dis ],
27(3), 193-199.
I ?.Kimberlin DW, Brady Ml'. Jackson MA, Long SS. (Eds.) (2015). American Academy of
Pediatrics. Red book: 2015 report of the Committee on Infectious Diseases (30th ed.). Elk
Grove Village, IL: American Academy of Pediatrics.
18.Kliegman, R., ST GEME 111,)., Blum, N., Shah, S., Tasker, R., Wilson, K., & Behrman, R. (2020).
Nelson Textbook of Pediatrics (21st ed.). Philadelphia: Elsevie1c
I 9.Mandell GL, Bennett, JE and Dolin R. editors: Mandell, Douglas and Bennett's Principles
and Practice of Infectious Diseases, ed 7, Philadelphia, 2010,Elsevier
20.Natalie, N; Duchon, J; and Zachai-iah, P. Clinics in Perinatology, 2015-03-01, Volume 42,
Issue 1, Pages 77-103. 2015 Elsevier
21.National Antibiotic Guidelines Committee. National Antibiotic Guidelines 2017.
Department of Health Quezon City Philippines. 2017. Available from <https://drive.google.
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22.National Dengue Prevention and Control Program. Revised Dengue Clinical Case
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23.Neu J. Necrotizing enterocolitis: the search for a unifying pathogenic theory leading to
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24.Patterson Mj and Davies JD. Syphilis (Treponema pallidum). In: Kliegman RM, Stanton BF,
St Geme JW, Schor NF. (Eds.) (2016} Nelson Textbook of Pediatrics (20th ed.). Philadelphia,
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Philippine Pediatric Society Inc. 2014
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the Diagnosis, Management and Prevention of Leptospirosis 2010
27.Veeraraghavan B, Pragasam AK, Bakthavatchalam TD, and Ralph R. (2018). Typhoid fever:
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dengue and dengue haemorrhagic fever. Revised and expanded edition .. New Delhi: World
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31.Yip WCL. Dengue haemorrhagic fever: current approaches to management. Medical
Progress; 1980; 7(13):201-209

244
GASTROENTERO
 SECTION
APPROACH TO COMMON GASTROINESTINAL
MANIFESTATIONS OF DISEASES IN THE GASTROINTESTINAL TRACT
MANIFESTATIONS DEFINITION
• Difficulty in swallowing
• Odynophagia: painful
swallowing
• Globus: sensation of something
stuck in the throat
Dysphagia • Oropharyngeal dysphagia:
transfer of food from mouth to
esophagus is impaired
• Esophageal dysphagia:
difficulty in transport of food
bolus down the esophagus
• Effortless movement of
stomach contents into the
esophagus and mouth
Regurgitation • It may be a developmental
process (physiologic) that
improves/resolves with
maturity
Anorexia • Prolonged lack of appetite
• Highly coordinated reflex
process that may be preceded
by increased salivation and
begins with involuntary
Vomiting
retching
• Bilious vomiting suggests
obstruction below the second
part of the duodenum
• Excessive loss of fluid and
electrolytes in stool
• Loss is >10 mL/kg/day in
Diarrhea infants and >200 g/24hr in
older children that lasts <14
days (if more than 14 days:
persistent/chronic diarrhea)
247
ONE
COMPLAINTS
DIFFERENTIAL DIAGNOSIS
• Acute viral stomatitis
• Oral trauma
• Nasal obstruction
• Achalasia
• Tracheoesophageal fistula
• Strictures
• Vascular rings
• Mediastinal tumors
• Vertebral anomalies
• Esophageal web
• Gastroesophageal reflux
disease (GERO)
• Gastroesophageal reflux
(GER)
• GERO
• Systemic diseases (e.g.,
I
neoplastic and inflammatory
diseases)
• Iatrogenic: drug, unpalatable
diets
• Depression
• Gastroenteritis
• GERO
• Overfeeding
• Gl obstruction
• Infections
• Others: inborn errors of
metabolism, medications/
toxins, increased intracranial
pressure (ICP), labyrinthitis,
adrenal insufficiency, migraine
• Lactose intolerance
• Malabsorption
• Acute gastroenteritis
• Toxins
• Tumors (neuroblastoma)
• Adrenal insufficiency
• Motility disorders

• Definition is relative and
depends on stool consistency
and frequency as well as
Constipation difficulty in passing the stool
• Hard stool passed with
difficulty every 3rd day is
considered constipation
• Considerable variation
among children in their
perception and tolerance for
abdominal pain
• Distinguish organic* and
nonorganic (functional)
causes of abdominal pain
• Visceral pain: dull and
Abdominal pain aching pain felt in the
dennatome from which the
organ receives innervation
• Somatic pain: intense
and well localized (e.g.
peritonitis)
• Referred pain: pain from
other organs
• Hematemesis: originates
from esophagus, stomach, or
duodenum
• Hematochezia: red blood in
the stools; distal bleeding
Gastrointestinal site or massive bleeding
hemorrhage above the distal ileum
• Melena: blackened stools of
tarry consistency; from mild
to moderate bleeding above
the distal ileum
I • Colonic polyps
• Distention results from
diminished tone of the wall
Abdominal musculature or increased
distention abdominal content
• If with as cites, fluid analysis
aids in determining etiology
'Consider organicetiologyof abdominalpain ifwithfever,weightloss, biliousemesis,jaundice,hepatosplenomega-
ly,back or flankpain, awakenschildfromsleep, referredpain to shoulder,groinor back, elevatedESR,WBCcount,
CRP,anemia,edema, hematochezia,or withstrong historyof IBD
Source:KliegmanR.et al. NelsonTextbook of Pediatrics(21sted.).Canada:Elsevier:2020
WyllieR.et al. PediatricGastrointestinal
andLiverDisease(3rded.).Philadelphia:
248
• In neonates: Hirschsprung
disease, intestinal
pseudoobstruction,
hypothyroidism
• Fecal impaction
• Functional constipation
• Spina bifida
• Imperforate anus
• Developmental delay
• Dehydration
• Medications: narcotics
• Lead poisoning
• Botulism
• Functional abdominal pain
• Irritable bowel syndrome
• Constipation
• Lactose intolerance
• Esophagitis
• Intestinal parasitism
• Inflammatory bowel disease
(IBD)
• Cholelithiasis
• Choledochal cyst
• Pancreatitis
• Appendicitis
• Inguinal or abdominal hernia
• Intestinal obstruction
• Non-GI causes (e.g., renal colic,
pneumonia, pelvic inflammatory
disease, sexual abuse,
psycho logic causes, HSP,
anaphylaxis)
• Erosive damage
(most common cause)
• Bacterial enteritis
• Milk protein allergy
• lntussusception
• Anal fissure
• Lymphonodular hyperplasia
• Peptic ulcer/gastritis
• Swallowed epistaxis
• Prolapse gastropathy
• Mallory-Weiss syndrome
• Ascites
• Solid masses (Wilms
tumor, hydronephrosis,
neuroblastoma,
hepatoblastoma, lymphoma,
teratoma, mesenteric cyst)
• Pregnancy
Elsevier:2006
 SECTION TWO
GASTROINTESTINAL DISORDERS OF THE NEONATE

ESOPHAGEAL ATRESIA (EA) & TRACHEOESOPHAGEAL FISTULA (TEF)

• Esophageal atresia: esophagus ends in a blind pouch rather than connecting normally to stomach
• Tracheoesophageal fistula: abnormal connection (fistula) between esophagus & trachea

I.ETIOPATHOGENESIS
TYPES OF TEF ILLUSTRATION DESCRIPTION

• Isolated EA
Type A (8%) • Pure esophageal atresia without
a TEF

Type B (<1%) • EA with proximal TEF

I
• EA with distal TEF
• Most common type
• Esophagus ends blindly 10-20 cm
Type C (87%)
from the nares
• The distal esophagus communicates
with the posterior trachea

Type D (<1 %) • EA with double TEF

Type E (4%) • Isolated TEF

II. MANIFESTATIONS
• Infant is unable to handle secretions with subsequent excess salivation and aspiration of
pharyngeal contents due to the esophageal obstruction, requires frequent suctioning
• Feeding usually exacerbates the symptoms, causes regurgitation & precipitates aspiration

249
III. DIAGNOSIS
• Inability to pass an NGT or OGT in the newborn is suggestive especially in the setting of
early-onset respiratory distress
• Plain radiographic films may show coiled NGT/OGT tube in the esophageal pouch
• 50% have associated anomalies (VATER/VACTERL):
Vertebral • Esophagus
• Anorectal Renal
• Cardiac Radial
• Trachea • Limb syndrome

IV. MANAGEMENT
• Maintain a patent airway
• Pre-operative proximal pouch decompression to prevent aspiration of secretions
Supportive
• Antibiotic therapy for consequent pneumonia
• Prone positioning to reduce regurgitation & esophageal suctioning
• Current standard surgical approach: surgical ligation of the TEF & primary
Surgery
end-to-end anastomosis of the esophagus via right-sided thoracotomy
Sources:DunkleyM, et al. InternationalSurgery:2014
ClohertyJ, et al. Manualof NeonatalCare(7thed.).LippincottWilliams& Wilkins:2012
GomellaTl, et al. Neonatology
7th Edition.McGraw-Hill:2013

HYPERTROPHIC PYLORIC STENOSIS

• Gradual hypertrophy of the pyloric musculature which eventually leads to gastric outlet
obstruction if untreated
• Most common cause of nonbilious vomiting in infants
I. ETIOPATHOGENESIS
• Most common in healthy term neonates and infants (3 weeks to 5 months)
• Unknown cause but abnormal muscle innervation is implicated

II. MANIFESTATIONS
• Postprandial, nonbilious vomiting is the initial symptom that usually starts
at 3 weeks of age
Symptoms
• Non-bloody, nonbilious emesis increasing over the course despite small
frequent feedings
• Firm, movable, olive-shaped mass, easily palpable after vomiting
Signs
• Visible gastric peristaltic wave after feeding may be seen

III. DIAGNOSIS
REMARKS/FINDINGS
• Persistent muscle thickness in the presence of functional gastric outlet
obstruction
Ultrasound • Confirmatory findings:
• Pyloric thickness 3-4 mm
• Overall pyloric length 15-19 mm
• Pyloric diameter 10-14 mm

Barium • String sign: elongated pyloric channel

swallow • Shoulder sign: bulge of the pyloric muscle into the antrum
test • Double tract-sign: streaks of barium in the narrowed channel
Laboratory • Hypochloremic metabolic alkalosis because of vomiting
findings • Hyperbilirubinemia: most common association (icteropyloric syndrome)

IV. MANAGEMENT
• Correcting fluid, acid-base, and electrolyte losses
• Surgical procedure: Ramstedt pyloromyotomy
Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Canada:Elsevier:2020
GomellaTL, et al. Neonatolgy7th Edition.McGraw-Hill:
2013
250
HIRSCHSPRUNG DISEASE (HD)
• Congenital aganglionic megacolon causing loss of ability of the muscles in the bowels to
move stool through the intestine
• Most common cause of lower intestinal obstruction in neonates
I. ETIOPATHOGENESIS
• More common in males
• Due to arrest of neuroblast migration from the proximal to distal bowel, leading to
absence of ganglion cells in the bowel wall beginning in the internal anal sphincter
• Absence of Meissner & Auerbach plexus and hypertrophied bundles with high
concentrations of acetylcholinesterase between the muscular & sub mucosa layers leads to:
0 Decreased motility in the affected bowel segment
0 Lack of propagation of peristaltic waves into the aganglionic colon
0 Abnormal or absent relaxation of this segment and of the internal anal sphincter

II. MANIFESTATIONS
• Distended abdomen, failure to pass meconium, bilious emesis or
aspirates, feeding intolerance
Symptoms • Suspected in any full-term infant with delayed passage of stool (no
meconium output> 48 hours of life)
• Some present with history of chronic constipation
• Tympanitic and distended abdomen
Signs • Large fecal mass palpable in the left lower quadrant
• Pellet-like or ribbon-like stool or with fluid consistency

I
Rectal • Rectum usually empty of feces on exam with normal anal tone
Examination • May have an explosive discharge ("gush of air") once finger is removed

Ill. DIAGNOSIS
DIAGNOSTIC REMARKS/FINDINGS
Rectal suction
biopsy • Gold standard (easy and reliable J
• Transition zone between normal dilated proximal colon and the
Abdominal smaller-caliber obstructed distal colon due to nonrelaxation of the
X-ray with
aganglionic bowel
contrast
enema • A rectal diameter that is the same as or smaller than the sigmoid colon
is suggestive

Anorectal • Evaluates internal anal sphincter while a balloon is distended in the

manometry rectum
(difficult to • Normal response to rectal distention is relaxation of the internal anal
perform in sphincter
infants) • Internal sphincter fails to relax in response to rectal distention

IV. MANAGEMENT
• Operative repair with surgical options/ techniques: Swenson, Duhamel, Soave
• Currently, many infants undergo primary pull-through procedure unless there is
associated enterocolitis or other complications

251
OTHER DISORDERS ENCOUNTERED IN THE NEONATE
I. DUODENALATRESIA
• Due to failure to recanalize the lumen during the 4th-5th week of gestation
Etiopathogenesis • Obstruction usually distal to the ampulla of Vater
• More common in preterm infants

• Hallmark: bilious vomiting (without abdominal distention) usually

noted on the 1stday oflife
Clinical • Prenatal examination: polyhydramnios in 50% due to failure of
Presentation absorption of amniotic fluid in the distal intestine
• Associated with Down syndrome, esophageal atresia and
imperforate anus
• Abdominal radiograph: "double bubble sign" pathognomonic of
Diagnosis duodenal obstruction where two lucencies are seen in the stomach
and the other in the first portion of the duodenum
• Gastric decompression (NGT,OGT) to control vomiting and allow
Management "elective' surgery correction
• Surgical repair: duodenoduodenostomy with gastrostomy tube

II. DISTAL INTESTINAL OBSTRUCTION

• Denotes partial or complete obstruction of the distal GI tract: small
Etiopathogenesis
bowel or colon, functional or physical obstruction
• Distended abdomen, failure to pass meconium, and bilious emesis
Clinical • Differentials include jejunalfileal atresia, meconium ileus, colonic
Presentation atresia, meconium plug syndrome, hypoplastic left colon syndrome,
and Hirschsprung disease
• Abdominal radiographs: multiple dilated loops of intestine
• Contrast radiologic studies: contrast enema to identify colonic
atresia, microcolon due to complete distal small bowel obstruction,
Diagnosis
transition zone of Hirschsprung disease, identify and treat
meconium plug
• Mucosa! rectal biopsy for histologic detection of ganglion cells
• Promotion of return of peristaltic by digital examination (colonic
stimulation) and by rectal enemas
Management • Rectal mucosa biopsy
• Repeated enema to loosen inspissated meconium
• Urgent surgical intervention for atresias, complicated meconium ileus

III. IMPERFORATEANUS
Etiopathogenesis • Absence ofan anal opening of proper location and size

• Delayed passage of meconium and intestinal obstruction

• High imperforate anus: rectum ends above the puborectalis sling
Clinical
(this muscle maintains fecal continence)
Presentation
• Low imperforate anus: rectum has traversed the puborectalis sling
in correct position
• Through perinea I inspection and calibration of any opening that drains
meconium (fifth digit probe, rectal thermomete1; soft feeding tube)
Diagnosis
• Radiographic studies of lumbosacral spine, urinary tract
• Spinal ultrasound and MRI to evaluate tethered cord
• Colostomy for high anomalies
Management
• Perinea! anoplasty or fistula dilatation for low anomalies
Source:GomellaTL.et al. Neonatology 71hEdition.McGraw-Hill:
2013
R.et al. NelsonTextbook
Kliegman of Pediatrics
(21s1ed.).Philadelphia:
2020
252
IV. OMPHALOCELEAND GASTROSCHISIS
OMPHALOCELE GASTROSCHISIS
• Failure of gut migration from yolk
sac to abdominal cavity • Occlusion ofomphalomesenteric
Pathophysiology • Amnion covers omphalocele and arte1y resulting in ischemia and
protects this from infection and atrophy of abdominal wall layers
fluid loss
• Average maternal age • Young maternal age (<20 years)
Epidemiology
• Male > Female • Male = Female
• Midline abdominal wall defect • Abdominal wall defect lateral to
Location • Herniation into base of the umbilical cord
umbilical cord (more commonly to the right)
Umbilical Cord • Center of the membrane • Left of the defect
Covering
membrane/ • Yes • No
Presence of sac
• Inflamed
Bowel
• Normal • Matted, foreshortened,
appearance
edematous, exudative
• 60%
• Beckwith-Wiedemann • 10%
Associated syndrome • Malrotation
Anomalies

Alimentation
• High association with
congenital anomalies/
chromosomal abnormalities
• Normal
• Volvulus
• Atresia

• Delayed
I
Surgical • Not urgent with intact
management • Urgent
membrane
• Total parenteral nutrition
• Total parenteral nutrition
• Hydration
• Hydration
• Ruptured omphalocele: similar
• Prevent evap.orative heat loss by
management to gastroschisis
Other Supportive decompression and wrapping
• Intact sac: timing of repair
Ma11agement with moist clean dressing
depends on size of defect,
• Nasogastric decompression
gestational age and co-morbid
• Broad spectrum antibiotics as
conditions
indicated
• P:ntibiotics as indicated

Sources:LedbetterDJ.SurgClin NorthAm: 2012

WyllieR, et al. PediatricGastrointestinal
andLiverDisease.Philadelphia:
2016
Christison-Lagay ER,et al. SeminFetalNeonatalMed:2011
KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Canada:Elsevier:2020

253
 SECTION THREE
DIARRHEA AND GASTROENTERITIS

ACUTE GASTROENTERITIS
I.ETJOPATHOGENESIS
• Acute diarrhea (less than 14 days) must be differentiated from chronic or persistent
diarrhea (more than 14 days duration)
• Majority are foodborne illnesses
• Viruses are one of the most common causes of acute diarrhea in children
A. Etiology of Acute Diarrhea
• C.jejuni, C. difficile, EIEC (Enteroinvasive £. coli), Salmonella,
Inflammatory
Shigella, Yersinia
• EPEC (Enteropathogenic £. co/1), ETEC (Enterotoxigenic E. coh),
Noninflammatory
V.cholera
Viral • Rotavirus, Adenovirus, Astrovirus, Norwalk, Calicivirus
Parasitic • G.lomblia, E. histolytica, B. coli, Strongyloides, spore-forming protozoa

• Malabsorption, endocrinopathies, food poisoning, neoplasms

Other causes • Miscellaneous (milk allergy, immunodeficiency states, laxative
abuse, ulcerative colitis, motility disorders)

8. Two Types of Acute Infectious Diarrhea

TYPE PATHOPHYSIOLOGY
Inflammatory • Due to bacteria that invade intestine directly or produce cytotoxins
• Enterotoxin production by bacteria
Noninflammatory • Destruction of villus by viruses
• Adherence by parasites

C. Mechanisms of Diarrhea
OSMOTIC DIARRHEA
• Presence of nonabsorbable
solutes in the GITas a result of:
• Intestinal damage
• Reduced absorptive surface
area (e.g., active celiac disease)
Pathogenesis
• Defective digestive enzyme (e.g.,
lactose intolerance)
0 Decreased intestinal transit time
• Nutrient overload (e.g.,
overfeeding.sorbitol in fruit juice)
SECRETORY DIARRHEA
• Activation of intracellular
mediators (e.g., cAMP,
cGMP) that stimulate active
chloride secretion & inhibit
the neutral coupled NaCl
absorption
• Toxin-mediated injury to the
tight junctions
• Results in extremely watery stool

• Lactose intolerance due to • Cholera, E. coli enterotoxins

Causes/
• Clostridium difficile
examples lactase enzyme deficiency
• Vasoactive peptides
Volume of stool • Moderately increased • Very large volume diarrhea
Response to
• Diarrhea stops • Diarrhea continues
fasting
Stool Na+ • <70 mEq/L • >70 mEq/L
Stool pH .. • <5.5 • Usually >S.6
Ion Gap • >100 mOsm/kg • <SO mOsm/kg

254
 D. Stool Output in Infants/Children contains approximately per liter (average composition of diarrhea):
55 mEq of Na+
25 mEq of K+
15 mEq of HCO-

II. MANIFESTATIONS (OF DEHYDRATION)

In history taking and examination, ask/check the following:
Oral intake
Frequency & volume of stool output
General appearance & activity of the child
Frequency of urination
0 Recent travel
Use of antimicrobials
0 Intake of seafood, contaminated wate1; uncooked meat or unwashed vegetables
0 Duration of diarrhea
, Presence of blood
Other symptoms (fever, tenesmus, vomiting)
• See Fluids and Electrolytes chapter for classification of degree of dehydration

III. DIAGNOSIS
• Diagnosis is based on clinical recognition, evaluating severity by rapid assessment
• For most cases, no laboratory tests are required except for epidemiologic purposes
DIAGNOSTICS REMARKS/FINDINGS
• Examine for mucus, blood, and leukocytes
Stool exam • Fecal leukocytes indicate bacterial invasion of colonic mucosa, or early

Stool culture
infection by Shigella, Shiga toxin-producing£. coli, or£. histo/ytica
• Usually done in the following cases:
0

0
Hemolytic Uremic Syndrome (HUS) is suspected
Bloody diarrhea
I
0Stool with fecal leukocytes
0During outbreaks
0Immunocompromised patients

IV. MANAGEMENT
• Management of dehydration is the cornerstone
Rehydration
• Rehydration with replacement of losses during the first 4-6 hours
• Reintroduce food once rehydration is complete
• Continued enteral feeding in diarrhea aids in recovery
Feeding
• Zinc supplementation reduces duration & severity of diarrhea and
prevents recurrence
Anti-diarrheals • Generally not recommended
• Generally not administered
• Antibiotics may be necessary for some infectious causes to reduce the
Antibiotics fluid requirements and limit the excretion of organism:
0 Shigella: Ciprofloxacin

° Cholera: Doxycycline /Tetracycline/ Co-trimoxazole/ Macrolides

0 Amoebiasis: Metronidazole

Source:KliegmanR,et al. NelsonTextbook

of Pediatrics
(21sted.).Canada:Elsevier:2020
EhererA, et al.Gastroenterology;
1992

255
APPROACH TO MANAGEMENT OF ACUTE GASTROENTERITIS (AGE)
• Assess the degree of dehydration & provide fluid & electrolyte replacement
Prevent spread of the enteropathogen
• Determine etiologic agent in selected cases & provide specific therapy if indicated

I. ADDRESS HYDRATION STATUS AND REPLACE FLUIDS & ELECTROLYTES

WHO Assessment Chart to Assess Hydration Status

PARAMETER• 1-.~~~D~
DEH~git~
General Lethargic***,
Alert Irritable Unconscious
condition
Eyes•• Normal Sunken Sunken
Drinks poorly, not
Thirst None Drinks eagerly able to drink
Skin
Quick Slow Very slow
retraction ••

Weight loss % <5% 5-10% >10%

Does not fulfill 2e2of the 4 severe
;;,2 of the 4 moderate
Criteria criteria for some or signs
signs
severe dehydration
Fluid deficit
<50 mL/kg 50-100 mL/kg >100 mL/kg
ml/kg
• Atleast2 parameters(generalcondition,eyes,thirst,skinretraction)mustbe fulfilled
to classifypatient
•• Eyesandskinretractionnotreliablein malnourished patients
'"Lethargic:notthe sameas sleepy;child'smentalstate is dulland cannotbe fullyawakened
Source:WorldHealthOrganization.
Thetreatment
of diarrhea.Geneva;2005

II. ORAL REHYDRATING SOLUTION (ORS)

• Use of ORS reduces stool output, vomiting, and need for unscheduled IV therapy
• Reduced osmolarity ORScontains 1:1 sodium to glucose ratio to maximize the sodium-
glucose co-transport
REDUCED OSMOLARlTY ORS (mEq/L)

Glucose 75
Sodium 75
Chloride 65
Potassium 20
Citrate 10

Osmolarity 245
Source:Kliegman R.el al. NelsonTextbookof Pedialncs(21sted.).Philadelphia:
Elsevier:
2020
WorldHealthOrganization.Thetreatment of diarrhea.Geneva;
2005
LongS,et al. Pnnciples
andpracticeof pediatncinfectious
diseases(4thed.).Saunders Elsevier:
2012

Practical Tips: For patients with vomiting it is better to administer ORS slowly via
dropper, with few minutes of rest in between, instead of drinking it from a cup or bottle.
If patient remains to vomit ORS despite the above strategy, it can be given in smaller
volume with longer interval in between. For example: give a teaspoonful every 1-2 minutes.
If the child vomits, wait 5-10 minutes then continue ORS administration more slowly

256
III. COMPOSITION OF COMMONLYUSED FLUIDS
• Fruit juice, soda, sports drinks, and commercially prepared soup a,·e not recommended since
their electrolyte composition & osmolarity does not match physiologic replacement of losses
• WHO recommends the use of reduced osmolarity ORS (applicable for patients without
severe malnutrition)
• For those with severe malnutrition
In hospital management and referral to a specialist is recommended
° Full strength ORSshould not be used for oral rehydration Q1alfstrength ORSis recommended)

IV. WHO-CDC PROTOCOLON REHYDRATION

PLAN REMARKS
• Fluids: Give oral rehydrating solution (ORS) after every loose stool
AGE AMOUNT
<2 years • 50-100 mL per loose stool
2-10 years • 100-200 mL per loose stool
Plan A
>10 years • As much as tolerated

• Feeding: Continue breastfeeding/ regular feeding to prevent malnutrition

• Give supplemental zinc 10 mg/day ( <6 months) or 20 mg/day (2:6 months) for
10-14 days
• Follow-up: if child does not improve in 3 days or shows repeated vomiting, frequent
watery stools, marked thirst, eating & drinking poorly, feve1;bloody stools
• Fluids: Give 75 cc/kg ORS per orem over 4 hours
Re-assess hydration status after 4 hours and adjust treatment as needed

I
0

0May be repeated if some signs of dehydration are still present after 4 hours
• The following table may be used to approximate amount of ORS from patient's
age if the weight is unknown
---~---....-----....-------.--------,,------,
<4 mos 4-11 mos 12-23 mos 2-4 yrs 5-14 yrs 2:15 yrs
PlanB
<5 5-7.9 8-10.9 11-15.9 16-29.9 ~30

200-400 400-600 600-800 800-1.200 1,200-2.200 2.200-4,000

• After 4 hours, evaluate and continue treatment using plan A, B or C

• Feeding: continue feeding as in Plan A
• Follow-up as in Plan A
• Give a total of 100 cc/kg (PLR or PNSS) IV as follows:
° First 30 cc/kg over 1 hour for infants (<12 months) or 30 mins for children
0 Next 70 cc/kg over 5 hours for infants ( <12months) or 2.5 hours for children
• Fluid of choice is pLR, alternative is pNSS
• 30 ml/kg may be repeated once if the patient is still in shock
Plan C • Re-assess and adjust fluids accordingly
• Give ORS 5 ml/kg/hour as soon as the patient can drink (usually after 2-4 hours
for infants and 1-2 hours for children)
• After 6 hours for infants or 3 hours for children, evaluate and continue
treatment using plan A, B or C

IV. SAMPLE CASES

Case 1. A 4 year-old female was brought to your OPDclinic due to 1 day history of LBM.
On physical examination patient is active. PE is unremarkable.
Assessment • No signs of dehydration
• Treatment Plan A
0 Replace losses with ORS 100-200 mL per loose stool
Plan ° Continue feeding
0 Give zinc
0 Advise follow up

257
 Case 2. A 4 year-old female, weight of15 kg, was brought to the OPDdue to 4 day history
of LBMwith several episodes of vomiting. BP= 90/60 HR=120 RR= 20. Patient is awake,
drinks eagerly, with sunken eyeballs and poor skin turgor.
Assessment • Some signs of dehydration
• Treatment Plan B
, 75 mL/kg x 15 = 1.125 mL ofORS to be given within 4 hours
Plan
' If ORS is tolerated and patient has no signs of dehydration after 4 hours.
may send patient home on treatment plan A

Case 3. A 4 year-old male was brought in the ER due to 3 days of LBM. Patient is
lethargic, does not drink fluids, sunken eyeballs with a BP of90/60
Assessment • Severe dehydration
• Treatment plan C
Plan , pLR 450 mL IV for 30 minutes, then 1,050 mL to run for 2 ½ hours
, Reassess patient after 3 hours & manage accordingly (Plan A, B or C)

V. DETERMINEETIOLOGICAGENT & PROVIDETREATMENT

TYPE OF POSSIBLE CAUSATIVE
MANAGEMENT
DIARRHEA AGENT
• Rotavirus • Supportive
• Staphylococcus aureus • Supportive
• Salmonella • See IDS chapter for details
• Tetracycline OR erythromycin OR
Watery • Vibrio cholera 01 & 0139
azithromycin OR doxycycline
Diarrhea
• Giardia lamblia • Metronidazole
• Supportive
• Campylobacter jejuni
• If severe: azithromycin, erythromycin
• Clostridium difficile • Metronidazole or vancomycin
• Ciprofloxacin
• Shigella
Bloody • See IDS chapter for details
Diarrhea
• Entamoeba histolytica • Metronidazole

Watery or
Bloody • Escherichia coli • See IDS chapter for details

R.et al. NelsonTextbook

Sources:Ktiegman of Pediatrics
(21sted.}.Philadelphia:
Elsevier:2020
WortdHealthOrganization.
Thetreatment of diarrhea.Geneva;2005

CHRONIC DIARRHEA
I. ETIOPATHOGENESIS
• Increased total daily stool output with increased stool water content lasting <!4weeks
• Persistent diarrhea lasts 14 days or more. In clinical practice this is equivalent to loose or
watery stool more than 3 times per day
• Results from altered intestinal water and electrolyte transport

II. MANAGEMENT
• General supportive measures
• Nutritional rehabilitation
• Elimination diet
• Medications as indicated (for specific etiologies)

258
 SECTION FOUR
DISORDERS O.F THE GASTROINTESTINAL TRACT

GASTROESOPHAGEAL REFLUX DISEASE (GERD)

• Retrograde movement of gastric contents across the lower esophageal sphincter (LES)
into the esophagus

I.ETIOPATHOGENESIS
• Most common esophageal disorder in children of all ages
• GER is considered a physiologic process
• GERO is pathologic: frequent or persistent episodes and with complications (esophagitis,
respiratory symptoms, failure to thrive, feeding refusal)

A. Pathophysiology:
LES is supported by the crura of diaphragm at the gastro-esophageal (GE) junction
which has valve-like functions & forms the anti-reflux barrier
Transient LES relaxation (TLESR) is the major mechanism allowing reflux to occur
Gastric distention is the main stimulus for TLESR (straining, coughing, large volume or
hyperosmolar meals)

B. Epidemiology
Infant reflux evident at first few months of life, peaks at 4 months, resolves mostly at
12 months & nearly all at 24 months old
Genetic predisposition: autosomal dominant (locus GERDl in chromosome 13q14)

II. MANIFESTATIONS
AGE GROUP MANIFESTATIONS BASED ON AGE GROUP
• Regurgitation, excessive crying/irritability, vomiting, food refusal
I
Infants
• Persistent hiccups, abnormal posturing, impaired quality of life
• Impaired quality oflife, vomiting, esophagitis
Children • Peristent/chronic cough, aspiration pneumonia, wheezing
• Ear infections, stridor, heartburn
Adolescents • Impaired quality of life, esophagi tis, dysphagia
and adults • Heartburn, epigastric pain, chest pain
Source:KllegmanR. et al. NelsonTextbookof Ped,atncs(21sted.).Canada:Elsevier;2020

Ill. DIAGNOSIS
A. Clinical Diagnosis
Thorough history and PE suffice initially to reach diagnosis
No single test to definitely diagnose GERO
Empirical anti-reflux therapy using a trial of high-dose proton pump inhibitors (PPI)
may be done [if responsive, then it is virtually diagnostic of GERO)
Bo· :
DIAGNOSTIC REMARKS
Barium study of
the upper GIT
• To rule out anatomic causes of vomiting

Esophageal pH • Document acidic reflux episodes [normal value of distal

monitoring esophageal acid exposure <5-8% of total monitored time)
(gold standard) • No longer considered the sine qua non of GERO diagnosis
• Finding: erosive esophagitis
Endoscopy
• May be used to dilate reflux-induced strictures

Gastroesophageal
• To scan for reflux using 99mTc-labeled markers
scintigraphy

259
IV. MANAGEMENT
ASPECT MANAGEMENT
• Lifestyle modification is the foundation of GERO therapy
Conservative
• Maintain on prone or upright carried position (avoid prone position
therapy
for infants who are asleep J
• Thickening of formula by adding up to 1 tablespoon of dry rice cereal per 1
Dietary
oz of formula or using commercially thickened formula for full term infants
measures
• Avoid acidic/spicy foods, juices, alcohol, caffeinated & carbonated drinks
• Proton pump inhibitors (PPI): current standard of care (e.g.,
Medical omeprazole, lansoprazole, esomeprazole)
• Others: Antacids, HZ Receptor antagonists, prokinetic agents
• May be an option for intractable GERO, refractory esophagitis &
Fundoplication
strictures, with morbidities from chronic pulmonary disease
Source:KliegmanR. et al. NelsonTextbook
of Pediatrics(21sted.).Canada:Elsevier:2020
LightdaleJ. et al. Pediatrics:2013

PEPTIC ULCER DISEASE (PUD)

I.ETIOPATHOGENESIS
• Ulcers are deep lesions that breach the integrity of the epithelium & penetrate the
muscularis mucosa
• Erosions are superficial & stop short of the muscularis propria

II. MANIFESTATIONS
• Majority present with poorly localized dull or aching abdominal pain, which may be
relieved after antacids in some
• Classic symptom: epigastric pain relieved by food intake (seen only in minority of children)
• Hematemesis or melena is reported in 50%
School-age children and adolescents: dyspepsia, abdominal fullness, epigastric pain, nausea
Infants and younger children: feeding difficulty, vomiting, irritability

Ill. DIAGNOSIS
• Esophagogastroduodenoscopy: method of choice
• Diagnostic tests for H.pylori

IV. MANAGEMENT
• First line drugs for treatment of PUD in children are PP ls and HZ receptor antagonists
• Cytoprotective agents (sucralfate 40-80 mg/kg/day) can also be used as adjunct in the
presence of mucosa I lesions
• Management should be based on H.pylori susceptibility studies
Children infrequently develop complications from H.pylori infection (compared to adults)
Majority of H.pylori-infected children are asymptomatic
For treatment failures, refer to specialist

260
 SECTION FOUR
OBSTRUCTION IN THE GASTROINTESTINAL TRACT

INTESTINAL OBSTRUCTION
I.ETIOPATHOGENESIS
• Accumulation of food, gas, and intestinal secretions proximal to the point of obstruction
causes bowel distention
• Distention leads to decreased intestinal absorption, increased fluid & electrolyte
secretion, and isotonic intravascular depletion
• Intestinal contractions initially increase, then hypoactive bowel sounds persist
• Classified as either intrinsic (atresia, stenosis, meconium ileus, aganglionic megacolon) or
extrinsic (malrotation, constricting bands, intra-abdominal hernias, duplications)
• Definition of terms:
0 Atresia: complete obstruction of the bowel lumen
0 Stenosis: partial block ofluminal contents

II. MANIFESTATIONS
ASPECT MANIFESTATIONS
• Nausea & vomiting
Classic
• Abdominal distention
symptoms
• Obstipation
High • Large volume, frequent, bilious emesis

I
obstruction • Intermittent pain in epigastrium or periumbilical area, relieved by vomiting
Low • Moderate/marked distention with emesis that is progressively feculent
obstruction • Diffuse pain over the entire abdomen

III. DIAGNOSIS
DIAGNOSTIC REMARKS/FINDINGS
• Essential X-ray views in intestinal obstruction
0 Plain supine/upright/decubitus X-rays
° Cross-table lateral view: distended bowel above the obstruction with fluid
level and gas in the distended loops
• Abdominal X-ray findings in obstruction:
0 Poor gas distribution or gasless
0 Smooth bowel walls like sausages/hose
Radiograph 0 Preferential dilatation of the bowel proximal to the obstruction
• Many dilated air fluid levels in a given loop at different heights (candy canes)
0 Dilated loops in "stepladder" fashion
• Pneumoperitoneum may be seen in perforation (free air in the subphrenic
area or over the liver in the left lateral decubitus position)
• Ground-glass appearance in the RLQ with trapped air bubbles seen in
meconium ileus
Ultrasound • Identifies stenosis, malrotation, volvulus, intussusception
'
Contrast • Used when plain films or sonograms fail to identify source of obstruction
studies

IV. MANAGEMENT
• Fluid resuscitation
• Nasogastric decompression
• Cultures and antibiotics
• Surgery for strangulation
• Conservative measures for adhesions or strictures
• Water-soluble contrast enemas: useful in malrotation, meconium ileus/plug,
intussusception (diagnostic and therapeutic)
261
SOME CAUSES OF INTESTINAL OBSTRUCTION
I. MECKEL DIVERTICULUM
• It is a remnant of the omphalomesenteric duct which connects the yolk sac to the gut in
the embryo & provides nutrition
• Most common congenital GI tract anomaly
A. EtionathoQenesis: "Rule of 2's"
• Present in ~2% of the general population
• Usually located 2 feet proximal to the ileocecal valve
• Approximately 2 inches in length
• Can contain 2 types of ectopic tissue (gastric or pancreatic)
• Generally present before 2 years old
• Female to male ratio: 2:1

B. Manifestations, Diagnosis, and Management

• Symptoms usually arise within the first 2 years of life
• Diverticula is lined by an acid-secreting ectopic mucosa causing
Manifestations intermittent painless rectal bleeding and brick-colored or currant
jelly-colored stool
• Accounts for 50% of all lower GI bleeds in children <2 years of age
• Typical diverticulum is a 3-6 cm outpouching of the ileum along the
Diagnostics antimesenteric border 50-75 cm from the ileocecal valve
• Meckel radionuclide scan using Tc-99m pertechnetate
Management • Surgical excision (diverticulectomy) if symptomatic

II. INTUSSUSCEPTION
• Occurs when a portion of the alimentary tract is telescoped into an adjacent segment
(most often ileocolic)
• Upper portion of bowel (intussusceptum) invaginates into the lower part
(intussuscipiens) dragging its mesentery along with it into the enveloping loop
• Mesentery constricts and obstructs venous return
• lntussusceptum engorges leading to edema & bleeding from the mucosa
A. Etiopathogenesis
Most common cause of intestinal obstruction between 5 months to 3 years; M > F
Most common abdominal emergency in children< 2 years
Idiopathic in 90% of cases with some degree of correlation with adenovirus

B. Manifestations
• Pain
Classic Triad
• Palpable sausage-shaped abdominal mass (in RUQ)
(in 30%)
• Currant-jelly stool
• Severe paroxysmal colicky pain that recurs at frequent intervals
• Child usually has straining efforts with legs and knees flexed & loud crying
• Infant may initially be comfortable and play normally between
paroxysms, becoming progressively lethargic
Symptoms
• If not reduced, a shock-like state, with fever and peritonitis, can
develop
• Vomiting is usually present in the early phase and later on becomes
bile-stained

• 60% of infants pass currant jelly stool

Signs • Digital rectal exam [DRE): presence of bloody mucus is supportive of
diagnosis

262
 C Dia0. nosis
DIAGNOSTIC REMARKS/FINDINGS
• Preferred screening tool (98-100% sensitive and 98% specific)
Ultrasound • Tubular mass in the longitudinal view
• Doughnut or target sign in transverse view
X-ray (plain • Vague and non-specific findings
abdominal) • A density may be seen in the area of intussusception
Barium • Coiled-spring sign (thin rim of barium trapped around the
enema invaginating part within the intussuscipiens)

D. Management: Reduction of an Acute lntussusception

Reduction of an acute intussusception is an emergency procedure
Reduction may be done through the following:
Hydrostatic/saline reduction (should not be attempted if with signs of shock,
peritoneal irritation or intestinal perforation)
Pneumatic/air reduction
Surgical reduction (if with refractory shock, bowel necrosis or perforation,
peritonitis and multiple recurrences)
4-10% of patients can have spontaneous reduction

Ill. OTHER CAUSESOF INTESTINAL OBSTRUCTION

STEP VOLVULUS MALROTATION
• Incomplete rotation of
• Twisting of a loop of intestine

Pathogenesis
around its mesenteric
attachment site which may occur
at various parts of the GIT
• Sigmoid & cecum more
commonly affected
the intestine during fetal
development which is completed
by 3 months of gestation
• Most common type involves
failure of the cecum to move into
I
the RLQ
• *Triad ofvolvulus ("S+R"): • Majority present within the 1st
• Sudden onset of severe year oflife with symptoms of
epigastric pain
Manifestations acute or chronic obstruction
• Inability to pass a tube into
• Vomiting is the most common
the stomach
• Retching with emesis symptom in this age group

• Findings on UGIS:*
• Findings on Abdominal X-ray: • Corkscrew sign: the distal
• Dilated stomach duodenum and proximal
• Inverted U sign (distended jejunum do not cross the
sigmoid loop) midline and instead take an
Diagnosis*
• Coffee bean sign (a midline inferior direction (loops twist
crease corresponding to the on a small bowel mesentery)
mesenteric root in a greatly • Bird's beak appearance of the
distended sigmoid) duodenum as it encounters
the volvulated loop
• Derotation & decompression
by barium enema or with
• Surgery (Ladd procedure) for
rectal tube, sigmoidoscope,
any patient with a significant
Management colonoscope if no signs of bowel
rotational abnormality
ischemia or perforation
regardless ofage
• Laparoscopic derotation or
laparotomy + /- bowel resection
'UpperGISeries (UGIS)is the imagingtest of choice& the goldstandardin the diagnosisof malrotation& volvulus

Source:KliegmanR. et al. NelsonTextbookof Pediatrics(21sted.).Canada:Elsevier;2020

Holcomb,et al.Ashcraft'sPediatricSurgery(5thed.).Philadelphia:
SaundersElsevier;2010

263
 SECTION SIX
DISORDERS OF THE LIVER AND PANCREAS

Spread
Fecal-oral Yes No No No Yes

Blood/Blood No
Rare Yes Yes Yes
Product

Vertical/ No
No Yes Rare Yes
Perinatal

Saliva Yes Yes Yes ? ?

Sexual Rare Yes Yes Yes (Rare) No

Etiopathogenesis
Incubation Variable* 21-63 days
15-19 days 60-180 days 14-160 days
period

Age Young Any Any Any Any

Carrier State No Yes Yes ? No

Diagnosis and Management

HBsAg
Anti-HBs
Anti-HAV HBeAg Anti-HCV Anti-HOV Anti-HEV
Diagnostics
lgM Anti-HBe HCV RNA lgM and lgG lgM
Anti-HBc lgM
HBVDNAPCR
Peg-interferon
Management Supportive See below Supportive Supportive
Ribavirin
Prognosis
Chronic
Liver No Yes Yes Yes No**
Disease

Liver Cancer No Yes Yes Yes No

Fulminant
Rare Yes Rare Yes Yes
Disease

1-2%
Mortality
<0.5% <1% <1% <1% (if pregnant:
(Acute)
10-20%)

264
 HEPATITISA HEPATITIS B HEPATITIS C HEPATITIS D HEPATITIS E
(HAV) (HBV) (HCV) (HDV) (HEV)

Immunization

lmmuno-
globulin
Passive HBlg No No Yes
0.04-0.06
mL/kg
HBVaccine
Active Vaccine Vaccine No to prevent Vaccine
coinfection
(similarto HBC)
and60-180daysfor coinfection
·incubationperiodfor HepatitisD: 2-8weeksfor HOVsuperinfection
inimmunosuppressed
··chronichepatitis

2020
(21sted.).Philadelphia;
Source:KliegmanR. et al. NelsonTextbookof Pediatrics
Redbook.Reportof theCommittee on InfectiousDiseases.31sted;2018
Principles
Mandell.DouglasandBennett·s 2010
andPracticeof InfectiousDiseases;Philadelphia.

HEPATITIS-B
I. MODEOF TRANSMISSION
• Infected blood or body fluids: serum, semen, vaginal secretions, CSF,synovial, pleural,
pericardia!, peritoneal and amniotic fluid (most infectious)
• Percutaneous and permucosal exposure to fluids
• Sharing unsterilized needles, syringes, or glucose monitoring equipment
• Sharing inanimate objects such as razors and toothbrush
• Sexual contact with an infected person
• Risk of transmission is greatest if mother is also HbeAg-positive (up to 90% of these
infants become chronically infected if untreated)
I
• Perinatal exposure to infected mother: in utero, during labor and delivery:
Infants born to HBsAg ( +), HBeAg ( +) mothers: 70-90% risk of HBV
0Infants born to HBsAg ( +), HBeAg (·) mothers: 5-20% risk of HBV

II. DIAGNOSTICTESTS FOR HBV ANTIGENS& ANTIBODIES

DIAGNOSTIC USES/REMARKS
• First marker to appear & its rise coincides with onset of symptoms
• Detection of acutely or chronically infected
HBsAg
• During self-limited HBV infection, disappears a few weeks to several
months after infection
• Indication of resolved HBV infection
Anti-HBs • Determines immunity after vaccination (present long after hepatis B
vaccination)
• Identification of HBVinfected at risk of transmitting HBV(highly infectious)
HBeAg
• Serves as a marker of active viral replication
Anti-HBe • Identification ofHBV infected with lower risk of transmitting HBV
• Identification of acute, resolved or chronic HBV infection
Anti-HBc
• Passively transferred maternal Anti-HBc detectable for 24 months among
(total)
infants born to HBsAg+ women
• Identification of acute or recent HBV infection
• The only marker highly specific for establishing the diagnosis of acute
IgM anti-HBc
HBV infection during the "window" phase of infection
• Not reliable for detecting perinatal HBV infection

265
 Prodrome Convalesce11ce
Incubation Acute Disease Earty Late
Important diagnostic tests HBsAg HBsAg (Anti-HBC) Anll-HBc Anti-HBs (Anli-HBc)

Symptoms

SGPT(ALT)
....-........
.........
DNA polymerase ,,
H8Vparticle
, Core windows
.. • .... • •Ant1-H8c

'

Level of detection

Months after exposure 2 3 4 5 6 8

Hepatitis B Profile
Relative concentration of reactants

• Anti-HBs & total Anti-HBc: indicate resolved HBV infection

• HBsAg & total Anti-HBc: indicate chronic HBV infection
• HBeAg & HBV DNA: useful in the selection of candidates for antiviral therapy and for
monitoring response to therapy

III. INTERPRETATIONOF TEST RESULTSFOR HBV INFECTION

I
TIME FRAME HBsAg Anti-HBs Anti-HBc HBeAg

Never infected - - - -
Incubation period + - - +
Acute Infection + - +IgM +
Window period - - +lgM -
Complete recovery - + +IgG -
Chronic carrier + - +lgG -
Chronic active + - +IgG +
Vaccinated
- + - -
IV.MANAGEMENTOF HBV
• Acute infection: no specific therapy available (i.e., supportive)
• Chronic infection: treatment to prevent cirrhosis, hepatic failure, & hepatocellular carcinoma
• Treatment for patients in the immune-active form of the disease as shown by increased
levels of liver function tests, those with fibrosis on liver biopsy:
lnterferon-a2b
0

Lamivudine
Adefovi1; Entecavi1; Tenofovir
Source:Kliegman
R. el al. NelsonTexlbookof Pediatrics
(21sted.).Canada:Elsevier; 2020
DavisonS, et al. BMJ;2014
Redbook. Reportof theCommitteeon lnfecliousDiseases, 31sted;2018
266
ACUTE PANCREATITIS (AP)
I.ETIOPATHOGENESIS
• Most common pancreatic disorder in children
• Following an insult, trypsinogen is prematurely activated to trypsin within the acinar cell,
activating other pancreatic proenzymes, leading to autodigestion
• Common causes:
Blunt abdominal injury
Multisystem diseases (e.g. HUS and IBD)
Biliary stones and microlithiasis
0Drugs (valproic acid, L-asparaginase, 6-mercaptopurine, azathioprine)

II. MANIFESTATIONS

• Moderate to severe abdominal pain, • Severe nausea, vomiting,

Manifestations
persistent vomiting and fever abdominal pain
Recovery • Within 1 week • May be longer • Protracted (mortality rate 20%)
• Cullen sign (bluish discolor-
• Sterile pancreatic
• Most common ation around the umbilicus)
Others necrosis may be
form • Grey Turner sign (bluish
seen
discoloration in the flanks)

III. DIAGNOSIS
A. Diagnostic Criteria (2 out of 3 of the following are needed)

0
Abdominal pain
Serum amylase AND/OR lipase 3 times elevated (compared to upper normal limit)
Imaging findings characteristic or compatible with pancreatitis
I
B. Diagnostics
DIAGNOSTIC REMARKS/FINDINGS
• Serum amylase level typically elevated for up to 4 days
• Serum lipase (test of choice) is more specific than amylase):
Lipase or amylase
rises by 4-8 hours, peaks at 24-48 hours, remains elevated 8-14
days longer than serum amylase
• Hemoconcentration, coagulopathy, leukocytosis, hyperglycemia,
Laboratory
hyperbilirubinemia
findings
• High gamma-g!utamyl transpeptidase
• 20% of children may have normal findings
Abdominal • Findings include: pancreatic enlargement, pancreatic masses,
Ultrasound and fluid collections, abscess
CT scan • CT scan: has a major role in the diagnosis and follow-up of
children with acute pancreatitis

IV. MANAGEMENT& PROGNOSIS

• Analgesia, correction of fluid/electrolyte imbalance
• Refeed when vomiting has resolved, amylase is falling, and symptoms are resolving
• Early refeeding decreases complication and length of hospital stay
• Severe disease: enteral or TPN, antibiotics, gastric acid suppression, peritoneal lavage

Source:KliegmanR,et al. NelsonTextbook

of Pediatrics
(21sted.).Canada:Elsevier;
2020
Abu-EI-Haija
M,et al.JPGN;2018

267
 SECTION SEVEN
NUTRITION & RELATED DISORDERS
SEVERE CHILDHOOD UNDERNUTRITION (SCU)
• Spectrum of conditions
• Most severe forms:
0 Marasmus: non-edematous SCU with severe wasting
° Kwashiorkor: edematous

I.ETIOPATHOGENESIS
A. Reductive Adaptation - adaptive responses to inadequate energy /protein intake
Mobilization of fat stores
0 Once fat is depleted, protein catabolism takes place to maintain basal metabolism
Energy conserved by reducing activity and growth, basal metabolism, functional
reserve of organs, inflammatory and immune response

B. Changes include one or more of the following

Liver makes less glucose (hypoglycemia)
Liver makes less albumin, transferrin and other proteins
0 Heat production is less (hypothermia)
Kidneys excrete less (fluid overload)
0 Heart is smaller and weaker (cardiac failure)
Sodium builds up due to leaky membranes and inactive pumps (edema)
Potassium leaks out of the cell
0 Less gastric secretions and motility (bacterial colonization may occur in stomach and
small intestine)
Impaired immune fuction and RBC mass
Micronutrient deficiences (increased cell damage leading to edema and hair/skin
changes)

II. MANIFESTATIONS

FEATURE KWASHIORKOR MARASMUS

Growth failure • Present • Present
Wasting • Present • Marked
Edema • Present (may be mild) • Absent
Hair changes • Common • Uncommon
Mental
• Very common • Uncommon
changes

Dermatosis • Common • Does not occur

Appetite • Poor • Good
Anemia • Severe (in some cases J • Present but less severe
Subcutaneous
• Reduced but present • Absent
fat

Face • May be edematous • Drawn in, monkey-like

Fatty liver
• Present • Absent
infiltration

Source:KliegmanR. et al. Nelsontextbookof pediatrics(21sted.).Philadelphia:

Elsevier;2020

III. DIAGNOSIS
• Decreased serum albumin: most characteristic change
• Other findings include hypoglycemia, low plasma amino acid, low serum cholesterol,
decreased pancreatic & liver enzymes, anemia, delayed bone growth

268
IV. MANAGEMENT
A. Indications for Admission
THE FOLLOWING SHOULD BE THE FOLLOWING MAY BE
ADMITTED MANAGED AS OUTPATIENT
Severe edema or mid-upper arm circumference Edema or MUAC >115 mm and all of the
(MUAC) <115 mm and any of the following: following:
• Anorexia • Good appetite
• Clinically unwell • Clinically well
• Not alert • Alert

B.Aspects in Management
Immediate management of acute problems
Give appropriate antibiotics for bacterial infections
Correct dehydration, electrolyte abnormalities, hypothermia and hypoglycemia
° Correct micronutrient deficiencies
Cautious feeding and catch up growth
Co-management with GI specialist for caloric catch-up and nutritional support
Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Canada:Elsevier;2020
WHO.Guidelines
fortheinpatient
treatment of severelymalnourishedchildren.
Geneva:WHO;2013

MICRONUTRIENT DEFICIENCY
VITAMIN IMPORTANCE MANIFESTATIONS OF DEFICIENCY
1) Water Soluble Vitamins
• Beri-beri
Thiamine
(Bl)

Riboflavin
• Coenzyme in ketoacid
decarboxylation

• FAD coenzyme in
•
•
•
•
•
Polyneuropathy
Calf tenderness
Heart failure
Mucositis
Cheilosis
I
oxidation-reduction
(B2) • Anemia
reactions • Nasolabial seborrhea
• NAO coenzyme in
Niacin (B3) • Pellagra: dermatitis, diarrhea, dementia
oxidation-reduction reactions
• Microcytic anemia
Pyridoxine • Cofactor in amino acid • Cheilosis
(B6) metabolism • Glossitis
• Dermatitis

• Cofactor in carboxylase • Alopecia

Biotin (B7) • Dermatitis
reactions of amino acids
• Hypotonia
• Megaloblastic anemia
Vitamin • Coenzyme for 5-methyl-
• Peripheral neuropathy in 812 deficiency
B12 and tetrahydrofolate
Folate (differentiates it from folate deficiency
formation
wherein there is no neurologic manifestation)

• Scurvy
• Maintains integrity of
intracellular material
Vitamin C • Facilitates absorption of
iron and conversion of
folic acid to folinic acid
, Early symptoms: fever, irritability,
tachypnea, anorexia, generalized
tenderness especially in the legs
0 Pseudoparalysis with hips & knees semi-
flexed & the feet rotated outward
0 "Scorbutic rosary" at the costochondral
junction & depression of the sternum
0 Bluish,purple spongy swellings of the mucous
membranes especiallyover the upper incisors
, Perifollicular hemorrhages, hyperkeratosis
of hair follicles, "corkscrew hair"

269
 VITAMIN IMPORTANCE MANIFESTATIONS OF DEFICIENCY
2) Fat Soluble Vitamins
• Component of retinal • Nyctalopia, photophobia, xerophthalmia,
pigments keratomalacia, faulty epiphyseal bone
Vitamin A
• Role in bone, tooth, and formation, defective tooth enamel, impaired
epithelial development resistance to infection, Bitot spot

• Content in human milk • Rickets:

0 General softening of bones
depends on maternal status
0Widening of wrists & ankles due to increase in
• Facial exposure of 2 hours
VitaminD growth plate and metaphyseal circumference
at least once a week or 30 • Others: craniotabes, rachitic rosary (widening
minutes with bare arms of costochondral junction), Harrison groove
and legs is sufficient (depression along the lower cos ta I border)
• RBC hemolysis in premature infants
• Loss of neural integrity
Vitamin E • Antioxidant
• Posterior column and cerebellar dysfunction
• Pigmentary retinopathy
• Vitamin K deficiency bleeding
• Affects coagulation factors
• Most common site of bleeding are GI,cutaneous,
VitaminK X, IX,VII, II (Mnemonic:
umbilicalstump & post-circumcisionsite, mucosa!
1972), Protein C, Protein S • Intracranial bleeding is less common
Source:KhegmanR, et al. NelsonTextbookof Ped1atncs(21st ed.). Canada: Elsevier;2020

REFERENCES
1. Abu-El-Haija, M., Kuma1; S., Quiros, J., Balalaishnan, K, et al. Management of Acute Pancreatitis in tl1e Pediatric
Population: A Clinical Report From the Nortl1 American Society for Pediatric Gastroenterology, Hepatology and
Nutrition Pancreas Committee. JPGN,2018 66(1), 159-176.
2. American Academy of Pediatrics. Kimberlin DW, Brady MT. Jackson MA, Long SS, eds. Red Book: 2018 Repo,~ of
the Committee on Infectious Diseases. 31st ed. Itasca,IL:American Academy of Pediatrics2018
3. Christison-LagayER,Keller,CMand Langer,JC.Neonatal abdominal wall defects. Semin Fetal Neonatal Med. 16(3), 2011
4. Clohe1~, J., Eichenwald, E., Hansen, A., and Stark, A. Manual of Neonatal Care 7th ed. 2012. Philadelphia:
LippincottWilliams and Wilkins
5. Davison, S. and Strasser, S. Ordering and interpreting hepatitis B serology. BM); 2014
6, Dunkley, M., Zalewska, K, Shi, E,, & Stalewski, H, Management of Esophageal Atresia and Tracheoesophageal
Fistula in North Queensland. International Surgery, 2014: 99(3), 276-279.
7. Eherer; A, & Fordtran, J.Fecal osmotic gap and pH in experimental diarrhea of various causes. Gastroenterology,
1992, 103(2), 545-551. doi: 10.1016/0016-5085(92)90845-p
8. Gamba P and Midrio P. Abdominal wall defects: prenatal diagnosis, newborn management, and long-term
outcomes. Semin Pediatr Surg 2014, 23(5):283-90
9. Gomella TL, Cunningham, Mand Eyal F..Neonatolgy 7th Edition. 2013. McGraw-Hill.
10. Holcomb, G.,and Murphy, J. Ashcralt's Pediatric Surge,y 5th ed. 2010, Philadelphia: Saunders Elsevier.
11. Jones, N., Koletzko, S., Goodman, K., et al. Joint ESPGHAN/NASPGHAN Guidelines for the Management of
Helicobacter pylori in Children and Adolescents (Update 2016), JPGN,64(6), 991-1003.
12. Kliegman, R., Stanton, B., St. Geme, J., Scho1; N., & Behrman, R.. Nelson Textbook of Pediatrics 21st ed. 2020.
Philadelphia: Elseviei:
13. Ledbetter DJ: Congenital abdominal wall defects and reconstruction in pediatric surgery: gastroschisis and
omphalocele, Surg Clin Notth Am, 2012, 92(3):713-27, 2012
14. Lightdale, J., & Gremse, D, Gastroesophageal Reflux: Management Guidance for the Pediao•ician. Pediatrics, 2012
131(5), e1684-e1695. doi: 10.1542/peds.2013-0421
15. Long, S.,Probe,; C.,& Fischet; M,P1inciples and practice of pediatric infectious diseases 4th ed. 2012, Saunders Elseviei:
16. Mandell, Douglas and Bennett's Principles and Practice of Infectious Diseases; Philadelphia, 2010
17. NeuJ.Necrotizingenterocolitis:the search fora unifyingpathogenictheol}' leadingto prevention Pedian·ClinNorth Arn 1996
18, Prefumo Fetal: Fetal abdominal wall defects, Best Pract Res Clin ObstetGynaecol. 28(3):391-402, 2014, Table 1
19. Reshetnyak, V.,Karlovich, T.,& llchenko, L.Hepatitis G Virus. World J Gastroenterol, 2008 14(30), 4725-4734.
20. Walther AE et al: Newborn abdominal wall defects. In: Wyllie R et al, eds: Pediatric Gastrointestinal and Liver
Disease, 5th ed. Philadelphia, PA: Elsevier: 2016:654-65,e4, Table 58-1
21. World Health Organization. Guidelines for the inpatient treatment of severely malnourished children. 2013
Geneva: WHO. Available from< https://www.who.int/nuo·ition/publications/guide_inpatient_text.pdf>
22. World Health Organization, The treatment of diarrhea. Geneva: 2005 Wyllie, R., and Hyams, J. Pedian·ic
gastrointestinal and liver disease 3rd ed. 2006. Philadelphia: Elsevier,
23. Zella, G.,and Israel, E. Chronic Diarrhea in Children. 2012 Pedian·ics In Review, 33(5), 207-218
270
HEMATOLOGY
AND
ONCOLOGY
 SECTION ONE
THE ANEMIAS

GENERAL APPROACH TO ANEMIA

• Anemia is generally defined as a hemoglobin (Hgb) concentration 2:2 standard
deviations below the mean for a health population of the same age and gender
• Refer to Essentials chapter for the actual values

Low Hemoglobin Level

What is the MCV?

LowMCV Normal MCV High MCV

... J
Iron Deficiency Anemia Drugs
Thalassemia Reticulocyte Vitamin 812/Folate
Sideroblastic Anemia Immune Hemolytic
Count
Anemia of Chronic Disease Diamond-Blackfan

Retie Count >3%

Are there clinical signs of hemolysis?

Yes
(e.g.,jaundice, indirect hyperbifirubinemia, high
LOH. decreased hopcoglobon)

No
I
Hereditary spherocytosis Hemorrhage
Hereditary el!iptocytosis
G6PD
Sickle Cell Disease
HUS/TTP

Source:NathanandOski'sHematologyandOncology,8th ed. Philadelphia;

2015
Introduction
to Hematology. Philadelphia;
1987

ANEMIAS OF INADEQUATE PRODUCTION

I. IRON DEFICIENCYANEMIA
• Insufficient total body iron to maintain normal physiologic functions
• Most common nutritional deficiency in children
A. Etiopathogenesis (Risk Factors)
INFANTS AND TODDLERS
• Low birth weight infants
• Prematurity
• Perinatal blood loss
• Early cord clamping
• Excessive consumption of cow's
milk (cow's milk is low in iron)
OLDER CHILDREN AND ADOLESCENTS
• Increased requirements (e.g., growth spurt,
pregnancy)
• Occult/chronic blood loss (e.g., peptic ulcer,
polyp, hemangioma)
• Menstrual blood loss
• Infection with intestinal hookworm, Tricht1ris,
Plasmodium, Helicobacter pylori, Giardia lamblia

273
B. Manifestations
• Most patients with mild to moderate anemia are asymptomatic
• Hgb 6-10 g/dL: mild irritability
General
Manifestations • Hgb 7-8 g/dL: pallor (most important clinical sign)
• Hgb <5 g/dL: lethargy, anorexia, easy fatigability, systolic flow
murmurs, and high-output cardiac failure
• Koilonychia: spoon nails
• Pica: desire to eat non-nutritive substances
Other Signs
• Pagophagia: desire to ingest ice
• Irreversible neurocognitive effects
Co· !
DIAGNOSTIC REMARKS
• Low RBC,MCV,reticulocyte count
CBC • Increased red cell distribution width (versus Thalassemia,
which has a normal ROW)
Peripheral blood • Microcytic, hypochromic RBCs (due to decreased hemoglobin
smear production or faulty function)
• Low serum iron & ferritin
Other tests • High total iron binding capacity (versus anemia of chronic
disease, which has low iron and low TIBC)
D.Management
• Look for cause of iron deficiency & address underlying problem
• Dietary counselling if anemia is due to a nutritional cause:
General Aspects
Delay introduction of cow's milk until at least 1 year of age
0

Limit intake of cow's milk to 24 ounces per day

0

• Iron salts at 4-6 mg/kg/day of elemental iron

° Ferrous sulfate: 20% elemental iron
Iron ° Ferrous fumarate: 33% elemental iron
Supplementation ° Ferrous gluconate: 12% elemental iron
• Iron polymaltose complex (causes less gastrointestinal upset)
• Continue treatment up to 2-3 months after Hgb has normalized
• Indicated for patients with imminent heart failure or severe
Blood transfusion
anemia with ongoing blood loss
Source:WHO.Serumferntmconcentrations.
Geneva:WHO;2011

12-24 hours • Subjective improvement (e.g.,increased appetite, decreased irritability)

36-48hours • Initial bone marrow response (e.g., erythroid hyperplasia)
48-72 hours • Reticulocytosis, which peaks at 5-7 days
4-30 days • Increase in hemoglobin levels
1-3 months • Repletion of iron stores

2. Follow-Up
Repeat CBC4 weeks after iron therapy (at this time, the hemoglobin has usually
risen by at least 1-2 g/dL and is often within normal levels)
If the anemia is severe, check for reticulocytosis within 2-3 days of therapy
Consider other causes of anemia when there is poor response to iron therapy

274
II. APLASTIC ANEMIA
• Life-threatening bone marrow failure
• Hallmark is peripheral pancytopenia with marrow hypoplasia or aplasia

A. Etiopathogenesis
Due to defect of hernatopoietic stem cells, defect in the rnicroenvironrnent of the
hematopoietic cells, or antibody-mediated mechanisms
• May be inherited (e.g. Fanconi anemia, Shwachrnan-Diarnond syndrome,
dyskeratosis congenital or acquired
Majority of cases are idiopathic
ACQUIRED ETIOLOGY CAUSES
Cytotoxic drugs and • Chemotherapy
radiation • Radiation therapy
• Anti-seizure agents (carbamazepine, phenytoin)
• Antibiotics (sulfonamides, chloramphenicol)
Drug/chemical reaction • NSAIDs[indornethacin, phenylbutazone)
• Anti-thyroid drugs (methimazole, propylthiouracyl)
• Others: gold, arsenicals, benzene
• Epstein-Barr virus
Viral infections • Seronegative hepatitis
• Others: HIV,Herpes viruses
• Eosinophilic fasciitis
Immune disorders • Systemic lupus erythernatosus
• Graft-versus-host disease
• Paroxysmal nocturnal hemoglobinuria
Others
• Thymoma, pregnancy, anorexia nervosa

I
8. Manifestations
Recurrent episodes of infection due to leukopenia
• Mucosa! hemorrhage or rnenorrhagia due to thrombocytopenia
• Pallor and easy fatigability due to anemia
C. Diagnosis
1. Diagnostics
DIAGNOSTIC FINDINGS/REMARKS
CBC&PBS • Pancytopenia (anemia, leukopenia, thrornbocytopenia)
• Required to establish diagnosis
Bone Marrow
• Hypocellular marrow with a decrease in all cell components,
Aspiration Biopsy
composed mostly of fat cells and marrow stroma

2 S f AI I A
MODERATE APLASTIC SEVERE APLASTIC
ANEMIA ANEMIA
Absolute Neutrophil 5OO-1OOO/rnm' <5OO/mm'
Count (ANC)

Platelet Count 2O,OOO-1OO,OOO/rnm

3 <2O,OOO/rnm'
Corrected <1% <1%
Reticulocyte Count

D. Management
Allogeneic hernatopoietic stern cell transplant (treatment of choice)
lmrnunosuppressive therapy with horse anti-thyrnocyte globulin and cyclosporine
(treatment of choice if without HLA-rnatched sibling or donor)
RBC and platelet transfusion
• Granulocyte colony-stimulating factor (G-CSF) or granulocyte-macrophage colony-
stimulating factor (GM-CSF)
• Withdrawal of any toxic causative agent
275
III. CONGENITALANEMIAS
ETIOPATHOGENESIS
Fanconi Anemia
• Most common inherited
a plastic anemia
• Cells cannot properly
repair DNA damage
known as interstrand
crosslinks
Shwachman-Diamond Syndrome
• Autosomal recessive
inherited aplastic
anemia
• Genetic mutation affecting
1ibosome synthesis
Diamond-Blackfan Syndrome
• >90% diagnosed
within 1 year old
• Genetic mutations
affecting ribosome
synthesis
DIAGNOSIS
• Based on physical
manifestations and
cytogenetic analysis
• Based on bone marrow
dysfunction and exocrine
pancreatic dysfunction
• Criteria (all should be met):
0 Age <1 year
0 Macrocytic anemia with
no other cytopenias
0 Reticulocytopenia
0 Normal marrow
cellularity with a paucity
of erythroid precursors
MANAGEMENT
• Allogenic hematopoietic bone
marrow transplantation is the
only established curative therapy
• Androgen therapy to improve
blood counts
• Blood transfusion, G-CSF
• Oral pancreatic enzyme
replacement
• Fat-soluble vitamins
• Blood transfusion, G-CSF
• Hematopoieticstem cell transplant
• Steroids: mainstay of therapy
• Blood transfusion for those
who do not respond to or
tolerate steroids

HEMOLYTIC ANEMIAS
I. GLUCOSE-6-PHOSPHATEDEHYDROGENASE(G6PD) DEFICIENCY
A. Etiopathogenesis
Most common inherited enzymatic disorder
• Genetic defect in the red blood cell glucose-6-phosphate dehydrogenase
• Lack of this enzyme causes hemolytic anemia when exposed to oxidative stress

• Methylene blue
• Antibacterial: nitrofurantoin, • Arsine
furazolidone, nitrofurazone • Phenylhydrazine
• Analgesic/antipyretic: acetanilid, • Toluidine blue
aspirin • Trinitrotoluene
• Antihelminthic: B-napththol, • Aniline dye
niridazole, stibophan FOOD AND DRINKS
• Antimalarials: chloroquine,
primaquine, pentaquine, pamaquine • Fava beans
• Sulfonamides: TMP-SMX,sulfonylureas •Redwine
• Legumes
• Miscellaneous
• Blueberry
0 Aceytylphenylhydrazine
• Soya food
0 Dimercaprol
° Flutamide (anti-androgen) OTHERS
• lsobutyl nitrite
• Menthol (alaxan gel, efficascent oil,
0 Mepacrine (antiprotozoal) Listerine mouthwash, omega pain kille1;
• Phenazopyridine mentopas medicated plaster)
0 Probenecid ( uricosuric drug) • Camphor
0 Thiazolesulfone (lupus vulgaris) • Naphthalene (moth balls)
• Rasburicase (uricolytic agent) • Henna
• Some herbs

276
 B. Manifestations
0 Most are asymptomatic unless triggered by precipitants
0 Hemolysis develops in 24-48 hours after exposure to precipitant
C. Diagnostics
0 G6PD Assay
° CBCwith PBS - low hemoglobin, Heinz bodies, anisopoikilocytosis, bite cells

D. Management
0 Avoidance of precipitants
0 Acute hemolytic episode
0Identify and remove the culprit agent
Hydration and transfusion for severe anemia
Source:Bolton-Maggs PH,et al. BrJ Haemalol2012
KliegmanR. et al. NelsonTextbookof Pediatrics (21sted.).Elsevier;2020
MichlilschJ. et al. PediatrBloodCancer;2009
II. THALASSEMIA
• Disorders wherein the alpha and globin chains are disrupted due to disease-causing
variant in one or more globin genes
ALPHA THALASSEMIA BETA THALASSEMIA
• Highly prevalent in Southern • Highly prevalent in Africa followed
China, Malaysia, and Thailand
by Asia
• Caused by gene deletions in one
• Caused by mutations in one or both
Pathogenesis or more genes encoding for alpha
of the beta globin genes
globin chain
• Severity correlates with the
• Vary depending on numbers of
gene deletion amount of Beta-globin production

• Severe
• Hydrops • B-Thalassemia microcytic
• Severe

11
Fetalis with Major (both beta anemia with
microcytic
Barts Hgb globin genes target cells
anemia • Transfusion
(4 Foci deleted) mutated)
dependent

• Thalassemia • B-Thalassemia • Moderate

• Moderate lntermedia microcytic
Major
microcytic (at /eastl beta anemia
Manifestations (HbH Disease) glob in genes • Non-transfusion
anemia
(3 Foci deleted) mutated) dependent
• Thalassemia • Mild,
Minor microcytic • B-Thalassemia
(2 Foci deleted) anemia Minor /Trait • Mild,
(one beta globin microcytic
• Normal gene mutated anemia
• Silent Carrier
hemoglobin, (carrier))
(1 Foci deleted)
normal MCV
• Definitive diagnosis through hemoglobin electrophoresis
• On CBC:
Diagnostics 0Anemia & low red cell indices with normal red cell distribution width
0 Decreased reticulocyte count due to ineffective erythropoiesis
• PBS: microcytic, hypochromic RBCs,Target cells and Heinz bodies
• Hematopoietic stem cell transplantation is the only cure for 4-foci
deletion and for beta thalassemia major
• Frequent transfusions, iron chelation therapy with deferoxamine or
Management deferasirox as needed
• Folate supplementation, low iron diet
• Splenectomy for those who develop hypersplenism (falling steady state
hemoglobin with thrombocytopenia or rising transfusion requirements)
Source:Fucharoen
S, et al. Hematology
AmSocHematolEducProgram;2009
MartinA, et al. PedialrClinNorthAm;2013

277
III. OTHER HEMOLYTICANEMIAS
HEREDITARY
SPHEROCYTOSIS
• Most common cause of
hemolytic anemia due to a red
cell membrane defect
Pathogenesis
• Due to abnormalities of ankyrin
and spectrin (proteins involved
in RBCcytoskeleton)
• Pallor, jaundice
• Splenomegaly
Manifestations • Susceptibility to a plastic crisis
(when infected by certain
viruses like Parvovirus Bl 9)
• Osmotic fragility test: confirms
presence of fragile sphere-shaped
RBCs
Diagnosis • CBCreveals high MCHC,normal
MCV,high reticulocyte count
• Peripheral smear: spherocytes
• Increased indirect bilirubin level
• Splenectomy is curative and
recommended for:
0 Transfusion-dependent
patients
0 Severe disease
Management 0 Moderate disease with
frequent hypoplastic or
aplastic crises, poor growth,
or cardiomegaly
• Folate supplementation may also
be useful
KliegmanR.et al. NelsonTextbookof Pediatrics (21sted.).Elsevier:2020
278
SICKLE CELL DISEASE
• Autosomal recessive disorder
• Develops at around 6 months of
age when sickle cell hemoglobin
replaces HbF
• Clinical hallmarks: vasoocclusive
phenomena & hemolysis
• Recurrent painful episodes from
hypoxic tissue injury
• Organ-system complications
(e.g., a plastic crisis, splenic
sequestration, priapism, stroke,
avascular necrosis, acute chest
syndrome)
• High performance liquid
chromatography
(preferred method of diagnosis)
• Hemoglobin electrophoresis
• Peripheral blood smear - Howell
Jolly Bodies and sickle cells
• "Crew cut" or "hair on end"
appearance on skull radiographs
• Analgesia and hydration for acute
crises
• Hydroxyurea to promote production
offetal hemoglobin
• Vaccinations against encapsulated
organisms
• Folate supplementation
Source:Bolton-Maggs PH,et al. Br J Haematol2012
MichlitschJ, et al. PediatrBloodCancer:2009
 SECTION TWO
B.LEEDING DISORDERS

GENERAL APPROACH TO BLEEDING

Bleeding

Mucocutaneous Bleeding Soft Tissue Bleeding

Platelet Count PT/PTT

Low Normal Abnormal Normal

i Cti·o·n·
o·ecrea
~·e
·Prc;d·u - 'vWD
• Aplastic anemia i Factor XIII deficiency
: Platelet function disorders
Increased Destruction I Vascular abnormalities
• ITP,DIC,TTP

PT abnormal

I
PT normal PT abnormal
PTT normal PTT abnormal PTT abnormal

Factor Vlll, IX, XI, XII deficiency

Factor VII Deficiency lupus anticoagulant
Warfarin toxicity Heparin
vWD

Sources:NathanandOski'sHematology
andOncologyof InfancyandChildhood(8thed).Philadelphia:
2015
RapaportSI. Introduction
to Hematology.
Philadelphia;
1987

OVERVIEW OF THE FINDINGS IN THE DIFFERENT BLEEDING DISORDERS

PT PTT . BLEEDING PLATELET

TIME COUNT
ITP Normal Normal Prolonged Decreased
Hemophilia Normal Prolonged Normal Normal
Normal or
vWD Normal Prolonged Prolonged
Decreased
DIC Prolonged Prolonged Prolonged Decreased
Vitamin K Normal or
Prolonged Normal Normal
deficiency Prolonged
DIC:Disseminated
lntravascular
Coagulation
ITP:ImmuneThrombocytopenia
vWD:vonWillebrandDisease

279
IMMUNE THROMBOCYTOPENIA (ITP)
I. ETIOPATHOGENESIS
• Previously called Idiopathic Thrombocytopenic Purpura
• Most common cause of thrombocytopenia in childhood
• Immune-mediated disorder which can be triggered by viral infection, immunologic, or
environmental trigger
• Peak age: 1-4 years old

II. MANIFESTATIONS
• Classic presentation is previously healthy 1-4-year-old child with sudden onset of
generalized petechiae and purpura
• Recent history of viral illness in 50-65%
• Variable severity of bleeding
Mild: bruising and petechiae
0 Moderate: more severe skin and mucosa I lesions, troublesome epistaxis, and menorrhagia
0 Severe: bleeding episodes requiring transfusion or hospitalization

III. DIAGNOSIS
DIAGNOSTIC REMARKS
• Common finding: severe thrombocytopenia ( <20,000/uL)
CBC
• Normal Hgb, WBC count
Peripheral blood smear • Large platelets
• Indications: abnormal WBC count, unexplained anemia
Bone marrow aspiration
• Normal or increased megakaryocytes
biopsy
• Normal erythroid and myeloid precursors

IV. MANAGEMENT
• No treatment for mild and moderate symptoms
• Intravenous immunoglobulin 0.8-1 g/kg
• Prednisone 1-4 mg/kg/day
• Splenectomy for:
0 Life-threatening hemorrhage (intracranial bleed)
° Children ;,,4 years old with chronic ITP lasting> 1 year
° Children whose symptoms are difficult to control

THROMBOTIC THROMBOCYTOPENIC PURPURA

I. ETIOPATHOGENESIS
• Thrombotic microangiopathy resulting from reduced activity ofvon Willebrand factor-
cleaving protease ADAMTS13

II. MANIFESTATIONS
• Characterized by platelet rich thrombi in small vessels causing microangiopathic
hemolytic anemia and thrombocytopenia
• Five cardinal symptoms (FAT RN):
Fever
0 Anemia (microangiopathic, hemolytic type)
0 T hrombocytopenia
0 Renal dysfunction
Nervous system changes (changes in affect or orientation; aphasia, blindness, seizures)

III. DIAGNOSTICS
• Microangiopathichemolytic anemia (schistocytes, spherocytes, helmet cells)
CBCwith PBS • Elevated reticulocyte count
• Thrombocytopenia
Blood Chemistry • Elevated BUNand creatinine

IV. MANAGEMENT
• Plasmapheresis (to reverse platelet consumption) effective in 80-95% of cases
• Rituximab, steroids, and splenectomy are for refractory cases
280
HEMOPHILIA
• Hereditary, sex-linked hematologic disorder occurring almost exclusively in males
• Marked by delayed clotting of the blood caused by a deficiency of clotting factors

I. ETIOPATHOGENESIS
Three types
Hemophilia A or Factor VIII deficiency
• Hemophilia B or Factor IXdeficiency
• Hemophilia C or Factor XI deficiency
• Hemophilia A and Bare X-linked recessive, hemophilia C is autosomal recessive
• Hemophilia A more common and more severe than hemophilia Band C
INTRINSIC PATHWAY EXTRINSIC PATHWAY

I Contact Activation I
I
~-~
~
Tissue Factor

~~Xa PF-3

VIII
Tlla
•~
r---::-7 ,..ca++ ~
~ • •L_'.'.~

~-~~-~
!! Prothrombin Thrombin

Coagulation
Extrinsic

intrinsic
cascadeshowingthe Intrinsic,
and CommonPathways.Note
thatHemophiliaA,BandCare foundinthe
pathway.
Fibrinogen

Xllla
Soluble Fibrin

Insoluble Fibrin
I
II. MANIFESTATIONS
• First sign of early joint hemorrhage: warm, tingling sensation in the joint
Symptoms
• Easy bruising
• Hallmark of hemophilia: hemarthrosis
• Most common earliest joint involved: ankle
Signs • Older children and adolescents: knees and elbows
• Intramuscular hematomas
• Mucosa) bleeding is rare

III. DIAGNOSTICS
DIAGNOSTICS FINDINGS/REMARKS
Bleeding time • Normal

Prothrombin Time and INR • Normal

PTT • Prolonged (Usually 2-3x upper limit of normal)
Factor Levels • Decreased

281
IV. MANAGEMENT
• Factor replacement during bleeding or as prophylaxis before surgical and dental procedures
• For mild/moderate bleeding: factor VIII or factor IXvalues must be raised to ~35-50% levels
• For major hemorrhages: the doses of factors VIII or IXshould achieve levels of 100% activity
• Factor VIII transfusion
Hemophilia A
• Alternatives: Cryoprecipitate, FFP,Emicizumab
• Factor IXtransfusion
Hemophilia B
• Alternatives: Cryosupernate, FFP
• Factor XI transfusion
Hemophilia C
• Alternatives: Cryosupernate, FFP

VON WILLEBRAND DISEASE (vWD)

I. ETIOPATHOGENESIS
• Most common inherited bleeding disorder
• It is due to missing or defective van Willebrand Factor (VWF)
• Characterized by mutation that results to a quantitative (type 1 or 3) or
qualitative (type 2) defect in von Willebrand Factor (vWF)
0 vWF binds platelet to injured subendothelium
0 vWF also acts as a carrier for factor VIII
• Transmitted in an autosomal dominant manner affecting both males and females

II. MANIFESTATIONS
• Disturbs both primary and secondary hemostasis
• Increased bleeding time causing mucous membrane bleeding, petechiae, purpura
• Often have a family history of bleeding

III. DIAGNOSTICS
• Decreased vWF levels
• Decreased Factor VIII
• Prolonged bleeding time
• Abnormal platelet adhesion
• Increased PTT
• Ristocetin cofactor assay (measures vWF antigen levels and activity)

IV. MANAGEMENT
• Desmopressin: increases amount of circulating vWF by release from storage
• Replacement therapy
• Antifibrinolytics
• Hormonal treatment with estrogen in women

282
 SECTION THREE
ONCOLOGY
LEUKEMIAS
• Most common form of cancer in children
• Types of leukemias and frequency:
• Acute lymphoblastic leukemia [ALL): most common
• Acute myelogenous leukemia (AML)
• Chronic myelogenous leukemia (CML)
Juvenile myelomonocytic leukemia (JMML)

I. ACUTE LYMPH0BLASTIC LEUKEMIA (ALL)

A. Etiopathogenesis
Malignant proliferation oflymphoblasts
Peak incidence 2-6 years old, affecting more males than females
Etiology is unknown
Risk factors: Down syndrome, Neurofibromatosis type 1, exposure to medical
diagnostic radiation in utero and in childhood, drugs (e.g., particularly alkylating
agents, benzene exposure, advanced maternal age)

B. Manifestations
Acute onset (<4 weeks duration of symptoms)
Most common presentation are non-specific (e.g., anorexia, irritability, lethargy)
Signs of marrow failure (e.g., anemia, bleeding, purpuric/petechial lesions, low-grade fever)
Signs of infiltration (e.g., bone pain, lymphadenopathy, hepatosplenomegaly)

C. Diagnosis
DIAGNOSTICS FINDINGS/REMARKS
Bone marrow aspiration
biopsy

CBC
• >25% of the bone marrow cells are a homogenous
population of lymphoblasts
• Anemia, thrombocytopenia
• Atypical lymphocytes
I
• Some with hyperleukocytosis (WBC > 100xl0 9 /L)
Peripheral blood smear • Presence of blasts
Chest radiograph • Mediastinal mass (usually in T cell immunophenotype)
CSF • May contain lymphoblasts [if with CNS involvement)

D. Risk Stratification (for treatment and prognostication)

HIGH
CRITERIA STANDARD (DOES NOT FIT
STANDARD RISK CRITERIA)

Age • >l year old & <10 years old • <l year old & >10 years old
WBC at diagnosis • <50xl0 9 /L • > 50 x 10 9 /L
Immunophenotype • Pre B Cell • T Cell, mature B, Biphenotypic
CNS involvement • Negative • Positive

Response to • Day 7 absence of blast on • Day 7 presence of blast on

steroids peripheral blood smear peripheral blood smear

E. Management
• The single most important prognostic factor for ALL is receiving treatment
• Standard treatment: chemotherapy for 2-3 years with a goal of eradicating detectable
leukemia cells or remission:
BMA blast count <5%
Return of neutrophil and platelet counts to near-normal levels
283
 l. Phases of Chemotherapy:
PHASE REMARKS
Remission induction • Eradicate leukemic cells from the bone marrow
• Focuses on intensive CNS therapy in combination with
Consolidation
continued intensive systemic therapy
• Phase of aggressive treatment as well as relatively non-
Intensification
toxic phase of treatment depending on risk stratification
Maintenance • Given to maintain patient on remission

2. Complication of Chemotherapy: Tumor lysis syndrome (TLS)

Metabolic complications that may occur alter the treatment of neoplastic disorders
Manifestations: hyperphosphatemia, hypocalcemia (caused by precipitation of
calcium phosphate), hyperuricemia, hyperkalemia
Laboratory TLS: at least 2 metabolic abnormalities
Clinical TLS: laboratory TLS with increased creatinine level, seizures, cardiac
dysrhythmia, or death
Management: hydration, allopurinol, hemodialysis (for severe cases)

3. Bone Marrow Transplantation

For patients with poor prognostic features (high incidence of relapse, induction
failure, or extreme hypodiploidy)

II. OTHER LEUKEMIAS

AML CML JMML

• Associated with
Philadelphia
• Relative frequency
chromosomal • Typically affects <2
Pathogenesis of AML increases in
translocation (t(9;12)) years of age
adolescence
resulting in BCR-ABL
fusion protein gene
• Signs of marrow • Nonspecific symptoms
failure • Splenomegaly
• Subcutaneous • Initial chronic phase
nodules or (3-4 years) with
• Rashes,
"blueberry muffin" mild anemia and
lymphadenopathy,
Manifestations lesions thrombocytosis
splenomegaly,
• Gingival infiltration • Alter chronic phase,
hemorrhage
• Disseminated moves into accelerated
intravascular or blast crisis phase
coagulation with course similar to
• Chloromas acute leukemia
• Leukocytosis
• High WBC count
with increased
• >20% of bone with myeloid cells
monocytes
marrow cells at all stages of
• Thrombocytopenia
consist of differentiation in the
Diagnosis • Anemia with
homogeneous PBS and bone marrow
erythroblasts
population of blast (usually the same
• Myelodysplastic
cells appearance under the
pattern in bone
microscope)
marrow
• lmatinib, dasatinib
• Chemotherapy
(BCR-ABLtyrosine • Stem cell
Management • Stem cell
kinase inhibitor) transplantation
transplantation
• Hydroxyurea

284
LYMPHOMAS
• Neoplasms of lymphoid cells (lymphocytes, histiocytes & their precursors)
childhood cancers
• Two types: Hodgkin and Non-Hodgkin Lymphoma
HODGKIN LYMPHOMA (HL)
• Bimodal age distribution: 20-30
years and >SOyears
• Most common cancer in
adolescents and young adults
Pathogenesis
• Associated with viral infections
(EBV,CMV,HHV-6)
• Pathologic hallmark: presence of
Reed-Sternberg and Hodgkin Cells
• Painless lymphadenopathy
(cervical, supraclavicular, axillary
or inguinal)
• Mediastinal mass
Manifestations
• Systemic symptoms (fatigue,
anorexia, & B symptoms: weight
loss, fever and night sweats)
• Hepatic and splenic enlargement
ANN ARBO~RSTAGING
STAGE DESCRIPTION
One lymph node group
I involved
Two lymph node groups
II involved on the same side
of the diaphragm
Staging
Two lymph node groups
involved on both sides of
III the diaphragm OR extra-
lymphatic involvement
Diffuse extra-lymphatic
IV site involvement (usually
bone marrow, CNS, liver)
• Imaging Studies (To determine extent of the disease): CXR,CT,PET Scan
• Excision biopsy of lymph node demonstrating Reed-Sternberg Cells (HL) and
Diagnosis
malignant cells (NHL) (fine needle may not be adequate)
• Others (to check for organ involvement): CBC,ESR, LDH,creatinine, AST,ALT
• Chemotherapy
Management
• Radiation therapy
285
- 6% of
NON-HODGKIN LYMPHOMA (NHL)
• 60% of all lymphomas jn children
• Increasing prevalence with age
• Associated with EBV,HIV
• 4 major subtypes: lymphoblastic
lymphoma, Burkitt lymphoma,
diffuse large B-cell lymphoma,
anaplastic large cell lymphoma
• Aggressive Lymphomas
• Rapidly growing mass
• B symptoms (fever, night sweats
and weight loss)
• Indolent Lymphomas
• Slowly growing lymphadenopathy,
hepatomegaly, splenomegaly or
cytopenia
ST. JUDE STAGING
STAGE DESCRIPTION
Single extranodal tumor
I OR single nodal area
involved
• Single extranodal tumor
with regional node
involvement;
I
• At least 2 nodal areas on
II same side of diaphragm;
• 2 extranodal tumors on
same side of diaphragm;
• Primary GI tumor
• 2 extranodal tumors on
both sides of diaphragm
• At least 2 nodal areas on
III both sides of diaphragm
• lntrathoracic tumor
• Extensive intraabdominal
disease
CNS or bone marrow
IV involvement
• Radiation for CNSinvolvement
• Chemotherapy for B symptoms,
Stages Ill and IV
• Possible bone marrow transplant
BRAIN TUMORS
• Most common solid tumor in children

• Most common:
Pilocytic
astrocytoma • Second most common • Solid or solid-cystic
• Classic site of posterior fossa tumor tumors arising
occurrence: of childhood from remnants of
Pathogenesis
cerebellum • Most common Rathke pouch of the
• Most common malignant brain pituitary stalk in the
posterior fossa tumor of childhood suprasellar region
tumor: Cerebellar
astrocytoma
• Subacute • Increased intracranial
• Visual deficits that
progressive pressure because of
can be secondary to
neurologic signs obstruction of the 4th
compression on the
and symptoms ventricle (headache,
optic chiasm
(headache, nausea, vomiting,
• Direct damage to
Manifestations seizure, weakness, altered mental status,
structures can cause
memory loss, hypertension)
endocrine abnormalities
motor weakness, • Cerebellar
such deficiency of
visual symptoms, dysfunction (gait
growth hormone,
personality ataxia, dysmetria,
TSH, ACTH, ADH
changes) poor balance)
• MRI
• PET (to determine • MRI of brain & spine
metabolically active • Skeletal survey • MRI or CT scan
Diagnosis parts of the tumor)
• CSF cytology • Endocrine studies
• Biopsy for
malignancies not • Liver function tests
amenable to surge1y
• Surgical resection • Surgical resection
Management
• Radiotherapy and chemotherapy • Radiotherapy

MUSCULOSKELETAL TUMORS
OSTEOSARCOMA EWING SARCOMA

• Most common primary • Second most common primary

Pathogenesis malignant bone tumor
• Affects metaphysis of long bones
• Local pain and swelling, limping
• Systemic symptoms are
Manifestations generally absent
• Common site of metastases:
lungs and bones
• Sunburst pattern on X ray
Diagnostics • Spindle-cell-producing osteoid
on histopathology
malignant bone tumor
• Affects diaphysis of long bones
• Local pain and swelling, limitation
in range of motion
• Systemic manifestations (fever,
weight loss)
• Common site of metastases: lungs
and bones
• Onion-skinning, Cadman triangle,
permeative or moth-eaten
appearance on X ray
• Undifferentiated small round cell
of neural origin on histopath

• Chemotherapy
• Chemotherapy
Management • Radiation
• Surgery
• Surgery

286
ABDOMINAL TUMORS
NEUROBLASTOMA WILMSTUMOR HEPATOBLASTOMA

• Most common • Most common

extracranial solid primary malignant
tumor in children renal tumor in • Hepatic tumors are
• Third most children rare in children
common pediatric
Pathogenesis • Second most common • Median age of
cancer
• Median age of malignant abdominal diagnosis is 1 year
diagnosis is 22 tumor in children
months • Commonly affects 2-5
years old
• Abdominal mass
that crosses midline
• Abdominal pain
• Proptosis
• Periorbital
ecchymoses • Large asymptomatic
(raccoon eyes) • Painless abdominal abdominal mass
• Horner syndrome enlargement with • As disease
( unilateral ptosis, flank mass that does progresses,
Manifestations myosis, and not cross the midline abdominal pain,
anhidrosis) • Hematuria fatigue, fever, weight
• Localized back • Hypertension loss, anorexia,
pain, weakness vomiting
• Palpable
nontender

I
subcutaneous
nodules
• Systemic symptoms
(fever & weight loss)
• Abdominal CTscan
• Abdominal UTZ, CT
• 24-hour urine
homovanillic scan or MRI
• Abdominal UTZ, CT
acid [HVA)and • Electrolytes, renal
scan or MRI
vanillylmandelic function tests
• Liver biopsy
Diagnosis acid (VMA) • Chest X ray or
• Bone marrow • Tumor marker: alpha
CT scan to check
showing fetoprotein
for pulmonary
myelophthisic metastasis
marrow
• Biopsy
• Surgery • Surgery
Management • Chemotherapy • Chemotherapy
• Radiation • Liver transplant

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288
PULMONOLOGY
'
SECTION ONE
APPROACH TO COMMON COMPLAINTS

APPROACH TO DISORDERS OF THE RESPIRATORY SYSTEM

RESPIRATORY DIFFERENTIALS
MANIFESTATIONS
PROBLEMS

• Changes in voice, cry, or

presence of barking cough
• Stridor (usually inspiratory but • Croup
Signs of upper airway may be expiratory) • Anaphylaxis
obstruction • Poor chest rise • Foreign body aspiration
• Poor air entry on auscultation
• Drooling, snoring, or gurgling
sounds

• Tachypnea
• Wheezing (usually expiratory)
Signs of lower airway • Bronchiolitis
• Prolonged expiratory phase
obstruction • Asthma
with increased expiratory effort
• Cough

• Tachypnea (often marked)

• Pneumonia
• Grunting
• Pulmonary edema
Signs of lung • Crackles and decreased air
parenchymal disease • Trauma
movement
• Infiltrative disease
• Diminished breath sounds
• Tachycardia

• Variable or irregular RR
and pattern (i.e., tachypnea
alternating with bradypnea)
• Toxins

I
• Variable respiratory effort • Neuromuscular disease
Signs of disordered • Shallow breathing with • Metabolic
controlofbreathing inadequate effort • Drug overdose
• Central apnea (apnea without • Increased ICP
respiratory effort)
• Normal or decreased air
movement

Source:AHAandAAPPediatricAdvancedLifeSupport2016

291
APPROACH TO COUGH
• Cough is defined as a reflex response of the lower respiratory tract to stimulation of
irritant or cough receptors in the airway mucosa.

CHARACTER OF
CONSIDERATION
COUGH
• Atypical infections like Chlamydia and Mycoplasma, pertussis,
Staccato, paroxysmal
foreign body, cystic fibrosis
Whooping • Pertussis
All day, never during
• Habit cough
sleep
Barking, brassy • Croup, habit cough, tracheitis, tracheomalacia, epiglottitis
Hoarseness • Laryngeal involvement
Acute onset • Foreign body, pulmonary embolism
After exercise • Reactive airway disease
Accompanies eating, • Gastroesophageal reflux (GER), tracheoesophageal fistula,
drinking aspiration
Throat clearing • Postnasal drip, vocal tic
Productive (wet cough) • Infection, bronchiectasis, cystic fibrosis
Nocturnal • Asthma, reactive airway disease, GER, sinusitis
Seasonal • Allergic rhinitis, reactive airway disease
lmmunosuppressed • Bacterial pneumonia, Mycobocterium tuberculosis, M. avium,
patients cytomegalovirus, Pneumocystis jiroveci pneumonia
• Chlamydia psitacci (birds), Yersinia pestis (rodents), Francisel/a
Animal exposure tularensis (rabbits), hantavirus (rodents), histoplasmosis
(pigeons), Q fever ( cattle, sheep)
Workdays with
• Occupational exposure
clearing on days off
Source:KhegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Elsevier:2020
KliegmanR. el al. PracticalStrategiesin PediatricDiagnosisandTherapy(2nded.):2004

NOMENCLATURE OF LUNG SOUNDS

LUNG PROBLEM
TIMING DURATION PITCH AMPLITUDE TYPE

• Upper airway
Stridor • lnspiratory • Continuous
obstruction

• Lower airway
Wheeze • Expiratory • Continuous • High
obstruction

Coarse • Discontinuous • Lung tissue

• lnspiratory • Low • High
crackles • Long disease
Fine
• Discontinuous • Lung tissue
crackles • lnspiratory • High • Low
(rales) • Short disease

• Lower airway
Rhonchi obstruction
• Either • Continuous • Low
or lung tissue
disease
Source:KliegmanR. et al. NelsonTextbookof Pediatrics(21sted.).Elsevier:2020
American HeanAssociation Pediatric
Advanced LifeSupport Manual

292
 SECTION TWO
PULMONARY DISORDERS IN THE NEONATE

RESPIRATORY DISTRESS SYNDROME (HYALINE MEMBRANE DISEASE)

• Syndrome in premature infants with respiratory distress due to inability to generate the
intrathoracic pressure necessary to inflate the lungs without surfactant

I. ETIOPATHOGENESIS
• Deficiency or immaturity of surfactant, complicated by an overly compliant chest wall
• Effects of surfactant deficiency:
Increased surface tension causing alveolar collapse
, Progressive atelectasis, V/Q mismatch & hypoxia
' Leads to failure to develop an effective functional residual capacity (FRC)
• Incidence is inversely proportional to gestational age (e.g., highest incidence in those
delivered <28 weeks AOG)

II. MANIFESTATIONS
• Respiratory distress soon after birth: tachypnea, prominent grunting, intercostal/
subcostal retractions, nasal flaring, cyanosis
• Breath sounds may be normal or decreased with a harsh tubular quality
• Fine crackles on deep inspiration

III. DIAGNOSIS
DIAGNOSTIC REMARKS/FINDINGS
• Shows characteristic "ground-glass" pattern or fine reticular
granularity of the parenchyma
Chest radiograph
• Low lung volumes and air bronchograms within the first 24 hours of
life may be present
Arterial blood gas • Findings may include hypoxemia, hypercarbia, metabolic acidosis

Other tests
• Sepsis workup (early-onset sepsis can be indistinguishable from
RDS clinically)
• 20 echocardiography to exclude possible cardiac pathology
• Rule out other causes of respiratory distress (e.g.,electrolyte imbalance)
I
IV. MANAGEMENT
ASPECT MANAGEMENT
• Recommended within 15 min of birth to infants <26 weeks AOG
• For all preterm with RDS who require delivery room intubation for
stabilization
Surfactant
• Technique INSURE:Intubate-Surfactant-Extubate to CPAP(Intubate,
replacement
administer surfactant intratracheally through ET tube, connect to
CPAP)reduces the need for mechanical ventilation and development
of bronchopulmonary dysplasia (BPD)
• Most cases of mild RDS are self-limited
• CPAPis increasingly becoming the mainstay therapy for RDS
• Mechanical ventilation only for severe RDS, respiratory failure, or
Supportive care
persistent apnea
• Fluid and nutritional support
• Antibiotic therapy to cover for the most common neonatal infections
• Obstetric measures to prevent preterm delivery
Prevention • Antenatal corticosteroids (dexamethasone or betamethasone) for
mothers at risk of preterm delivery
Source:GomellaTL.et al. Neonatolgy 7thEdition.McGraw-Hill;2013.
KliegmanR,et al. NelsonTextbook of Pediatrics
(21sted.).Elsevier;2020
ClohertyJ, et al. Manualof Neonatal
Care(7thed.).Philadelphia;
2012
293
TRANSIENT TACHYPNEA OF THE NEWBORN (TTN)
Benign, self-limited respiratory distress syndrome of term and late preterm infants
related to delayed clearance of lung fluid
• Most common perinatal respiratory disorder ( 40% of respiratory distress after birth)

I. ETIOPATHOGENESIS
• Central mechanism: delayed fluid resorption
• Lung fluid inhibits gas exchange leading to increased work of breathing and
compensatory tachypnea
• Hypoxia develops due to poorly ventilated alveoli
• Infants born by elective cesarean section have a higher risk for TTN because they are not
exposed to the stress (lack of catecholamine surge & active Na· reabsorption in lung) and
absence of uterine contractions (contractions result in high transpulmonary pressure
leading to lung fluid efflux)

II. MANIFESTATIONS
• Early onset of tachypnea soon a~er birth or within the first 6 hours after delivery
Retractions, nasal flaring, expiratory grunting, or cyanosis relieved by 0, supplementation
Barrel chest due to increased A-P diameter (hyperinflation) ·
• Crackles on auscultation
Liver & spleen may be palpable due to hyperinflation

III. DIAGNOSIS
DIAGNOSTIC REMARKS/FINDINGS
• Mild to moderate hypoxemia
Arterial blood gas
• Normal partial CO2 due to tachypnea
• Hyperinflation/over aeration, prominent perihilar streaking
( engorged lymphatics)
• Flattened diaphragm on lateral view
Chest radiograph
• Fluid in the intralobar fissures 01; rarely, small pleural effusions
• Prominent pulmonary vascular markings: "fuzzy vessels"
sunburst pattern, peripheral air trapping
• Double lung point
Lung 0 Diagnostic

ultrasonography 0 Difference in echogenicity between upper and lower lung areas,

comet-tail a1tifacts

IV. MANAGEMENT
ASPECT MANAGEMENT
• Oxygenation & thermoregulation
Supportive • Monitor fluids and electrolytes
Management • Beta agonists (e.g., salbutamol)
• Most cases resolve in 3-5 days
• Elective cesarean section (CS) scheduled at gestational age of
39 weeks or later
Prevention • Vaginal birth
• Establish fetal maturity prior to CS
• Antenatal betamethasone prior to elective CS at term
Source:GomellaTL,el al. Neonalolgy
7thEdition.McGraw-Hill:2013
KliegmanR, el al. NelsonTextbook
of Pediatrics
(21sted.).Elsevier;2020

294
PERSISTENT PULMONARY HYPERTENSION OF THE NEWBORN (PPHN)
• Marked pulmonary hypertension resulting from elevated pulmonary vascular resistance
and altered pulmonary vasoreactivity, leading to right to left extrapulmonary shunting of
blood across the foramen ovale and the ductus arteriosus
• Also referred to as Persistent Fetal Circulation

I. ETIOPATHOGENESIS
Failure of the normal circulatory transition after birth, due to high pulmonary vascular
resistance, secondary to:
Maladaptation from an acute injury
, Remodeling of pulmonary vasculature with vascular wall thickening and smooth
muscle hyperplasia
Pulmonary hypoplasia
Obstruction or any anomalies or abnormalities of pulmonary vessels
With inadequate pulmonary perfusion, neonates are at risk for developing refractory
hypoxemia, respiratory distress, and acidosis

RISK FACTOR EXAMPLES

• Meconium aspiration
• RDS
• Pneumonia
Lung disease • Pulmonary hypoplasia
• Cystic lung disease
• Diaphragmatic hernia
• Congenital alveolar capillary dysplasia
• Polycythemia
• Hypoglycemia
• Hypoxia, acidosis
Systemic disorders
• Hypocalcemia
• Hypothermia
• Sepsis

Congenital heart
disease
•
•
•
•
Total anomalous venous return
Hypoplastic left heart
Coarctation of the aorta
Enclocardial cushion defects
I
• Transposition of the great arteries
• Aortic stenosis
• Asphyxia
Perinatal factors • Perinatal hypoxia
• Maternal ingestion of aspirin or indomethacin

II. MANIFESTATIONS
Respiratory distress with cyanosis (hypoxemia) despite adequate ventilation
Marked !ability in oxygenation
Significant decrease in pulse oximetry with routine nursing care or minor stress
• Signs of heart failure

Ill. DIAGNOSIS
DIAGNOSTIC REMARKS/FINDINGS
Chest radiograph • May be normal (depends on comorbid conditions)
• Gold standard to confirm the diagnosis of PPHN
• Useful in identifying sites of extrapulmonary shunting
2D-echocardiography
and assessing right and left ventricular function (to guide
appropriate vasodilator therapy)

295
IV. MANAGEMENT
ASPECT MANAGEMENT
• Reduces pulmonary vascular resistance
Inhaled • Improves oxygenation by directing blood to better aerated distal air
Nitric Oxide spaces
• Improves V/Q mismatch
• Mechanical ventilation to ensure adequate oxygenation
Initial
Management
• Presser agents (e.g., dopamine) to maintain normal blood pressure and
increase cardiac output
• Careful and intensive monitoring
• Prevent aggravation of left to right shunting through adequate hydration
• Maintain normal serum glucose and calcium
Supportive
• Thermoregulation
• Avoid acidosis
• Sedation to address !ability with minor stress
• Adequate resuscitation and support from birth may prevent or
Prevention
ameliorate PPHN
Source:GomellaTL, et al. Neonatolgy 7thEdition.McGraw-Hill;2013.
KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia;
2020

MECONIUM ASPIRATION SYNDROME (MAS)

• Meconi um: the first intestinal discharge of a newborn
• With the passage ofmeconium in utero, the meconium-stained amniotic fluid (MSAF)
may be aspirated
• Most common in term, postmature infants, and infants who are small for gestational age

I. ETIOPATHOGENESIS
• After in utero passage of meconium (affected by fetal distress & vagal stimulation), deep
irregular respiration/gasping can cause aspiration of MSAF
• Distal progression into the airways of meconium stained fluid occurs in conjunction with
reabsorption of lung fluid
• Early consequences include:
Airway obstruction
° Chemical pneumonitis
Release of inflammatory mediators
Surfactant dysfunction
Increase in pulmonary vascular resistance and pulmonary hypertension

II. MANIFESTATIONS
• Mild to severe respiratory distress with cardiopulmonary failure
General
• Early onset respiratory distress in an infant with MSAF
• Proportional to length of meconium exposure & meconium concentration:
Meconium 0 Stained umbilical cord: 15 minutes exposure to thick MSAF or 1 hour
staining of exposure to lightly stained fluid
skin 0 Yellow stained nails: indicate 4-6 hours exposure
0 Stained vernix caseosa: indicate 12 hours exposure

• Respiratory distress, apnea, and/or cyanosis

If with • Poor air exchange
airway • Air-trapping and atelectasis
obstruction • Increased AP diameter of the chest
• Air-leak syndromes

296
III. DIAGNOSIS
DIAGNOSTIC REMARKS/FINDINGS
• Hypoxemia
• Respiratory acidosis due to airway obstruction/atelectasis/
Arterial blood gas pneumonitis
• Combined respiratory and metabolic acidosis seen in
concomitant perinatal asphyxia
• Hyperexpansion of the lungs with flat diaphragms and widened
rib spaces
• Widespread, coarse, asymmetric, and patchy infiltrates
• Areas of lung atelectasis (complete obstruction) flanked by
Chest radiograph
irregular areas of overexpansion (partial obstruction)
• Diffuse homogeneous ground-glass lung density (similar to
that seen in respiratory distress syndrome)
• Others: pneumothorax, pleural effusion

IV. MANAGEMENT
ASPECT MANAGEMENT
• Routine intubation to aspirate the lungs of vigorous infants is
not effective in reducing MAS
General measures • Depressed infants may benefit from endotracheal intubation
and suctioning to remove meconium in the airway before the
first breath (however, data for this is inconclusive)
• Administer oxygen for respiratory distress or decreasing
oxygen saturation
• Endotracheal intubation with assisted ventilation for moderate
to severe MAS

I
• Sedation for infants on mechanical ventilation (pain
Supportive
precipitates hypoxia and right-to-left shunting)
• Antibiotic coverage: meconium (though sterile) inhibits the
normally bacteriostatic quality of amniotic fluid
• ECMO (Extracorporeal membrane oxygenation) as final rescue
therapy
• Mild disease requires <40% oxygen for up to 48 hours
Oxygen • Moderate disease requires >40% oxygen for >48 hours
administration • Severe disease requires assisted ventilation for >48 hours
(associated with PPHN)
Source: GornellaTL, et al, Neonalolgy7th Edition.McGraw-Hill;2013.
KliegrnanR. et al. NelsonTextbookof Pediatrics(21sted.). Philadelphia:2020

297
BRONCHOPULMONARY DYSPLASIA (NEONATALCHRONIC LUNG DISEASE)
• Form of chronic lung disease that affects newborns (mostly premature) and infants resulting
from damage to the lungs caused by mechanical ventilation and long-term use of oxygen
• Persistent oxygen dependency up to 28 days of life
• Incidence is influenced by lung maturity (increased incidence with lower birth weight)

I. ETIOPATHOGENESIS
• Exaggerated inflammatory response
• Volutrauma/barotrauma during mechanical ventilation
• Hyperoxia due to prolonged exposure (>150 hours) to FiO2 >60%

II. MANIFESTATIONS
• Worsening respiratory status with increased work of breathing
• Increased oxygen requirement
• Apnea
• May have manifestations of heart failure
SEVERITY DESCRIPTION
Mild BPD • Infants who have been weaned from any supplemental oxygen
Moderate BPD • Infants who continue to need up to 30% oxygen
• Infants who need >30% oxygen or require CPAP/mechanical
Severe BPD
ventilation

III. DIAGNOSIS
DIAGNOSTIC REMARKS/FINDINGS
• CO2 retention
Arterial blood gas
• pH derangements
• Shows diffuse haziness and lung hypoinflation among pre-
matures with persistent oxygen requirements
Chest radio graph • Streaky interstitial markings, patchy atelectasis intermingled
with cystic areas, severe lung hyperinflation
• "Bubbly lungs"
• Done for non-improving or worsening BPD
ECGand 2D echo
• To detect car pulmonale and/or pulmonary hypertension
• Electrolyte abnormalities from chronic CO2 retention, diuretic
Laboratory
examination therapy, fluid restriction
• CBC:to rule out infection

IV. MANAGEMENT
ASPECT MANAGEMENT
• Ventilatory and nutritional support
• Control of infection
Supportive
• Diuretic therapy, fluid restriction
• Bronchodilators, corticosteroids, pulmonary vasodilators
• There remains a lack of effective intervention that prevents BPD
• Strategies include:
0 Early use of nasal CPAP (nCPAP)

Prevention 0Early surfactant therapy

° Caffeine to prevent apnea
0Vitamin A supplementation
0Systemic corticosteroids (not routine)

298
CONGENITAL DIAPHRAGMATIC HERNIA (CDH)
I. ETIOPATHOGENESIS
A. Two Types
TYPE DESCRIPTION

Bochdalek Hernia • Defect in the pleuroperitoneal canal through the foramen of

Bochdalek (posterolateral defect more commonly on left side)
(95%) • Mnemonic: "BACKDA LEFT"
Morgagni Hernia • Central anterior defect of the diaphragm

B. Pathophysiology
• At 10-12 weeks AOG,the abnormal communication between
the peritoneal and pleural cavities allows herniation of
Prenatal period intestine into the pleural space
• This results to underdevelopment (pulmonary atresia/
hypoplasia) of the lungs due to compression
• After delivery, the anomaly contributes to pulmonary
Postnatal period
parenchymal insufficiency

II. MANIFESTATIONSAND DIAGNOSIS

• Significant respiratory distress within the first few hours of life
• Depending on the size of hernia, some may be asymptomatic
Manifestations • Scaphoid abdomen
• Diminished breath sounds on the affected side
• On auscultation of the chest, bowel sounds maybe heard
• Bowel gas pattern in the hemithorax
Chest radio graph

lll. MANAGEMENT
ASPECT
• Shift of mediastinal structures to the other side

MANAGEMENT
I
• Intubation with positive pressure ventilation
• Replacement surfactant therapy
• Nasogastric tube to lessen gaseous distention of the stomach
Supportive
and intestine
• Positive pressure ventilation (e.g., CPAP) is avoided for CDHas
added pressure in the stomach further compresses the lungs
• Surgical correction to reduce intrathoracic intestine and closure
Surgery
of the diaphragm
Source:GomellaTL, et al. Neonatolgy7th Edition.McGraw-Hill; 2013.
KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia;
2020

299
CHOANAL ATRESIA
• Choana (i.e., back of the nasal passage) is blocked by an abnormal bony (90%) or
membranous septum (10%) due to failed recanalization of the nasal fossae

I. ETIOPATHOGENESIS
• Most common congenital anomaly of the nose with unknown pathogenesis (theoretically
due to the persistence of the buccopharyngeal membranes or failure of the oronasal
membrane to rupture)
• More common in females
Most have other congenital anomalies, such as:
CHARGEsyndrome (most common)
VACTERLsyndrome
Treacher-Collins syndrome
° Kallman syndrome

CHARGE SYNDROME VACTERLSYNDROME

• C oloboma • Vertebral defects
• H eart anomalies • lmperforate Anus
• A tresia (Choanal) • Cardiac defect
• R etarded growth • TracheoEsophageal fistula
• Genital abnormalities • Renal defect
• Ear abnormalities • Radial and Limb anomalies

II. MANIFESTATIONS
• Difficulty with mouth breathing (e.g., they would suck in their
lips and may develop cyanosis)
Bilateral choanal
• Those able to breathe through their mouths may have difficulty
atresia (40%)
sucking and swallowing at the same time (e.g., as they attempt
to feed, they may develop cyanosis)
Unilateral choanal • Presents with unilateral purulent nasal discharge and obstruction
atresia (60%) • History of chronic sinusitis

III. DIAGNOSIS
DIAGNOSTIC REMARKS
• Diagnosis is established by inability to pass a firm catheter
Clinical
through each nostril 3-4 cm into the nasopharynx
Fiberoptic rhinoscopy • Atretic plate may be seen directly
High-resolution CTscan • Can best evaluate the anatomical abnormalities

IV. MANAGEMENT
ASPECT MANAGEMENT
• Prompt placement of an oral airway, maintain the mouth in an
Initial treatment open position, or intubation
• Bilateral cases: intubation or tracheotomy may be indicated
• Trans-nasal repair: operative intervention for neonates without
other serious medical problems
Surgery • Tracheotomy: for those with bilateral atresia and other
potentially life-threatening problems
• Elective surgery: for those with unilateral obstruction
Source:KliegmanR.et al. Nelsontextbookof pediatrics(21sted.).Canada:Elsevier
AndaloraC el al. ChoanalAtresia.In:Sta!Pearls;
2018

300
 SECTION THREE
DISORDERS OF THE UPPER RESPIRATORY TRACT

RHINITIS (THE COMMON COLD)

I. ETIOPATHOGENESIS
• Many viruses that cause rhinitis are also associated with cough, wheezing, and fever
• Young children have an average of 6-8 colds per year
• Incidence of infection is primarily a function of exposure to the virus

ETIOLOGIC AGENTS MECHANISMS OF SPREAD

• Rhinovirus (more than 50%) • Direct hand contact
• Coronavirus • Inhalation of small-particle aerosols (especially
• Respiratory syncytial virus (RSV) of influenza virus & coronavirus)
• Human metapneumovirus • Deposition of large-particle aerosols from
• Adenovirus sneezing that land on nasal or conjunctiva!
• Parainfluenza mucosa

II. MANIFESTATIONS
• Predominant in infants: nasal discharge and fever
• Sore throat, sneezing, nasal obstruction, rhinorrhea, irritability, decreased
appetite
• Cough usually begins after the onset of nasal symptoms and may persist for
Symptoms another 1-2 weeks after resolution of other symptoms
• Fever is more likely with influenza virus, RSV,human metapneumovirus,
and adenovirus
• Colds usually persist for 1 week

I
• Onset of symptoms typically occurs 1-3 days after viral infection
• Swollen, erythematous nasal turbinates
• Abnormal middle ear pressure
Signs • Anterior cervical lymphadenopathy or conjunctiva! injection
• Change in color of nasal secretion may indicate accumulation of
inflammatory cells and not necessarily bacterial superinfection

III.DIAGNOSIS
• Usually a clinical diagnosis
• Exclude conditions that are potentially more serious (e.g., sinusitis, foreign body)

IV. MANAGEMENT
• Supportive: topical nasal saline or saline nasal irrigation to reduce the symptoms
• Non-prescription cough & cold products should not be used for children <6 years old
• Complications: otitis media, sinusitis, pneumonia

Source:KliegmanR,et al. Nelsontextbookof pediatrics(21sted.).Canada:Elsevier

LowryJA,et al. Over-the-counter
medications:
updateoncoughandcoldpreparations. PediatrRev;2015

301
SINUSITIS
I. ETIOPATHOGENESIS
A. Etiology
S. pnewnoniae (30%)
Nontypeable H. inf/llenzae (20%)
0 M. catarrhalis (20%)
Anaerobes and Staphy/ococcl/s aureus [uncommon)

8. Pathophysiology
0 Anything that impairs mucociliary transport or causes nasal obstruction
predisposes to sinusitis (e.g., viral URTI, allergic rhinitis, cigarette smoke exposure)

C. Classification of Sinusitis
CLASSIFICATION DURATION
Acute sinusitis • < 30 days

Subacute sinusitis • 1-3 months

Chronicsinusitis • >3 months

II MANIFESTATIONS
• Nasal congestion, purulent nasal discharge, feve1;and cough
• Maxilla1y tooth discomfort and pain or pressure exacerbated by bending
Symptoms
forward
• Headache and facial pain are rare in children
• Erythema and swelling of the nasal mucosa with purulent nasal
discharge
Signs
• Sinus tenderness detectable in adolescents
• Transillumination shows an opaque sinus that transmits light poorly
• Periorbital cellulitis
• Orbital cellulitis (proptosis, chemosis, decreased visual acuity,
diplopia, impaired EOMs, eye pain)
• Meningitis
Complications • Cavernous sinus thrombosis
• Brain abscess
• Pott puffy tumor [osteomyelitis of the frontal bone)
• Mucoceles (chronic inflammatory lesions in the frontal sinuses that
expand and displace the eye)
Source:KliegmanR.el al. Nelsontextbookof pediatrics(21sted.).Canada:Elsevier
MorcomS. et al. Sinusitis.AustFamPhysician:
2016

III. DIAGNOSIS
• Clinical diagnosis is based on histo1y:
0 Persistent nasal discharge & cough >10 days without improvement
Clinical
0 Temperature of at least 39°C
0 Purulent nasal discharge for 3-4 consecutive days
• Findings (not diagnostic):
0 Air-fluid levels

Para nasal 0 Opacification of the sinuses

Radiograph 0 Mucosa! thickening

• These findings confirm sinus inflammation, but cannot differentiate

viral, bacterial, or allergic causes of inflammation

302
IV. MANAGEMENT
ASPECT MANAGEMENT
• Indicated for acute bacterial sinusitis with severe onset or a
worsening course
• Promotes resolution of symptoms & prevent suppurative complications
• Uncomplicated mild/moderate acute sinusitis: amoxicillin 45 mg/
kg/day BID for 10 days (or 7 days after resolution of symptoms)
• Alternatives if allergic to penicillin: cefuroxime, cefixime
Antibiotics • Amoxicillin-clavulanate 80-90 mg/kg/day for children with the
following risk factors:
0 Previous antibiotic treatment (past 1-3 months)
0 Daycare attendance

0 Age <2 years old

° Failure to respond to initial therapy with amoxicillin within 72 hours
0 Severe sinusitis

Referral to • When there is failure to respond to the above antibiotic regimen

Otolaryngologist • For consideration of surgical management
R.et al. Nelsontextbook
Source:Khegman of ped1atncs {21sted.).Canada:Elsevier
AndaloraC et al. ChoanalAtresia.In: StatPearls:2018

ACUTE PHARYNGITIS
I. ETIOPATHOGENESIS
• Inflammation of pharynx manifesting as erythema, edema, exudates, or enanthem
• Most of the pathogens are listed below
VIRUSES BACTERIA
• Adenovirus • Group A beta-hemolytic streptococcus or GABHS
• Coronavi rus (Strep. pyogenes): most common bacterial pathogen
• Cytomegalovirus • Fusobacterium necrophorum
•
•
•
Enterovirus
Epstein-Barr virus
Rhinovirus
• Corynebacterium diphtheriae
• Chlamydia trachomatis
• Chlamydophila pneumoniae
Source:Kliegman
R, et al. Nelsontextbook
of pediatrics
(21sted.).Canada:Elsevier
I
II. MANIFESTATIONS
VIRAL BACTERIA (due to GABHS)
• Gradual onset • Sore throat and fever
• Moderate throat pain • Headache, vomiting, abdominal pain
• Symptoms of viral URTI (e.g., • No URTI symptoms
conjunctivitis, coryza, cough) • Palatal petechiae
• Contacts with cold symptoms • Tonsillar exudate
• Vesicles and ulcers (HSV) • Scarlatiniform rash
• Conjunctivitis (adenovirus) • Swollen, tender anterior cervical nodes
Source:Shaikhet al. Journalof Pediatrics:2012

III. DIAGNOSIS
DIAGNOSTIC REMARKS
Throat culture • Gold standard for diagnosing GABHS pharyngitis

• For diagnosis of GABHS pharyngitis

• Not readily available in our setting
Rapid antigen
• All RADTs have generally >95% specificity
detection test
0 When RADT is positive: throat culture is unnecessary
(RADT) 0 If rapid strep test is negative: do a throat culture
0 RADTs are less sensitive than culture

303
IV. MANAGEMENT
ASPECT MANAGEMENT
• Amoxicillin SO mg/kg PO x 10 days
• Penicillin or amoxicillin
• Penicillin 250 mg (if <27 kg) or 500 mg
for 10 days
(if;,27 kg) PO twice a day x 10 days
• Erythromycin 40 mg/kg/day PO twice a
day x 10 days (max: 1 g/day)
• Azithromycin 12 mg/kg on day 1; then 6
mg/kg on days 2-5 PO once a day (max:
• Treatment options if
500 mg/day)
allergic to penicillin
Antibiotics for • Clarithromycin 15 mg/kg/day twice a day
GABHS x 10 days (max: 500 mg/day)
• Clindamycin 20 mg/kg/day PO thrice a
day x 10 days (max: 1.8 g/ day)
• With a clinical diagnosis of scarlet fever
• With a household contact with
• Start antibiotics
documented Streptococcal Pharyngitis
immediately even
• With a past histmy of acute rheumatic fever
without culture if
• With a recent history of acute rheumatic
fever in a family member
• Considered for recurrent streptococcal pharyngitis, defined as culture
positive streptococcal pharyngitis that has been severe and frequent
(Paradise Criteria):
Tonsillectomy 0 ;,7 episodes in the previous year, or
0 ;,5 episodes in each of the preceding 2 years, or
0 ;,3 episodes in each of the preceding 3 years

V. COMPLICATIONSOF GABHS:
• Rheumatic fever
• Post-streptococcal glomerulonephritis
• Peritonsillar / retropharyngeal abscess
Sources:KliegmanR, et al. Nelsontextbookof pediatrics(21sted.).Canada:Elsevier
Shaikhet al. Journalof Pediatrics;2012
CDC& Prevention. GroupA Streptococcal (GAS)Disease

PERITONSILLAR ABSCESS
I. ETIOPATHOGENESIS
• Bacterial invasion through the capsule of the tonsils
• Usually affects adolescents
• Etiology: Group A streptococcus and mixed anaerobes
II. MANIFESTATIONS
• Fever, sore throat, dysphagia, odynophagia, drooling, trismus
• Asymmetric tonsillar bulge with a displaced uvula (diagnostic PE finding)

Ill. DIAGNOSIS
• Diagnosis is mainly clinical
• CT scan: helpful for showing the abscess

IV. MANAGEMENT
• Majority resolve after surgical drainage (I & Dor needle aspiration) and antibiotics
• Consider tonsillectomy when:
° Failure to improve within 24 hours of antibiotics and I & D
0 History of recurrent tonsillitis
° Complications from peritonsillar abscess

Source:KtiegmanR, et al. Nelsontextbookof pedia!Jics

(21sted.).Canada:Elsevier
BochnerRE,et al. PediatrRev;2017
304
RETROPHARYNGEALABSCESS
I. ETI0PATH0GENESIS
A. Epidemiology
Most commonly occurs in <3-4 years old
More common in males
About 2/3 have a history of recent eat; nose, or throat infection

B. Pathophysiology
The retropharyngeal space is located between the pharynx & the cervical vertebrae
& extending down into the superior mediastinum
Infection of the retropharyngeal nodes usually occurs due to extension of a localized
infection of the oropharynx
° Can also result from penetrating trauma to the oropharynx, dental infection, and
vertebral osteomyelitis

C. Etiology
Group A streptococcus
Anaerobes
0 Staphylococcus aureus
Others: Klebsiella and Haemophi/us influenza

II. MANIFESTATIONS
• Fevet; irritability, decreased oral intake, drooling
• Neck stiffness or refusal to move the neck
• Limitation of neck extension, bulging of the posterior pharyngeal wall, muffled voice,
strider, respiratory distress

Ill. DIAGNOSIS
DIAGNOSTIC REMARKS
• Incision & drainage and culture of an abscessed node provides
Culture

I
the definitive diagnosis
• Can accurately identify abscess formation in majority of cases
• Findings include:
CT scan with contrast ° Central lucency
0 Ring enhancement

0 Scalloping of lymph node walls

Soft tissue neck

• Increased width or an air-fluid level in the retropharyngeal space
radiograph

IV. MANAGEMENT
ASPECT MANAGEMENT
• IV antibiotics with or without surgical drainage
Antibiotic Therapy • 3" 1 generation cephalosporin with ampicillin-sulbactam or
clindamycin
• Drainage is necessary in patients with respiratory distress or
Drainage
failure to improve with IV antibiotics

• Watch out for complications such as:

0 Significant upper airway obstruction
Anticipatory Care
0 Rupture leading to aspiration pneumonia
0 Extension to the mediastinum

Source:KliegmanR,et al. Nelsontextbookof pediatrics(21sted.).Canada:Elsevier:

BochnerRE,et al. PediatrRev:2017

305
LARYNGOTRACHEOBRONCHITIS (VIRAL CROUP)
I. ETIOPATHOGENESIS
• Acute inflammatory disease of the larynx (within the subglottic space)
Most common etiology is parainfluenza virus
• Affects children 3 months to 5 years old (peak incidence at 2 years old)
• More common in males
• Most common form of acute upper respiratory obstruction in children
II MANIFESTATIONS
• URTI and fever 1-3 days prior to upper airway obstruction
• "Barking cough"
Symptoms
• Hoarse voice
• Symptoms worse at night
• Coryza & rhinorrhea
• Normal to moderately inflamed pharynx
Signs
• Slight tachypnea
• lnspiratory stridor

III. DIAGNOSIS
A. Westley Croup Score (diagnosis of croup is mainly clinical)
SIGN DEGREE SCORE
• None 0
Stridor • At rest on auscultation 1
• At rest without auscultation 2
• None 0
• Mild 1
Chest wall retractions
• Moderate 2
• Severe 3
• Normal 0
Air entry • Decreased 1
• Severely decreased 2
• None 0
Cyanosis • With agitation 4
• At rest 5
• Normal 0
Consciousness level
• Altered 5
Interpretation of Results (total score can be 0-17 points):
• Mild: :s2
• Moderate: 3-7
• Severe: equal or >8
• Impending respiratory failure: equal or >12
Source:WoodsC. et al. Croup:Clinicalfeatures,evaluation,
anddiagnosis.
UpToDate:
2018

B. Findings on Neck Radiograph

0 Subglottic narrowing or "steeple sign"
0 X-rays do not correlate well with disease severity

IV. MANAGEMENT

• No stridor at rest
• Severe disease: poor air entry, altered
• Normal oxygen saturation
consciousness
• Good air exchange
• Deterioration even after initial medical management
• No cyanosis
• Severe dehydration
• Normal level of consciousness
• Features suggesting secondary bacterial infection
• Able to tolerate fluids by mouth
306
 B. Management of Croup based on Severity
SEVERITY INTERVENTION*
Mild • Dexamethasone 0.6 mg/kg/dose PO/IV /IM (max dose: 16 mg)
• Dexamethasone 0.6 mg/kg IV/IM
• Humidified oxygen (if02 saturation <92%)
Moderate to
• Give nothing by mouth
Severe
• Nebulize epinephrine over 15 minutes (0.5 mL/kg/dose, max 5 mL,
using 1:1000 dilution)

Impending • High concentration of oxygen (nonrebreathing mask preferred)

Respiratory • Dexamethasone IV/IM
Failure • ET intubation as indicated (use smaller ET tube size)
'It is stronglyrecommendedthat a singledose of glucocorticoids
be administeredto childrenpresentingto the
emergencydepartmentwithcroup
Sources:2011CPGon Croup,Level1A,CPSGuidelines2017
TovarPaduaLJ, et al. FeiginandCherry'sTexlbookotPedialricInfectiousDiseases,8thed; 2019

ACUTE EPIGLOTTITIS (SUPRAGLOTTITIS)

I. ETIOPATHOGENESIS
Serious, acute, rapidly progressive infection of supraglottic structures
• Affects children 1-7 years old
• Most common etiology:
H. influenzae type bin unvaccinated children
0 Streptococws pyogenes, S. pneumoniae, and S. aureus in vaccinated children

II. MANIFESTATIONS

• Acute onset of high fever, sore throat, dyspnea, rapidly progressing

respiratory obstruction
Symptoms
• Within hours, children develop dysphagia, drooling, hyperextended neck

Signs
to maintain the airway, muffled voice, "sniffing dog" or "tripod" position
• Direct visualization of inflamed cherry-red epiglottis under double set-up
( examine the throat only under double set-up because of risk for airway
obstruction and respiratory arrest)
I
III. DIAGNOSIS
DIAGNOSTIC REMARKS
• Blood, epiglottic surface, CSF (in some cases)
Cultures
• Done once airway is stabilized
Lateral neck
• Findings: "thumb" or "leaf" sign
radiograph
Fiberoptic
• Provides direct visualization of inflamed cherry-red epiglottis
laryngoscopy

IV. MANAGEMENT
• Secure airway (intubation or tracheostomy) under double set-up
• IV antibiotics (cefotaxime, ceftriaxone, or meropenem) for 7-10 days

V. PROPHYLAXIS:
• Indications for rifampicin prophylaxis (20 mg/kg orally once a day for 4 days with
maximum dose of600 mg/dose) for all household members with:
Any contact <4 years old who is incompletely immunized
Any contact <12 months old who has not received the primary vaccination series
0 An immunocompromised child in the household
Source:GuerraAM,et al. Epiglottitis.In: StatPearls.
Fl: StatPea~sPublishing;
2018
307
SUMMARY TABLE FOR EPIGLOTTITIS VS CROUP

FEATURE CROUP EPIGLOTTITIS

Age • Younger children • Older children
Narrowing • Subglottic • Supraglottic
Stridor • 88% •8%
Pathogen • Parainfluenza • H. influezae type B
Onset • Prodrome (1- 7 days) • Rapid onset (4-12 hours)
Fever • Low grade • High grade
Assosciated symptoms • Barking cough, hoarseness • Muffled voice, drooling
Response to racemic
• Improvement of stridor • None
epinephrine
CXR • "Steeple sign " • "Thumbprint/leaf sign"
Management • Supportive • Secure airway, antibiotics
Source:Kliegman R. et al. Nelsontextbook of pediatrics(21sted.).Canada:Elsevier
GuerraAM.el al. Epiglollilis.In: Sta!Pearls.Fl: Sla!PearlsPublishing:2018
2011CPGon Croup,Level1A,CPSGuidelines2017
TovarPaduaLJ.et al. FeiginandCherry's Textbook of PediatricInfectious Diseases.8thed:2019

BACTERIAL TRACHEITIS
• Most common cause is S. aureus
• Others:
• MRSA
0 S. pneumoniae
Etiopathogenesis 0 S. pyogenes
• M. catarrhalis
• Nontypeable H. influenza
0 Anaerobes
• Often follows a viral respiratory infection
• Brassy cough
• High fever and "toxicity" can occur immediately or after a few days
Manifestations of apparent improvement
• Can lie flat, does not drool, no dysphagia
• Mucosa I swelling at the level of the cricoid cartilage with copious,
thick, purulent secretions
• Based on bacterial upper airway disease (high fever, purulent airway
secretions, absence of classic findings of epiglottitis)
Diagnosis
• Neck radiograph: ragged tracheal air column, pseudomembrane
detachment in the trachea (lateral view)
Management • Vancomycin or clindamycin and a 3"' generation cephalosporin
Source:KliegmanR. el al. Nelsontextbookof pediatrics(21sted.).Canada:Elsevier

308
 SECTION FOUR
DISORDERS OF THE LOWER RESPIRATORY TRACT

BRONCHIOLITIS
I. ETIOPATHOGENESIS
• Acute inflammation of the small airways in children <2 years old resulting in bronchiolar
obstruction with edema, mucus, and cellular debris
• Most commonly caused by RSV
• More common in:
Boys
Those who have not been breastfed
Those who live in crowded conditions
Infants with mothers who smoked during pregnancy

II. MANIFESTATIONS
• Child with coryzal prodrome lasting 1-3 days followed by persistent cough,
Symptoms tachypnea, and presence of wheezing/crackles
• Low-grade fever, rhinorrhea, cough
• Tachypnea, wheeze/crackles
Signs • Hyperresonance to percussion
• Prolonged expiratory phase

III. DIAGNOSIS
• Routine tests are not necessary
• Chest radiograph (not routinely indicated in uncomplicated cases): shows hyperinflation
with patchy atelectasis

IV. MANAGEMENT
A Management Based on Severity
SEVERITY

Mild
• May be managed at home
• Increase fluids
MANAGEMENT*

• Trial of bronchodilator not recommended (does not alter the course)

I
• Ventilatory & oxygen support (cool humidified 0 2 for hypoxemic patients)
• Hydration
Severe • Do not routinely suction: consider upper airway suction for children
with respiratory distress or feeding difficulties due to upper airway
secretions, and children with apnea
'The followingare not routinelyrecommended:bronchodilators,chest physiotherapy,corticosteroids

B. Indications for Admission

Marked respiratory distress (nasal flaring, retractions, tachypnea, dyspnea, cyanosis)
Age <12 weeks
Toxic appearance, poor feeding, lethargy, dehydration
Apnea
Oxygen saturation <92%
History of prematurity
Underlying cardiopulmonary, neurologic, or immunologic disease
Unreliable caregivers

C. Prevention
Palivizumab (IM monoclonal antibody) for children <2 years with congenital heart
disease (CHD) and chronic lung disease (CLD), and history of prematurity
0 Hand hygiene practices
Exclusive breastfeeding for at least 6 months
Sources:NICEGuidelines
on Bronchiolitis
2015
AAPBronchiolitis
Guidelines2014
309
ACUTE BRONCHITIS
I. ETIOPATHOGENESIS
Inflammation of the large and medium-sized airways of the lungs
Tracheobronchial epithelium is invaded by the infectious agent that leads to activation of
inflammatory cells and release of cytokines
• Often follows a viral upper respiratory tract infection

II. MANIFESTATIONS
• Nonspecific URTI symptoms
Natural History • After 3-4 days, frequent dry, hacking cough
(entire course • Aher several days, sputum becomes purulent from leukocyte migration
lasts ~2-3 weeks) • Chest pain exacerbated by coughing may be prominent in older children
• Mucus gradually thins within 5-10 days and cough abates
• Early signs: low-grade feve1; nasopharyngitis, conjunctivitis, rhinitis
Signs
• Late signs: coarse breath sounds with crackles & scattered wheezing

III. DIAGNOSIS {mainly clinical)

• Principal goal is to exclude pneumonia which requires antibiotic therapy (absence of
tachycardia, tachypnea, fever, & a normal chest PE reduce likelihood of pneumonia)
• Chest radiograph: may be normal or can have increased bronchial markings

IV.MANAGEMENT
• No specific treatment and it is self-limited
• Antibiotics do not hasten improvement
• Use of humidifiers do not shorten the course
Source:KliegmanR, et al. Nelsontexlbookof pediatrics(21sted.).Canada:Elsevier
SinghA. et al. AculeBronchilis.In: StatPearls.FL: SlalPearlsPublishing

PNEUMONIA
I. ETIOPATHOGENESIS
• Inflammation of the lung parenchyma
• Most commonly viral etiology in childhood
A.Etiology
AGE GROUP FREQUENT PATHOGENS (in order of frequency)
• Group B streptococcus
•£.coli
Neonates
• Listeria
(0-28 days)
• S. pneumoniae
• H. influenzae*
• Respiratory syncytial virus (RSV)
• Parainfluenza
• Chlamydia
3 weeks to
• Mycoplasma pneumoniae
3 months
• S. pneumoniae
• H. influenzae
• S. aureus
• RSV and other viruses
4 months to • S. pneumaniae
4 years • H. influenzae type b
• Mycoplasma pneumoniae
• Mycoplasma pneumoniae
5 to 15 years • S. pneumonia
• Chlamydaphila pneumoniae
'H. influenzae
typebis uncommon
withroutineH.inftuenzae
typeb immunization
• Themostcommoncauseof pneumonia acrossall agegroupsareviruses
• S. pneumoniaeis the mostcommonbacterialcauseof pneumoniaacrossall agegroup

310
 B. Classification of Pneumonia According to Location
CLASSIFICATION DESCRIPTION
Pneumonitis • Inflammation of the interstitium
Lobar pneumonia • With consolidation of one or more lobes
Bronchopneumonia • Inflammation of the bronchioles with mucopurulent exudate
Source:KliegmanR.el al. Nelsonlextbookof pediatrics(21sled.).Canada:Elsevier

II. MANIFESTATIONS
A Overview of the Manifestations
• Clinical triad: feve,; cough, and tachypnea
Symptoms
• Abdominal pain is common in lower lobe pneumonia
• Tachypnea is the most consistent clinical manifestation
• Early: diminished breath sounds, scattered crackles and rhonchi
• Later: dullness on percussion & lag in respiratory excursion on
Signs
affected side (from consolidation and/or pleural effusion)
• Signs of respiratoty failure in severe cases: retractions, head
bobbing, cyanosis, grunting, apnea, and changes in sensorium

Recurrent • Defined as :c:2episodes in a single year or :c:3episodes overall,

pneumonia with radiographic clearing between occurrences

B. Differentiation between Types of Pneumonia by Etiology:

VIRAL BACTERIAL MYCOPLASMA
CHLAMYDIA
PNEUMONIA PNEUMONIA (walkingpneumonia)

• "Staccato"
• Cough cough of
• Cough • High fever • Less ill-looking insidious
Manifestations • Wheezing • Dyspnea • Non-productive onset
• Stridor

• WBC normal
• Dullness to
percussion
cough • Maternal
history of
infection
• WBCusually
I
• WBC count
or mildly • Leukocytosis normal
CBC usually
elevated • Neutrophilia • Eosinophilia
normal
• Lyrnphocytosis
• Pneumococcal:
confluent lobar
consolidation
• Hyperinflation • Staphylococcal: • Interstitial
• Bilateral irregularareas infiltrates • Hype1inflation
Chest interstitial of cavitation lead • Most • Interstitial
Radiograph infiltrates to pneumatocele, common in infiltrates
• Peribronchial empyema, or lower lobes
cuffing bro11d1opulmonary
fistula
• Large pleural
effusion
• SeePCAP • Erythromycin
Management • Supportive management • Clarithromycin
below • Azithromycin
Source:KliegmanR. et al. Nelsontexlbookof pedialrics(21sted.).Canada:Elsevier
PuljizI. et al. ChlamydiapneumoniaeandMycoplasma pneumoniae pneumonia. Epidemiollnfecl:2006

311
III. CLASSIFICATION BASED ON PAPP (2016) AND WHO GUIDELINES
I
PAPP Classification* I PCAPA I
PCAP BI PCAPC PCAPD
I

WHO Classification* I NON-SEVERE I SEVERE VERY SEVERE

I I i
Clinical

Dehydration None I Mild Moderate Severe

Malnutrition None Moderate Severe

Pallor None Present Present

Respiratory rate (per min]

• 3-12 months 50-60 >60 to 70 >70
• 1-5 years 40-50 >50 >50
• 2'5 years 30-35 >35 >35

Signs of failure:
• Retraction None IC/SC Supraclavicular/lC/SC
• Head bobbing None Present Present
• Cyanosis None Present Present
• Grunting None None Present
• Apnea None None Present
• Sensorium None Irritable Lethargic/com a tose

Diagnostic Aids

Radiographic findings•• None Present Present

0 2 saturation (room air)*** 95% <95% <95%

Management

Site of care Outpatient Ward Critical care facility

Specifics See Management Recommendations below

*In order to classify to higher risk, at least 2 variables should be present

*'Chest radiograph findings include any of the following:effusion, abscess, air leak, lobar consolidation
*"0 2 saturation using pulse oximetry
IC:intercostal SC:subcostal

IV. DIAGNOSTICS BASED ON PCAP CLASS

TYPE THE FOLLOWING TESTS MAY BE REQUESTED
PCAPA* • Chest radiograph
• Oxygen saturation by pulse oximetry
PCAP B* • Gram stain, aerobic culture and sensitivity (CS) of sputum
• Chest ultrasound

• Oxygen saturation using pulse oximetry

• ABG to assess gas exchange
PCAPC • Chest radiograph
• Blood work-up: CBC (for WBC count), CRP, procalcitonin
• Chest ultrasound or radiograph: if with clinical suspicion of multi-lobar
consolidation, necrotizing pneumonia, lung abscess, pleural effusion, air leak
• To determine etiology for PCAP C or D with lung abscess, empyema, or
PCAPD pneumothorax: do GS/CS of sputum, nasopharyngeal aspirate, tracheal
aspirate, pleural fluid, and/or blood (may consider doing anaerobic cultures)
'For PCAPA or B, !here is no benefitfor doing bloodculture,WBCcount, CRP,procalcitonin
Source:2016PAPPGuidelines
on PCAP

312
IV. MANAGEMENT
CLASS MANAGEMENT*
• For those without previous • Oral amoxicillin 40-50 mg/kg/day in 3
antibiotic regardless of doses for 7 days (max 1500 mg/day); but
immunization status against may be given for a minimum of 3 days, or
Hib or S. pneumoniae in 2 divided dose for a minimum of 5 days)
PCAPA&
• Azithromycin 10 mg/kg/day OD for 3 days
PCAP B • If with known
(max: 500 mg/day) or 10 mg/kg/day on
hypersensitivity to
day 1 then 5 mg/kg/day on days 2-5
amoxicillin or suspicion of
(max: 500 mg/day)
atypical organisms especially
• Clarithromycin 15 mg/kg/day in 2 doses
Mycoplasma pneumoniae
for 7 days (max: 1000 mg/day)
• No previous antibiotic use
• Penicillin G 100,000 units/kg/day every
requiring hospitalization &
6 hours
with complete HiB vaccination
• With incomplete or unknown
• Ampicillin 100 mg/kg/day every 6 hours
PCAPC status of HiB vaccination
• IV non-antipseudomonal beta-lactam
(beta-lactam with beta-lactamase inhibitor,
• lf>l5 years old, can be cephalosporin, or carbapenem) plus
treated as adult extended macrolide or fluoroquinolone
• Example: Ceftriaxone 2 g IV OD for 7 days
plus Azithromycin 500 mg OD 3-Sdays
PCAPD • Refer to a specialist
'For patientssuspectedto have MRSA:startvancomycinand referto specialist
'If withoutclinicalresponsewithin72 hours,re-evaluateand considershiftingof antibiotics
Source:2016PAPPGuidelines
on PCAP

I
PhilippineCPG:Diagnosis,EmpincManagement
andPrevention
of CAPin lmmunocompetent
Adults2016Update.

PULMONARY TUBERCULOSIS (PTB)

I. ETIOPATHOGENESIS
A. Pathophysiology
° Children usually infected by an adult or an adolescent in the immediate household
0 Usually involves the lung parenchyma and tracheobronchial tree
° Contributory factors in the environment increase the probability of transmission
such as residing in areas with poor ventilation
0 Patient's immune system plays an important role in development of disease
, Children <5 years old & immunocompromised individuals are more susceptible

8. Mycobacterium tuberculosis (Etiology)

Transmission • Inhalation of airborne mucus droplet nuclei
• Extensive upper lobe infiltrate or cavity
Factors increasing • Copious sputum production
transmission • Severe and forceful cough
• Poor air circulation
Incubation Period • 3 months to 2 years

313
II. MANIFESTATIONSOF PTB DISEASE
• Refers to the initial stage in children who inhale the
Mycobacterium tubeculosis bacilli
• The following three elements are present:
Ghan focus
0
Primary disease
Lymphadenitis
0

Lymphangitis
0

• Asymptomatic in up to 65% of children

• Low-grade fever can last for an average of 14-21 days
• Develops when initial infection fails to heal and continues to
Progressive Primary
progress over a period of months or years leading to further
Tuberculosis
pulmonary and other distant organ involvement
• Refers to a reactivation of an old, possibly subclinical infection
Secondary • Tends to produce more damage to the lungs
(Reactivation)
Tuberculosis • Occurs in <10% of the cases of primary infection
• More common in adolescents
• Due to hematogenous dissemination of Mycobacterium tuberculosis
• Denotes all forms of progressive, widely disseminated
Miliary Tuberculosis hematogenous tuberculosis
• Clinical presentation depends on the load of organisms and
sites where they lodge
Source:National
Tuberculosis
Control
Program:
ManualofProcedures.5thed.2014
Fontanilla,
JM,elal.Clin.Infect
Dis.2011
Galimi,
R.Europe
RevMedPharmacol
Sci.2011Apr:15(4): 365-66
Ill. DIAGNOSIS
A. Spectrum of TB Exposure, Infection, and Pulmonary Disease {modified from DOH/NTP Table)
TB EXPOSURE TB INFECTION TB DISEASE
Exposure Positive Positive Positive
Signs and symptoms Negative Negative Positive
Tuberculin skin test' Negative Positive Positive
Chest radiograph• Negative Negative May be positive
Direct sputum
Negative Negative May be positive
smear microscopy'
Other diagnostics Negative May be positive May be positive
a. TSTinTBinfectionand TBdisease:maybe falsenegativein manychildren
b. ChestX rayinTBdisease mayshowhilaradenopalhyand the Ghancomplex
c. DSSMmaybe negativedue to paucibacillary naturein childrenand difficulty
of specimencollection
Otherdiagnostic tests maybe positivesuchas IGRA(interferon-gamma releaseassay),an immunologic-based
test thatis fairlyspecificto M. tuberculosisbutitcannotdistinguishbetweenlatentinfectionanddisease. Others
includesputum/gastric AFBsmear,TBPCR,TBculture,nucleicacidamplification test (NAAT)
(e.g.,TBGeneXpert)
Source:
National
Tuberculosis
Control
Program:
ManualofProcedures.
5thed.2014
Training
Modules
forTBinChildren.
2008.Department
ofHealth/National
TBProgram

B. Classification of PTB
Bacteriologically- • Biological specimen is positive by smear microscopy, culture,
Confirmed or rapid diagnostic tests (such as Gene Xpert MTB/RIF)
• Child has been diagnosed with active TB by a medical
Clinically- practitioner who has decided to give the patient a full
Diagnosed course of TB medications, but does not fulfill the criteria for
bacteriologic confirmation

314
C. Presumptive Tuberculosis
Refers to any person with signs & symptoms suggestive of TB (whether pulmonary
or extra pulmonary), or those with radiographic findings suggestive of active TB
, Identification of "Presumptive TB" is summarized in the table below

1) For patients 15 years old and above, a presumptive TB has any of the following:
a. Cough of at least 2 weeks duration with or without the following symptoms:
' Significant and unintentional weight loss
' Fever
' Bloody sputum
' Chest/ back pains not referable to any musculoskeletal disorder
' Easy fatigability or malaise
' Night sweats
' Shortness of breath or difficulty of breathing

b. Unexplained cough of any duration in:

' A close contact of a known active TB case
' High-risk clinical groups (e.g., HIV-AIDS,diabetes, end-stage renal disease,
cance1; connective tissue disease, autoimmune disease, patients who
underwent solid organ transplantation, patients on prolonged systemic steroids)
' High-risk populations (e.g., elderly, urban poo1; inmates and other congregate
settings)

2) For patients below 15 years old, a presumptive TB has any of the following:
a. At least 3 of the following clinical criteria:
' Coughing/ wheezing of2 weeks or more, especially if unexplained
' Unexplained fever of 2 weeks or more after common causes have been excluded
' Loss of weight/ failure to gain weight/ weight faltering/ loss of appetite
' Failure to respond to 2 weeks of appropriate antibiotic therapy for lower
respiratory tract infection
' Failure to regain previous state of health 2 weeks after a viral infection or exanthem
' Fatigue, reduced playfulness, or lethargy
b. Any one (1) of the above in a child who is a close contact of a known active TB case

3) Radiographic findings suggestive of PTB, with/without symptoms, regardless of age I

Source:DOH.NalionalTuberculosis
ControlProgramManualof Procedures.
(5thed.).Manila;2014
Evidence-Based
ClinicalPracticeGuidelines.
ChildhoodTuberculosis.
Philippine
PediatricSociety;2008

D.Criteria for Diagnosis of Tuberculosis

A positive culture with or without a positive smear for M. tuberculosis is the gold
standard for the diagnosis of TB and must be sought for whenever possible
A child is presumed to have active TB if three or more of the following criteria are present:

1) Exposure to an adult/ adolescent with active TB disease (epidemiologic)

2) Signs and symptoms suggestive of TB (clinical)
3) Positive tuberculin skin test (immunologic)
4) Abnormal chest radiograph suggestive of TB (radiologic)
5) Laboratory findings suggestive of TB (e.g., histological, cytological, biochemical,
immunologic, and/or molecular) (laboratory)

315
IV. MANAGEMENT

• Pulmonary TB, new (whether bacteriologically-

confirmed or clinically-diagnosed)
I • Extra-pulmonary TB, new (whether
bacteriologically-confirmed or clinically-
2HRZE / 4HR

diagnosed) except CNS/ bones or joints

• Extra-pulmonary TB, new

Ia (CNS/ bones or joints)
2HRZE / l0HR

• Pulmonary or extra-pulmonary, previously

treated drug-susceptible TB (whether
bacteriologically-confirmed or clinically-diagnosed)
• Relapse
II • Treatment after failure
2 HRZES/ lHRZE / SHRE
• Treatment after lost to follow-up
0 Previous treatment outcome unknown
• Other
• Extra-pulmonary,previously treated drug-
Ila susceptible TB (whether bacteriologically-confinned
or clinically-diagnosedCNS/ bones or joints)
2 HRZES/ 1HRZE / 9HRE

Source:DOH.Manualof Proceduresof the NationalTuberculosis

ControlProgram.5th ed. 2014
WHO.Treatmentof Tuberculosis Guidelines.4th ed. 2010

B. TB Disease Registration Groups

GROUP DESCRIPTION
• Patient who has never been treated for TB or treated with <l
New
month of anti-TB drugs
• Previously treated for TB and declared cured or treatment
Relapse
completed, but presently diagnosed with TB

• Previously treated for TB but remain sputum smear or culture

Treatment after
positive at 5 months or late,; or clinically diagnosed TB who
failure
does not show clinical improvement anytime during treatment
Treatment after lost • Previously treated for TB but lost to follow-up for at least 2 months
to follow-up during course of treatment, and currently diagnosed with TB
Previous treatment • Previously treated for TB but outcome is unknown or
outcome unknown undocumented

C P reventive Th erapy
• Prevent development of infection among exposed contacts:
primary prophylaxis
Aims
• Prevent progression of latent TB infection to disease: secondary
prophylaxis

• lsoniazid prophylaxis therapy, once disease is excluded,

recommended for
• HIV positive individuals
DOH Guidelines • <S years of age if with bacteriologically confirmed
household contact regardless ofTST result
• <S years of age with clinically diagnosed household contact
IF TST is positive

• lsoniazid 10 mg/kg/day OD for 6 months

Dose
• Alternative: lsoniazid + Rifampicin for 3 months
316
V. PHARMACOLOGY
OF DRUGSFORTUBERCULOSIS
MECHANISM OF ADVERSE REACTIONS /
REMARKS
ACTION INTERACTIONS
1) lsoniazid (H) 10 (10-15) mg/kg/day (max: 300 mg/day)

• Bactericidal • Hepatitis, peripheral • Discontinue if ALT/AST is

against actively neuropathy, allergic skin more than 3-Sx the normal
growing reactions, possible hemolysis values
M. tuberculosis among G6PD-deficient patients • No dose adjustment for renal
• Inhibits mycolic • Inhibits drug-metabolizing dysfunction
acid synthesis enzymes leading to • Dose must not exceed 10 mg/
increased risk of phenytoin, kg/day when co-administered
ethosuximide, and with rifampicin
carbarnazepine toxicity

2) Rifampicin (R) 15 (10-20) mg/kg/day (max: 600 mg/day)

• Inhibits • Hepatitis, hypersensitivity • Discontinue if ALT/AST is

DNA-dependent reactions (including a systemic more than 3-Sx the normal
RNA polymerase flu-like syndrome with or values
without thrombocytopenia) • No dose adjustment for renal
• Orange discoloration of body dysfunction
fluids (e.g., urine)
• Results in decreased
plasma levels of some drugs
(e.g., antiepileptic drugs,
macrolides, hormonal therapy,
corticosteroids)

3) Pyrazinamide (Z) 30 (20-40) mg/kg/day (max 2 grams/day)

I
• Disruption of • Nausea, vomiting • Discontinue if AST/ ALT is
membrane energy • Most common cause of more than 3-Sx the normal
metabolism hepatotoxicity in regimens also values
containing H & R • Requires dose adjustment in
• Hypersensitivity reactions renal failure
• Polyarthralgia

4) Ethambutol (E) 20 (15-25) mg/kg/day (max 1.2 grams/day)

• Inhibits • Previously omitted from regimens for children <6 years old due to
transferase difficulty of monitoring optic neuritis
enzymes involved • New evidence shows safety in children at recommended doses in
in cell wall the absence of renal impairment
synthesis

5) Streptomycin (S) 30 (20-40) IM once daily (max 1 gram)

• Bactericidal • Potential for nephrotoxicity • Contraindicated in pregnancy
• Inhibits protein and electrolyte disorders • Dose adjustment in renal
synthesis • Eighth cranial nerve damage insufficiency
• Neuromuscular blockade

Note that dosing for children is higher compared to adults due to Jaster metabolism in children
Source:DOH.Manualof Procedures
of theNationalTuberculosis
ControlProgram.
5thed.2014
CDC.CoreCurnculum onTuberculosis.WhattheClinicianShouldKnow.6th
ed;2013

317
BRONCHIAL ASTHMA
I. ETIOPATHOGENESIS
• A reversible obstructive airway disease involving both the small & large airways with
increased residual lung volumes and a decreased FEVl/FVC ratio .
• Three components of an asthma attack: bronchospasm, airway edema, and increased
mucus production

II. MANIFESTATIONS
• Wheezing, shortness of breath, chest tightness, cough
• Symptoms worsen with certain "triggers" (e.g., viral infection, weather changes, exercise,
allergens, emotions, stress)
• Responds to therapy with bronchodilators
• Commonly with family history of asthma or atopy

III. DIAGNOSIS
A. Diagnosis in Children >5 years old (there are two main features for diagnosing asthma):
l. History of variable respiratory symptoms
Wheeze, shortness of breath, chest tightness, cough
Vary over time and intensity
Often occur or are worse at night or on waking
Often triggered by exercise, laughter, allergens, cold ai1; viral infections

2. Variable Expiratory Airflow Limitation

HOW TO MEASURE VALUES

Expiratory Airflow Limitation

Low FEV1/FVC ratio • <0.9

Abnormal Variation in Lung Function

Bronchodilator reversibility
Average daily diurnal peak
expiratory flow (PEF) variabilitay
After 4 weeks of anti-inflammatory
treatment
• FEVl increases by> 12%
after inhalation ofbronchodilator
• >13%
• FEVl increases by >12% of the predicted
value
Source:GINA2019updatefor adultsandchildrenolderthan5 years

B. Diagnosis in Children 55 years old

Pulmonary function tests are not done in young children due to inability to correctly
perform the expiratory maneuvers
0 Diagnosis is based on symptom patterns, frequency, family history & physical findings
Radiographs help to exclude structural abnormalities

FEATURES SUGGESTIVE OF ASTHMA FEATURES NOT SUGGESTIVE OF

ASTHMA
• Recurrent or persistent non-productive • Failure to thrive
cough worse at night • Neonatal or very early onset
• Recurrent wheezing during sleep or • Vomiting
exposure to triggers • Continuous wheezing
• Shortness of breath with exposure to • Failure to respond to asthma medications
triggers • Symptoms not associated with typical
• Reduced activity triggers
• TherapeutictJialwith low dose JCS& as needed • Focal lung or cardiovascular signs
SABArest~tsin clinicalimprovementin 2-3 • Finger clubbing
months &worseningwhen treatment isstopped • Hypoxemia outside of a viral illness
ICS:InhaledCorticosteroids
SABA:Short-ActingBeta-Agonists

Source:GINA2015updatefor children5 yearsandyounger

318
 C. Classification Based on Severity (For Initial Diagnosis)*
PERSISTENT
INTERMITTENT
MILD MODERATE SEVERE

Daytime :cclx/week but Daily & affects Daily & limits

< lx/week
symptoms less than daily daily activities activities
Nighttime
,;2x/month > 2x/month > lx/week > lx/week
symptoms

Peak expiratory c:80% c:80%

60-79% < 60%
flow rate (PEFR) predicted predicted
PEFR
< 20% 20-30% >30% > 30%
Variability

c:80% c:80%
FEV1 60-79% < 60%
predicted predicted
'Higherseverity
ofclassification
takesprecendence
(e.g.onefeatureofsevereclassifies
a patient
assevere)

Notethatthisis the 2002GINAclassification,it'suseis nolongerrecommendedexceptfor lheorelicalpurposes.However,

it
remainsto bea usefulclinical
toolforinitialdiagnosis andinitiating
therapy.
Source:Globallnit,at,veforAsthma.GlobalStrategyforAsthmaManagement
andPrevention,
2002

D.Classification Based on Control

>5 YEARS OLD
In the past 4 weeks, has the patient had:
• Daytime asthma symptoms >2x/week?
• Any night waking due to asthma 7
• Reliever needed for symptoms >2x/week 7
• Any activity limitation due to asthma?
:S5YEARS OLD
In the past 4 weeks, has the patient had:
• Daytime asthma symptoms >lx/week?
• Any night waking or night coughing due
to asthma?
• Reliever needed for symptoms >lx/week?
• Any activity limitation due to asthma?

Interpretation of Control:
• Well-controlled:

• Uncontrolled:
nonepresent
• Partlycontrolled:1-2present
3-4 present
I
Source:GINA2019updatefor adultsandchildrenolderthan5 years
GINA2015updatefor children5 yearsandyounger

IV. OVERVIEW OF THE MANAGEMENT OF ASTHMA

A. Recommended Inhaler Devices for Children
AGE PREFERRED ALTERNATE
0-3 years • pMDI with spacer & face mask • Nebulizer with face mask
• pMDI with spacer & • pMDI with spacer & face mask
4-5 years
mouthpiece • Nebulizer with mouthpiece or face mask
pMDI: Pressurized Metered-Dose Inhaler
GINA2015 updateforchildren5 yearsand younger

B. Recommendations for Initiating Controller Treatment

>5 YEARS OLD :S5YEARS OLD
• For the best outcomes, !CS-containing • Uncontrolled asthma symptoms
treatment should be initiated upon • Frequent wheezing episodes (:c:3per season)
diagnosis of asthma • Severe wheezing episodes
• SABAuse> lx/week
Source:GINA2019updatefor adultsandchildrenolderlhan5 years
GINA2015updalefor children5 yearsandyounger

319
V. PHARMACOLOGJC THERAPY FOR ASTHMA
DRUG CLASS EXAMPLES USES & MECHANISM CAUTION

Controller Medications (used for regular maintenance treatment)

Inhaled • Beclomethasone • Most effective anti- • Hoarseness/
Corticosteroids • Budesonide inflammatory agents dysphonia and oral
(ICS) • Fluticasone for persistent asthma candidiasis
• Best route for steroids • High doses increase
as it provides targeted risk of systematic
drug delivery, acts side effects
faste1; and a small dose
is required
• Reduce symptoms,
exacerbations, asthma-
related hospitalizations
and deaths; improves
lung function
JCSand long-acting • Beclomethasone/ • Addition of LABAto • LABAcomponent
beta-agonist (LABA) formoterol !CSreduce symptoms may cause
• Budesonide/ and exacerbations, and tachycardia,
formoterol improves lung function headache, cramps
• Fluticasone/ more rapidly than • LABAshould
salmeterol doubling the dose of ICS not be used
• Low-dose without ICS due to
beclomethasone/ increased risk of
formoterol or adverse outcomes
budesonide/formoterol • LABAhas only been
may be used for both studied in children
controller and reliever ;,4 years old
treatment
Leukotriene- • Montelukast • Block leukotriene • Elevated liver
Modifying Drugs • Zafirlukast receptors [montelukast, function tests
• Zileuton zafirlukast) or inhibit with zileuton and
lipoxygenase (zileuton) zafirlukast
• Less effective than ICS
in controlling asthma,
less effective than ICS/
LABAwhen used as
add-on therapy to ICS

Chromones • Cromolyn sodium • Inhibit mast cell and • Very short duration
• Nedocromil sensory nerve activation of action needing
sodium frequent dosing
• Favorable safety
profile
Long-Acting • Tiotropium • Blocks acetylcholine's • Not for children
Muscarinic effect on muscarinic < 12 years old
Antagonists (LAMA) receptors • Dry mouth
• Tiotropium as mist
inhaler is an add on
treatment for patients
with history of
exacerbations (Step 4)
Anti-lgE • Omalizumab • Inhibits lgE-mediated • Not for children
reactions ,;5 years old
• Limited to patients
with elevated
serum IgE levels

320
 Anti-I LS • Mepolizumab • Add-on therapy for • Not for children
• Benralizumab severe eosinophilic <12 years old
asthma • Headache, local
injection site
reactions

Anti-lL4R • Dupilumab • Add-on therapy for • Local injection site

severe eosinophilic
asthma or requiring
maintenance oral
corticosteroids
Systemic Steroids • Prednisone • Useful for treatment of
• Methylprednisolone acute exacerbations
• Hydrocortisone
reactions, blood
eosinophilia
• Truncal obesity,
easy bruisability,
osteoporosis, DM,
hypertension,
gastric ulceration,
proximal myopathy,
depression, cataracts

Reliever Medications
(for as-needed relief of breakthrough symptoms & prevention of exercise-induced asthma)
Short-Acting B2 • Salbutamol • Stimulates adenylyl • No effect on chronic
Agonists (SABAJ • Procaterol cyclase thus increasing inflammation
• Terbutaline cAMP levels and • May cause
• Albuterol causing bronchodilation tremors,
• Rapid onset of tachycardia, and
bronchodilation and a small fall in
best used for symptom potassium
relief
Short-Acting • lpratropium • Muscarinic receptor • Provide additional
Anticholinergics antagonists benefit when used
• Inhibit only the in combination

I
cholinergic reflex with SABAin those
components and with more severe
thereby less effective symptoms
than 82-agonists • Adverse effect:
dry mouth
(most common],
urinary retention,
glaucoma

Low-dose ICS/ • Beclomethasone/ • Used as both • Adverse effects

formoterol formoterol maintenance and of JCSand LABA
• Budesonide/ reliever treatment stated above
formoterol • Similar symptom
control with SABAuse
Source:GINA2019updatefor adultsandchildrenolderthan5 years
GINA2015updatefor children5 yearsandyounger

VL NON-PHARMACOLOGICTHERAPY FOR ASTHMA

• Smoking cessation for patients, parents or caregivers
• Regular physical activity
• Immunizations (Influenza, Pneumococcal Vaccine)
• Provide written asthma action plan

321
VII. STEPWISE APPROACHTO CONTROLSYMPTOMS AND MINIMIZEFUTURE RISK
• Asthma treatment is a continuous cycle: assess, adjust treatment, and review response
• A~er starting initial controlle1; review response after 2-3 months (or according to urgency)
° Consider stepping up if: uncontrolled symptoms, exacerbations or risks (but also
assess adherence to medications, correct inhaler technique, and persistent
exposure to triggers)
° Consider stepping down if: symptoms controlled for 3 months, low risk for
exacerbations
• Find the patient's lowest treatment that controls both symptoms and exacerbations

FOR CHILDREN > 5 YEARS OLD

sTEP~

As-needed Low-dose lCS Low-dose lCS

STEP 1 low-dose lCS/ whenever SABA whenever SABA is
formoterol is taken taken
Daily low-dose LTRA
!CS or as-needed Daily low-dose
STEPZ Low-dose !CS
low-dose !CS/ !CS
formoterol whenever SABA taken
As-needed
low-dose
Medium-dose Mod-high dose !CS lCS/
Low-dose !CS/
STEP3 !CS or low-dose formoterol*
LABA Low-dose !CS+ LTRA
!CS/LABA or as-
needed
High-dose !CS SABA
Medium-dose Refer for expert
STEP4 May add LTRA or
!CS/LABA advice
tiotropium
High dose !CS/LABA
Refer to specialist
STEP 5 Add low-dose OCS
Add on (e.g., anti-
lgE, tiotropium)

FOR CHILDREN S 5 YEARS OLD

STEP 1 None None

LTRA
STEPZ Low-dose !CS
Intermittent !CS
As needed
STEP3 Double 'low-dose' !CS Low-dose lCS+ LTRA SABA

Add LTRA
STEP4 Refer to specialist lncreaselCSfrequency
Add intermittent JCS
SABA:shortactingB2-agonist LTRA:Leukotrienereceptorantagonist
ICS:inhaledcorticosteroid OCS:oralcorticosteroid

"Low-doseICS/formoterol
is the relievermedication
for thoseprescribed
withlow-dosebudesonide/formoterol
or
low-dosebeclometasonelformoterolmaintenancetherapy
Source:GINA2019updatefor adultsandchildrenolderthan5 years
GINA2015updatefor children5 yearsandyounger

322
A. Dose of Commonly used Inhaled Corticosteroids for Children :5 5 years
DRUG LOWTOTALDAILYDOSE
Budesonide pMDI + spacer • 200 mcg
Budesonide nebulized • 500 mcg
Fluticasone proprionate
(HFA: hydroflouroalkane propellant) • 100 mcg

Beclometasone dipropionate
• 100 mcg
(HFA: hydroflouroalkane propellant)
Ciclesonide • 160 mcg
Mometasone furoate • Not studied in <4 years old
Triamcinolone acetonide • Not studied in this age group
GINA2015updatefor children5 yearsandyounger

B. Dose of Inhaled Corticosteroid (mcg) in Children >5 Years, Adults & Adolescents
RS OLD
DRUG
jHIGH
Beclometasone
dipropionate 200-500 500-1000 > 1000 100-200 200-400 >400
(CFC)
Beclometasone
di propionate 100-200 200-400 >400 50-100 100-200 >200
(HFA)
Budesonide 200-400 >400
200-400 400-800 >800 100-200
(DPI)
Budesonide
nebule
Ciclesonide
(HFA)
80-160 160-320 >320
250-500

80
500-1000

80-160
>1000

>160
I
Fluticasone
propionate 200-400 >400
(DPI)
100-250 250-500 >500 100-200
Fluticasone
propionate 200-500 >500
(HFA)
Mometasone 110-220 220-440 >440 110 220-440 >440
furoate
Triamcinolone >2000 400-800 800-1200 >1200
400-1000 1000-2000
acetonide
CFC-chlorofluorocarbon
propellant
DPI- drypowderinhaler
HFA-hydrofluroalkane
propellant

Source:GINA2019updatefor adultsandchildrenolderthan5 years

323
EXACERBATION OF BRONCHIAL ASTHMA
I. DEFINITION
• Acute or subacute deterioration in symptom control that is sufficient to cause distress or
risk to health
• Manifests as any of the following:
• Increase in wheeze or shortness of breath
• Increase in coughing, especially at night
0 Lethargy or reduced exercise tolerance
• Impairment of daily activities
Poor response to reliever medication

II. MANAGEMENTOF EXACERBATIONS>5 YEARS OLD

MILD OR MODERATE SEVERE LIFE THREATENING

Assess the Patient for Severity of Exacerbation

• Talks in phrases • Talks in words

Manifestations • Prefers sitting to lying • Sits hunched forward
• Not agitated • Agitated

Respiratory
• Fast • >30/min
rate • Drowsy, confused
Use ofaccessory • Silent chest
• No retractions • Yes
muscles
Pulse rate • 100-120bpm • >120 bpm
0 2 saturations • 90-95% • <90%
PEF • >50% predicted or best • $SO%predicted or best

Management depending on Setting

• SABA(4-10 puffs by
pMDI + spacer
q20 mins for 1 hour)'
• Prednisolone 1-2mg/kg
• Transfer to acute care facility
Management in POx 3-5days (max:40 mg)
• Give inhaled SABAand ipratropium bromide, 0 2
Primary Care • Oxygen (target sats
support, and systemic corticosteroids
Units 94-98% for children
• Consider referral to specialists
$12 years old, 93-95%
if>l2 years old)
• If worsening at 1 hour,
transfer to acute care
• SABA • SABA • ICU admission
• Consider ipratropium • Ipratropium bromide** • SABA & 0 2 support
bromide • Oxygen to ,naintain • Intubation
Management in
• 0_.(target sats 94-98% saturation 93-95% • Evidence
an Acute Care
for children $12 years old, • Oral or IVcorticosteroids regarding the role
Facility
93-95% if>l2 years old) (prednisolone, of non-invasive
• Oral corticosteroids hydrocortisone)
ventilation [NIV)
• Consider IV Mg***
is weak
• If continuing deterioration, treat as severe and • Refer to specialists
admit to ICU
Monitoring
• Consider referral to specialists
• See disposition or discharge planning below
'AfterfirsthourofinhaledSABA,
dosevariesfrom4-10puffsq3-4hoursupto6-10puffsq1-2hours
..Treatment withbothSABA andipratropium
(short-acting
anlichotinergic)
formoderate-severe
exacerbations
is betterthanSABA
alone
... IV magnesium
sulfateisnotrecommended
forroutine
useinexacerbations,
consider
if thepatient
doesnotrespond
toinitial
intensivetreatment

Source:GINA2018
updateforadultsandchildren
olderthan5 years
GINA2019updateioradultsandchildren
olderthan5 years
324
III. MANAGEMENTOF EXACERBATIONS,,;5 YEARS OLD

MILD OR SEVERE OR
MODERATE LIFE THREATENING
Assess the Patient for Severity of Exacerbation

• Talks in words
• Confused, drowsy
• Talks in phrases
• Unable to speak or drink
Manifestations • Breathless, agitated
• Central cyanosis
• Markedsubcostalor subglotticreactions
• Silent chest on auscultation

• ,;200 bpm (,;3 years old) or • >200 bpm (,;3 years old) or
Pulse rate
,;180 bpm (4-5 years old) >180 bpm (4-5 years old)
0 2 saturations • ~92% • <92%
• SABA(2 puffs by pMDI + spacer or
2.5 mg by nebulizer q20 mins for
1 hour)
• Transfer to high level care (ICU)
• Oxygen (target sats 94-98%)
• Give inhaled SABAand
• Prednisolone 1-2mg/kg PO x 3-5
Management ipratropium bromide, 0 2 support,
days (max: 20 mg for <2 years,
and systemic corticosteroids
30 mg for 2-5 years) - if with
• Consider referral to specialists
recurrence of symptoms in 3-4 hours
• If worsening at 1 hour, transfer to
high level care (ICU)
• If continuing deterioration, treat as severe and admit to ICU
Monitoring • Consider referral to specialists
• See disposition or discharge planning below
'Afterfirsthourof inhaledSABA,dosevariesfrom4-10puffsq3-4hoursupto 2-3puffsperhour
"TreatmentwithbothSABAandipratropium (short-acting
anticholinergic)
formoderate-severe
exacerbations
is betterthanSABAalone

GINA2015updatefor children5 yearsandyounger I

IV. DISPOSITION
ASSESS FOR DISCHARGE ARRANGE FOR DISCHARGE

• Symptoms improved not needing SABA • Reliever: continue as needed

• PEF improving and >60-80% of personal • Controller: start or step up
best or predicted • Prednisolone: continue for 3-5 days
• Oxygen sat >94% at room air • Follow up within 2-7 days
• Resources at home adequate

Follow up

• Reliever: reduce to as needed

• Controller: continue higher dose for short-term (1-2 weeks) or long-term (3 months)
depending on background to exacerbation
• Risk factors: check and correct modifiable risk factors that may have contributed to exacerbation
• Review if asthma action plan is understood, used appropriately or needs modification

Source:GINA2019updateforadultsandchildrenolderthan5 years

325
 SECTION FIVE
PATHOLOGIES OF THE PLEURA

PLEURAL EFFUSION
I. ETIOPATHOGENESIS
• Pleural fluid comes from the capillaries of the parietal pleura and is absorbed from the
pleural space via pleural stomas and lymphatics
• Only 4-12 mL of fluid is present in the pleural space and if formation exceeds clearance,
fluid will accumulate as pleural effusion

SEROFIBRINOUS OR
DRY OR PLASTIC EMPYEMA OR PURULENT
SEROSANGUINEOUS
EFFUSION EFFUSION
EFFUSION

Possible Etiologies
• Acute bacterial or viral
pulmonary infection
• Acute URTI
• TB
• Connective tissue disorders
such as rheumatic fever
Pathophysiology
• Process limited to the
visceral pleura
• Small amounts of yellow
serous fluid and adhesions
between the pleural surface
• In TB: pleura is thickened
& adhesions develop rapidly
• Lung infections
• Inflammatory conditions
of the abdomen/mediastinum
• Connective tissue diseases
(e.g., SLE)
• Primary or metastatic
neoplasms of the lung,
pleura, or mediastinum
• Fibrinous exudate on the
pleural surface
• Exudative effusion of
serous fluid into the
pleural cavity
• Pneumonia due to
S.pneumoniae (most
common) and S.aureus
(most common in
developing countries)
• Rupture of a lung abscess
into the pleural space
• Contamination introduced
from trauma or thoracic
surgery
• Mediastinitis or extension
ofintraabdominal abscesses
• Accumulation of pus in the
pleural space
• Three Stages
0 Exudative stage:
fibrinous exudate on the
pleural surface
° Fibrinopurulent stage:
formation of fibrinous
septa leading to
loculation of fluid and
thickening of parietal
pleura
0 Organizational stage:
fibroblast proliferation
(pockets of loculated
pus may develop into
thick-walled abscess
cavities)

326
II. MANIFESTATIONS
DRY OR PLASTIC
EFFUSION
Symptoms • Chest pain
exaggerated by deep
breathing, coughing,
and straining
• Patient often lies on
the affected side to
decrease respiratory
excursions
Signs • Leathery, rough,
biphasic friction rub
• Dullness to percussion
• Decreased breath
sounds
III. DIAGNOSIS
A. Basic Tests and Chest Imaging
DRY OR PLASTIC
EFFUSION
Chest • Diffuse haziness at
Radiograph the pleural surface
(X-ray) • Dense, sharply
demarcated shadow
Chest • If radiographs are
Ultrasound negative, ultrasound
or CT scan of the
chest may be done
'Othertestsfor empyema
or purulenteffusion:bloodculture,CBC,ESR
SEROFIBRINOUS OR
EMPYEMAOR
SEROSANGUINEOUS
PURULENT EFFUSION
EFFUSION
• Early manifestations • Feve1; respiratory
of dry pleurisy distress
• As fluid accumulates, • Ill-looking patient
pleuritic pain • Complications:
disappears bronchopleural
• Small effusion: fistula and
manifestations related pyopneumothorax
to underlying disorder with S. aureus,
• Large effusion: cough, purulent pericarditis,
dyspnea, retractions, lung abscess, peritonitis,
tachypnea,orthopnea, osteomyelitis of the
or cyanosis ribs, septicemia
• Dull to flat on • Similar to
percussion; serofibrinous
decreased or absent pleural effusion
breath sounds ( differentiated only
• Decreased tactile by thoracentesis)
fremitus
• Shift of mediastinum
away from the
affected side
• Signs may shift with
changes in position
if the fluid is not
loculated
SEROFIBRINOUS OR
SEROSANGUINEOUS
EMPYEMAOR
PURULENT
I
EFFUSION EFFUSION
• Homogenous density • Similar findings
obliterating the with any pleural
normal lung markings effusion
• If small effusion: • The absence of
obliterated fluid shift with
costophrenic or changes in
cardiophrenic angles position indicates
or widening of the a loculated
interlobar septa empyema
• Examine the patient
both in supine and
upright positions to
demonstrate a shift of
the effusion
• Helpful guide to thoracentesis if effusion is
loculated
327
 8. Pleural Fluid Analysis
° Culture for bacterial, fungal. mycobacterial organisms, gram stain
0Protein, lactic dehydrogenase and glucose (Glucose is <60 mg/dL in malignancy, TB,
and rheumatoid disease)
0Amylase
Specific gravity and pH
Total cell count and differential, cytologic examination (to reveal malignant cells)

To differentiate transudative from exudative pleural fluid, exudates have at least

1 of the following:
0 Protein >3.0 g/dL
0 pH <7.20
0 Pleural fluid:serum protein ratio >0.5*
0 Pleural fluid:serum LDH ratio >0.6*
0 Pleural fluid LDH level >200 IU/L or pleural fluid LDH >2/3 serum LDH upper limit
of normal*
·These3 parameters
formtheLight'sCriteria

C. Pleural Fluid Analysis in Empyema

Bacteria is present on Gram stain
, pH is <7.20
0 >100,000 neutrophils/uL
0 Pneumococcal empyema: culture is (+) in 58% of patients
0 If negative culture for pneumococcus: do pneumococcal PCR analysis

IV. MANAGEMENT
Dry or Plastic • Treatment is directed at the underlying disorder
Effusion • Patients with pleurisy and pneumonia should always be screened
for tuberculosis
• Analgesia with NSAIDs may be helpful
Serofibrinous or • Treatment is directed at the underlying disorder
Serosanguineous • Therapeutic thoracentesis unless small effusion only
Effusion • Rapid removal of :;,1 liter of pleural fluid may be associated with
re-expansion pulmonary edema
• If underlying problem is adequately treated, further drainage is
usually unnecessary
• Tube drainage is done for:
° Fluid re-accumulation leading to respiratory compromise
0 Older children with parapneumonic effusion, and the pleural
fluid pH is <7.20 or pleural fluid glucose level is <SO mg/dL
Antibiotics • Choice of antibiotic is based on in vitro sensitivities
of the responsible organism
• Clinical response is slow even with antibiotics (may
have little improvement for as long as 2 weeks)
• For staphylococcal infections: treatment for 3-4 weeks
Chest Tube • Controlled by underwater seal and continuous suction
Empyema or Drainage • Usually continued for 5-7 days
Purulent Effusion • Fibrinolytic agents are instilled to promote
drainage, decrease fever, lessen need for surgical
intervention, and shorten hospitalization
Surgery • If patient remains febrile and dyspneic >72 hours
after systemic antibiotics and chest tube drainage,
surgical decortication via VATS
• lfVATSis ineffective, open decortication is performed
Source:KliegmanR,et al. Nelsontextbookof pediatrics
(21sted.).Canada:Elsevier
CashenK, et al. Pleuraleffusions
andpneumothoraces. PediatricsinReview;
2017

328
PNEUMOTHORAX
I. ETIOPATHOGENESIS
• Accumulation of extra pulmonary air in the intrapleural space
• Most commonly due to leakage of air within the lung
A. Primary versus Secondary Pneumothorax
0 Primary pneumothorax: occurs without trauma or underlying lung disease
0 Seconda1y pneumothorax: complication of an underlying lung disorder but without trauma

• Most frequently in male teenager • Causes: blunt/penetrating trauma, high

who are tall and thin, thought to have
subpleural blebs
• Other causes: primary idiopathic,
secondary blebs, congenital lung
disease, asthma, foreign body, smoking,
infection, diffuse lung disease
'Other types:iatrogenicor catamenial(occurringduringmenstrualperiodswithdiaphragmaticdefect)
flow nasal cannula therapy, thoracotomy,
thoracentesis, mechanical ventilation,
tracheostomy
• When air enters the pleural space,
the lung collapses leading to alveolar
hypoventilation and V/Q mismatch
• In tension pneumothorax: continuing
air leak results to increased positive
pressure in the pleural space, further
lung compression, shift of mediastinal
structures toward the unaffected side, and
decrease in venous return & cardiac output

II. MANIFESTATIONS
• Onset is usually abrupt
Symptoms • Severity of symptoms depend on extent of lung collapse & pre-existing lung disease
• Dyspnea, pain, cyanosis
• Respiratory distress with retractions, decreased breath sounds, tympanitic

I
on percussion on the involved side
Signs • Larynx, trachea, and heart shift toward the unaffected side
• Gurgling sounds synchronous with respiration suggests an open fistula
(when fluid is present in the pleural cavity)

III. DIAGNOSIS
DIAGNOSTIC REMARKS
• Shows less lung collapse if taken early
• Expiratory view accentuates the contrast between lung markings and the
Chest
clear area of the pneumothorax
Radiograph
• Evidence of tension pneumothorax if with shift of mediastinal structures
away from the air leak
Ultrasound • Chest ultrasound may be used to establish the diagnosis

IV. MANAGEMENT
ASPECT MANAGEMENT
• 100% oxygen may hasten resolution
Supportive
• Analgesic if with pleural pain

Depending • Small ( <5%) or moderate-sized pneumothorax in a normal child: may

on extent of resolve without specific treatment (usually within 1 week)
collapse & • Recurrent, secondary, under tension, >5% collapse: chest tube drainage
underlying • Tension pneumothorax: needle aspiration on an emergency basis
disease • Closed thoracotomy or pigtail catheters: to re-expand the lung
Source:KliegmanR. et al. Nelsontextbookof pediatrics(21sted.).Canada:Elsevier
CashenK.et al.Pleural effusionsandpneumothoraces. PediatricsinReview:
2017
329
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330
 IMMUNOLOGY
AND
ALLERGY
 SECTION ONE
ALLERGOLOGY

OVERVIEW OF ALLERGY

I. ETIOPATHOGENESIS& MANIFESTATIONS
• Chronic condition involving an abnormal reaction to an allergen
• Allergic patients have altered state of reactivity to environmental and food antigens
Response to allergen exposure: expansion ofT helper type 2 (Th2) cells, which then
favors IgE synthesis & eosinophilia
A portion of the immune response induces proliferation ofThl cells which leads to
chronicity and the effector phase
• Strong familial predisposition with about 60% heritability (risk for allergic disease of a
child is 30-50% if 1 parent is allergic and 60-80% if both parents are allergic)

Reaction Patterns of IgE-Mediated Responses

RESPONSE PATHOPHYSIOLOGY MANIFESTATIONS
• Immediate response after allergen exposure
• Occurs within 1-30 minutes, resolves in 1-3 hours
• Itching, sneezing,
Early-phase • Cross-linking of lgE results in mast cell
wheezing, abdominal
response degranulation & release of preformed mediators
cramps
• Vasodilation, increased local vascular
permeability with edema
• Edema, redness,
• Occurs within hours of allergen exposure
warmth, induration
(peaks 6-12 hours), resolves in 24 hours
Late-phase of the skin
• Early infiltration of neutrophils and
response • Sustained nasal
eosinophils, followed by basophils, monocytes,
blockage; persistent
macrophages and Th2 cells
wheezing
• Tissue inflammation persist for days to years
• Tissue remodeling
Chronic • Repeated stimulation of allergic effector cells
leads to irreversible
allergic contributes to unresolved inflammatory conditions
disease • Th2 cytokines prolong survival of allergic
effector cells by delaying apoptosis
changes in target
organs

Source:KllegmanRM.et al. NelsonTextbookof Pedratncs

GalliSJ,et al. Thedevelopment
(21sted).Elsevier;2020
of allergicinflammation.Nature;2008
I
II. DIAGNOSIS
• CBCand nasal and bronchial secretions are checked for eosinophilia
In vitro tests • Serum total lgE level (poor diagnostic value)
• Allergen-specific lgE assay (directed towards clinically relevant allergens)
• Allergy skin tests by prick/puncture technique: chief advantage over in
vitro tests is interpretability within 15-20 minutes
• Selective skin test by intradermal technique
• Not recommended for food allergens due to risk of anaphylaxis
In vivo tests • Labor-intensive (skin prick test is preferred over intradermal test)
• Used for insect venom sensitivity, drug sensitivity, or when an allergen
is considered relevant but skin prick test is negative
• Oral food challenge for food allergy
• Metacho!ine challenge testing for asthma
Source:KliegmanRM.et al. NelsonTextbook
of Pediatrics
(21sted).Elsevier;2020
Heinzeninget al. 2013

333
Ill. MANAGEMENT
A. General Management
0 Avoidance of allergens (most common indoor allergens include house dust mites, cat,
cockroach)
Pharmacologic therapy (discussed in detail per disease entity)

B. Allergen lmmunotherapy
INDICATIONS* CONTRAINDICATIONS
• Allergic rhinitis • Patients under beta-blocker therapy
• Asthma triggered by allergen exposures • Severe or uncontrolled asthma
• Insect venom sensitivity • Significant cardiovascular disease
• Atopic dermatitis with aeroallergen • Patients poorly compliant or unable to
sensitization communicate clearly with physicians
'Notrecommended for<5yearsoldbecause ofincreased
riskofsystemic reactions,
specialexpertiserequired
to
treatanaphylaxis
in thisagegroup,inabilityto communicateclearlyintheeventof allergicreaction
Source:KliegmanRM,et al. NelsonTextbookof Pediatrics(21sted). Elsevier;2020
LeungDYM.et al. Pediatricallergyprinciplesandpractice.Elsevier;2015'Cox L. et al. J AllergyClin lmmunol.2011

ALLERGIC RHINITIS (AR)

I. ETIOPATHOGENESIS
• Inflammatory disorder of the nasal mucosa
• Repeated intranasal introduction of allergens cause "priming" - more brisk response with
lesser provocation

--
A. Classification
INTERMITTENT PERSISTENT

MILD MODERATE-TO-SEVERE
(ALL OF THE FOLLOWING) (AT LEAST 1)
• Normal sleep • Abnormal sleep
• Normal daily activities • lmpain11ent of daily activities, sport & leisure
• Normal work and school • Impaired work or school
• No troublesome symptoms • Troublesome symptoms
Source:KliegmanRM.et al. NelsonTextbookof Pediatrics(21sted). Elsevier:2020
BousquetJ. et al. AllergicRhinitisandits ImpactonAsthma(ARIA)2008update.Allergy:2008

B. Protective and Risk Factors

PROTECTIVE FACTORS RISK FACTORS

• Early exposure to dogs and cats • Family history of atopy

• High serum IgE levels before 6 years old
• Prolonged breastfeeding
• Maternal smoking
• Early introduction to wheat, rye, oats, • Cesarean section
barley, fish, eggs • 23 episodes of rhinorrhea in 1" year of life

II. MANIFESTATIONS
• Sneezing, rhinorrhea, nasal obstruction
Symptoms • Itching of the nose, palate, pharynx & ears
• Itching, redness & tearing of the eyes
• Allergic salute: upward rubbing of nose to relieve itching and blockage
• Transverse nasal crease
Signs • Allergic gape: continuous open-mouth breathing
• Chapped lips, dental malocclusion
• Allergic shiners: dark circles under eyes

Nasal speculum • Clear nasal secretions

exam • Edematous, boggy, bluish mucus membranes and swollen turbinates

334
III. DIAGNOSIS
DIAGNOSTIC REMARKS
• To avoid false-negative results, montelukast should be withheld
Skin tests for 1 day, sedating antihistamines for 3-4 days, and non-sedating
antihistamines for 5-7 days
• Provide a suitable alternative to skin tests in the following conditions:
0 Dermatographism or extensive dermatitis
Serum IgE 0 Intake of medications that interfere with mast cell degranulation
0 High risk for anaphylaxis

0 Uncooperative patients

Nasal smears • Presence of eosinophils in nasal smear supports diagnosis of AR

IV. MANAGEMENT
A. Pharmacologic Therapy
CLASS REMARKS
• Oral or intranasal antihistamine or intranasal glucocorticoids
Mild
• May add leukotriene receptor antagonists (LTRA)if with asthma
Intermittent
• May add decongestant
• Intranasal glucocorticosteroids
Moderate- • May add oral or intranasal antihistamine (2"' generation preferred
over 1st generation oral antihistamine)
SevereOR
Persistent AR • May add LTRAinstead of antihistamine if with concomitant asthma
• Intranasal chromones
• Add intraocular antihistamine/chromones for ocular symptoms

B. Commonly Used Drugs (see doses in Chapter 3)

DRUG CLASS EXAMPLES REMARKS
1st • Chlorpheniramine • Reduce sneezing, rhinorrhea, ocular
Gen maleate symptoms
Oral • Cetirizine
Antihistamines znct • Loratadine • Preferred over 1" generation

I
• Desloratadine
Gen • Levocetirizine antihistamines due to less sedation
• Fexofenadine
• Oral decongestant
• Cetirizine +
• Not favored for use due to irritability,
pseudoephedrine
insomnia, link with infant mortality
• Chlorpheniramine
Decongestants maleate + • Oral decongestant
phenylephrine HCl
• Intranasal decongestant
• Should not be used >3 days and not
• Oxymetazoline
to be repeated> lx per month to
avoid rebound nasal congestion

• Ipratropium • Relief of rhinorrhea

Anticholinergics • May cause epistaxis, nasal dryness
bromide • Nasal spray not locally available
Leukotriene • Modest effect on rhinorrhea and
• Montelukast
Receptors nasal blockage
• Fluticasone furoate/ • Most effective therapy for severe,
propionate
Intranasal persistent AR
Corticosteroids • Mometasone • May cause nasal irritation, epistaxis
• Triamcinolone • Monitor growth of patients

335
 C. Monitoring for Persistent Rhinitis (review after 2-4 weeks)
0 If improved: continue treatment for 1 month
Failure of treatment:
Review diagnosis, review compliance, query infections or other causes
Increase intranasal steroid dose
0 Add antihistamine if with itch/sneeze
Add ipratropium bromide if with rhinorrhea
Add intranasal decongestant if with blockage (not for use in infants & preschool children)
Give short-term oral steroids if with severe nasal or ocular symptoms
0 If persistent failure despite above regimen: surgical referral

D.Others
Avoidance of allergens
0 Immunotherapy if symptoms cannot be adequately controlled by avoidance & medication

ATOPIC DERMATITIS (AD)/ ATOPIC ECZEMA

I. ETIOPATHOGENESIS
• Most common chronic relapsing skin disease in infancy and childhood
• Complex genetic disorder that results in defective skin barrier, reduced skin innate immune
responses, and exaggerated T-cell responses to environmental allergens and microbes
• Pathology:
Acute AD: spongiosis (marked intercellular edema of the epidermis)
° Chronic AD: hyperplastic epidermis with hyperkeratosis

II. MANIFESTATIONS
• Severely dry skin is a hallmark of AD
• Cardinal features: intense pruritus especially at night and cutaneous reactivity
• Based on chronicity of AD:
Acute: pruritic, erythematous papules
0 Subacute: erythematous, excoriated, scaling pa pules
° Chronic: lichenification and fibrotic papules
• Patients often goes into remission as they grow olde1; leaving adolescents and adults with
skin prone to itching and inflammation when exposed to exogenous irritants

III. DIAGNOSIS
A. Classification Based on Severity of Disease

NICE GUIDELINES EUROPEAN

SKIN QUALITY OF LIFE GUIDELINES

• Dry skin, infrequent itching

• SCORAD <25 or
Mild (with or without small • Limited impact
transient eczema
areas of redness)
• Dry skin, frequent itching,
redness (with or without • SCORAD 25-50 or
Moderate • Moderate impact
excoriation and localized recurrent eczema
skin thickening)
• Widespread dry skin,
incessant itching,
redness (with or without
• SCORAD >50 or
Severe excoriation, extensive • Severe limitation
persistent eczema
skin thickening, bleeding,
oozing, cracking,
pigmentation changes)

'SCORAD:ScoringofAtopicDermatitis,a clinicaltoolto assess the extentand intensityof atopicdermatitis

Source:Wollenberg
A et al. JEADV:2018
NICEguidelines:2007

336
B. Diagnostic Criteria (Hanifin and Rajka)
MAJOR FEATURES
(AT LEAST 3)
• Pruritus
• Typical morphology and
distribution:
° Flexural lichenification or
linearity in adults
° Facial & extensor involvement
in infants & children
• Chronic or chronically-
relapsing dermatitis
• Personal or family history of
atopy
MINOR FEATURES
(AT LEAST 3)
• Xerosis
• lchthyosis/palmar hyperlinearity /keratosis pilaris
• Immediate skin reactivity (type I skin test reactivity)
• Elevated serum lgE
• Early age of onset
• Tendency toward cutaneous infections/impaired
cell-mediated immunity
• Tendency toward non-specific hand or foot dermatitis
• Nipple eczema
• Cheilitis
• Recurrent conjunctivitis
• Dennie-Morgan infraorbital fold
• Keratoconus
• Anterior subcapsular cataracts
• Orbital darkening
• Facial pallor /facial erythema
• Pityriasis alba
• Anterior neck folds
• Itch when sweating
• Intolerance to wool and lipid solvents
• Perifollicular accentuation
• Food intolerance
• Course influenced by environmentaljemotional factors
• White dermographism/delayed blanch

C □ -ff t' Io· .

DISEASE FEATURES TREATMENT
• T-cellmediated hypersensitivity
reaction from antigen on skin
• Avoidance of offending agent
Allergic contact • Lesions localized to area of contact
• Topical corticosteroids
dermatitis • Examples: poison ivy, nickel,
• Antihistamine
perfume, topical medications &

I
cosmetics
• Prolonged or repetitive contact
with physical, chemical or
• Removal of stimulus
Irritant contact mechanical irritants
• Topical corticosteroids
dermatitis • Examples: saliva, urine, feces,
fragrance, detergents, dyes,
caterpillars, chafing
• Infants:
• Chronic inflammatory disease 0 Emollients, baby oil, gentle
that parallels the distribution shampoo, soft brush to
and activity of sebaceous glands remove scales
• Unknown cause, ° For persistent lesions:
Ma/assezia fwfur implicated as topical corticosteroids,
Seborrheic
causative agent topical antifungal,
dermatitis
• Greasy, scaly, erythematous antifungal shampoo
papular dermatitis involving
scalp (cradle cap), face, neck, • Children and adolescents:
retroauricular areas, axillae, 0Antifungal shampoo
umbilicus, diaper area (first-line)
0Topical corticosteroids
Skin infections • Includes scabies, dermatophytosis {see infectious disease chapter]
Source:KliegmanRM,et al. NelsonTextbookof Pediatrics(21sted).Elsevier;2020
LeungDYM,et al. Pediatricallergyprinciplesandpractice.Elsevier;2015
HanifinJM,et al. 1980;Schneideret al. 2013

337
IV. MANAGEMENT
A. Overview of Management
ASPECT IN
REMARKS
MANAGEMENT
• First-line therapy
• Lukewarm soaking baths for 15-20 minutes followed
Moisturizers immediately by application of moisturizer
• Hydration promotes trans-epidermal penetration of topical
glucocorticoids
• Cornerstone of anti-inflammatory treatment
• Applied 1-2 times daily
• Includes: hydrocortisone, fluocinolone, betamethasone,
mometasone
• Proactive therapy: intermittent topical steroid therapy (1-2
times per week) may be administered as maintenance therapy
Topical on areas that commonly flare/relapse after control of AD is
corticosteroids achieved
• Monitor for cutaneous side effects (striae, skin atrophy)
• Commonly used method for selection of steroids:
0 Low potency (Group 6 and 7) steroids may be used for face,
axilla and genitals
0 Mid-potency (Group 4 and 5) may be used for the trunk and

extremities
• Topical calcineurin inhibitors (pimecrolimus, tacrolimus)
• Tar preparations
• Antihistamine
• Phototherapy
Others
• Systemic immunomodulatory agents in severe, refractory cases:
systemic steroids, cyclosporine, azathioprine, methotrexate,
mycophenolate mofetil, interferon gamma
• Antimicrobial therapy for secondary bacterial or viral infection
Source:KliegmanRM,et al. NelsonTextbookof Pediatrics(21sted). Elsevier;2020
Guidelines of careforthemanagement of atopicdermatitis;
2014

B.Stepwise Management of Atopic Dermatitis Based on Severity of Disease

° For each classification of disease, the following additional treatment strategies should
be considered:
DISEASE
ADD ON THERAPY
SEVERITY
Basic therapy • Emollients, patient education, avoidance of allergens
• Topical glucocorticosteroids, topical calcinuerin inhibitors,
Mild AD
antiseptics
• Proactive therapy with topical tacrolimus or topical
Moderate AD
corticosteroids, wet wrap therapy, UVtherapy
Severe AD • Hospitalization, systemic immunosuppression
Wollenberg
Aet al. JEADV;2018

338
 C. Classification of Commonly Used Topical Corticosteroids
0 Other countries adopt different classification method, the one presented here is the
one commonly used in our setting
GROUP ADD ON THERAPY
• Clobetasol propionate 0.05% ointment/cream
Group 1
• Betamethasone di propionate 0.05% ointment/lotion/gel
(most potent)
• Fluocinonide 0.1 % cream
• Mometasone furoate 0.1 % ointment
• Halcinonide 0.1 % cream
Group 2 • Fluocinonide 0.05% ointment/cream
• Desoximetasone 0.25% ointment/cream
• Betamethasone dipropionate 0.05% cream
• Fluticasone propionate 0.005% ointment
Group 3 • Halcinonide 0.1% ointment
• Betamethasone valerate 0.1% ointment
• Mometasone furoate 0.1 % cream
Group4 • Triamcinolone acetonide 0.1% ointment/cream
• Fluocinolone acetonide 0.025% ointment
• Fluocinolone acetonide 0.025% cream
Group 5
• Hydrocortisone valerate 0.2% ointment
• Desonide 05% ointment/cream/lotion
Group 6
• Alclometasone dipropionate 0.05% ointment/cream
Group 7
• Hydrocortisone 2.5%, 1%, 0.5% ointment/cream/lotion
(least potent)

FOOD ALLERGY
I. DEFINITION OF TERMS
• Food hypersensitivity: adverse immunologic response due to lgE-mediated or cell-
mediated mechanisms
0 lgE-mediated responses are more common, with rapid onset of symptoms
° Cell-mediated or non lgE-mediated food allergy is less common, with later onset of
symptoms
• Food intolerance: adverse physiologic responses based on functional properties of food
I
II. ETIOPATHOGENESIS
A. Etiology
Egg, milk, peanuts, fish soy, wheat account for 90% of food allergies during childhood
Virtually all milk allergies develop by 12 months of age, all egg allergies by 18
months of age
0 Most children "outgrow" milk and egg allergies, with about 50% doing so by school age.
0 About 80-90% of children with peanut, nut, or seafood allergy retain their allergy for life
0 Delayed introduction of these foods increases risk of allergy

8. Classes of Food Allergens

° Class 1 food allergens: penetrates the GI barrier
° Class 2 food allergens: penetrates the respiratory tract

339
III. MANIFESTATIONS
SYSTEM MANIFESTATIONS

• Irritability, vomiting, spitting up, diarrhea, poor weight gain

• Food protein-induced enterocolitis syndrome (FPIES): severe
bloody diarrhea, vomiting, failure to thrive
• Food protein-induced proctocolitis: blood-streaked stools in
otherwise healthy infants
• Food protein-induced enteropathy: protracted diarrhea, failure
Gastrointestinal
tract
to thrive, anemia, hypoalbuminemia, edema
• Eosinophilic esophagi tis: chronic gastroesophageal reflux,
emesis, food refusal, dysphagia, abdominal pain
• Eosinophilic gastroenteritis: features of eosinophilic
esophagitis + prominent weight loss
• Oral allergy syndrome: oral pruritus, tingling and angioedema
of the oral cavity
• Atopic dermatitis
Skin • Acute urticaria and angioedema
• Perioral dermatitis / flushing
• Allergic rhinitis symptoms: periocular pruritus, tearing, nasal
Respiratory congestion, nasal pruritus, sneezing, rhinorrhea
• Wheezing, cough, chest tightness, dyspnea, laryngeal edema
• Anaphylaxis
Multisystemic • Heiner syndrome (pulmonary infiltrates, failure to thrive, iron
deficiency anemia from cow's milk allergy)

Source:Kliegman
RM.et al. NelsonTextbookof Pediatrics(21sted). Elsevier;2020
Kananiet al. 2011,Kaplan2008

IV. DIAGNOSIS

DIAGNOSTIC REMARKS

Elimination diet • The only way to establish the diagnosis

followed by food • Suspected food should be eliminated for 10-14 days for lgE-
challenge mediated food allergy and 8 weeks for cell-mediated food allergy
• Demonstrate IgE sensitization (sensitization is defined as
presence of food-specific IgE antibodies)
• Negative skin test excludes IgE-mediated form of food allergy
Skin Tests
• Positive skin test does not always equate to clinical reaction
to the food; food elimination and challenge is necessary to
establish the diagnosis
Serum Food-Specific • Levels for milk, egg and peanut are associated with a >95%
IgE Levels likelihood of clinical reaction to the food

V. MANAGEMENT
• Elimination of food with periodic reevaluation (since most food allergies are outgrown)
• Self-injectable epinephrine in case of accidental ingestion

340
URTICARIA (HIVES) AND ANGIOEDEMA
I. DEFINITION OF TERMS

URTICARIA ANGIOEDEMA
• Transient, pruritic, erythematous, raised
• Edema involving deeper subcutaneous
wheals with flat tops and edema that may
tissues (eyelids, lips, tongue, genitals,
become tense and painful
dorsum of hands or feet, GI tract wall)
• Blanches with pressure
• Sometimes painful rather than pruritic
• Resolves without residual lesions
• Resolves without residual lesions
• Usually last 20 minutes to 3 hours, rarely
• Slower resolution (up to 3 days)
more than 24 hours

II. CLASSIFICATION
ACUTE URTICARIA/ CHRONIC URTICARIA/
ANGIOEDEMA ANGIOEDEMA
Duration • ,;6 weeks • >6 weeks
• Often caused by IgE-mediated
reaction, but may also be from
• Autoimmune, neoplastic,
non-IgE mediated stimulation of
physical* urticaria
mast cells
Etiology • Chronic spontaneous urticaria
• Most commonly from food,
(idiopathic) in approximately
drugs, insect stings, infection,
80%
transfusion, contact allergy
• Idiopathic in approximately 50%
• Primary clinical
• Primary clinical • CBC, ESR, antinuclear antibodies
Diagnosis • Skin prick tests (ANA), thyroid autoantibody
• Serum-specific IgE tests testing, liver function tests,
autologous serum skin tests (ASST)
• Avoidance of trigger

• Avoidance of trigger
• Non-sedating Hl antihistamine:
• Non-sedating Hl antihistamine:
mainstay of therapy
• If inadequate control after
2-4 weeks or symptoms
I
Management mainstay of therapy are intolerable, increase
• If severe: epinephrine IM, oral antihistamine dose up to 4x
corticosteroids • If still with inadequate control,
add omalizumab
• If still with inadequate control,
add cyclosporine
'Examples of physicalurticaria:dermatographism(abilityto writeon skin),cold urticaria(coldstimulus),heat contact
urtcaria(warmstimulus),cholinergicurticaria(exercise,hot showers,sweating,strong emotion),delayed pressure
urticaria(tightclothing,sitting),solar urticaria(sun exposure),aquagenicurticaria(waler),vibratoryurticaria
(vibrations)

Source:KliegrnanRM,et al. NelsonTextbookof Pediatncs(21sted).Elsevier:2020

Kananiet al. 2011
AdkinsonNF,et al. Middleton's
AllergyPnnciples& Practice(8thed).Elsevier:2014.
ZuberbierT, el al.Allergy.2018

341
ADVERSE REACTIONS TO DRUGS
I. ETIOPATHOGENESIS
A. Types of Drug Reactions

PREDICTABLE DRUG REACTION UNPREDICTABLE DRUG REACTION

(TYPE A) (TYPE B)
• Dose dependent • Dose independent
• Related to known pharmacologic actions • Unrelated to pharmacologic actions of
of the drug the drug
• Occur in patients without any unique • Occur in patients with genetic
susceptibility predisposition
• Examples: idiosyncratic reactions,
• Examples: drug toxicity /overdose, drug allergic reactions*, pseudo-allergic
interactions, adverse side effects reactions (non-lgE mediated), drug
intolerance
·Allergic
reactions
requirepriorsensitization,
manifest
withsignsandsymptoms
characteristic
of allergicmechanism
suchas
urticaria
oranaphylaxis,
andoccuringenetically susceptible
individuals

Source:KliegmanRM.et al. NelsonTextbookof Pediatrics(21sted). Elsevier;2020

EdwardsandAronson;2000

B. Gell and Coombs Classification of Hypersensitivity Reactions

• Anaphylactic or Immediate (lgE-mediated) hypersensitivity reaction

Type I
• Example: anaphylaxis, urticaria, angioedema
• Cytotoxic antibody reaction
Type II
• Example: transfusion reaction
• Immune complex reaction
Type Ill
• Example: serum sickness, post-infectious glomerulonephritis
• Delayed-type hypersensitivity reaction
Type IV
• Example: contact dermatitis
Source:KliegmanRM,et al. NelsonTextbookof Pediatrics(21sted). Elsevier;2020
EdwardsandAronson2000

II. MANIFESTATIONS
• Most common form of adverse drug reaction: cutaneous
• Most commonly implicated drugs: ampicillin, amoxicillin, penicillin, trimethoprim-
sulfamethoxazole

Ill. ALLERGICREACTIONS
• Risk Factors: prior exposure, previous reactions, age (20-49 years), route of
administration (parenteral or topical), dose (high), dosing schedule (intermittent),
genetic predisposition (slow acetylators)
• Diagnosis: skin test (most rapid and sensitive method)
• Management: desensitization for drugs in whom an alternative drug is not appropriate

342
III NON I : E MEDIATED DRUG ERUPTIONS
MANIFESTATIONS
1) Stevens-Johnson Syndrome (SJS)
• Erythematous macules
rapidly developing central
necrosis to form vesicles,
subepidermal bullae and
areas of denudation
• Involvement of 2 or more
mucosa! surfaces (eyes,
oral cavity, upper airway,
esophagus, GI tract,
anogenital mucosa)
• Affects <10% of body
surface area
2) Toxic Epidermal Necrolysis (TEN)
• Similar to SJS, but >30%
epidermal detachment
3) Drug Hypersensitivity Syndrome (DHS) or
Drug Rash with Eosinophilia & Systemic Symptoms (DRESS) Syndrome
• Severe exanthematous rash
(could become edematous,
pustular, purpuric),
exfoliative dermatitis
• Mucosa! lesions are
infrequent
• Feve1; facial edema,
eosinophilia, generalized
lymphadenopathy
4) Erythema Multiforme (EM)
• Classic lesion: doughnut-
shaped, target-like (iris or
bull's eye) pa pules with
e1ythematous outer borde1;
inner pale ring. dusky purple
to necrotic center
• Exhibits less necrosis
compared to SJS/TEN
• Most commonly affecting
extensor upper extremities
• Oral lesions may occu1; but
other mucosa! surfaces are
spared
CAUSATIVE MANAGEMENT
AGENTS
• Discontinue offending drug
• Supportive treatment:
0 Ophthalmic topical steroids
0 Mouthwashes and glycerin
swabs
• Sulfonamides, 0 Topical anesthetics
anticonvulsants, some
o Antibiotic therapy for
NSAIDs,allopurinol
secondary bacterial infection
• Also associated with
0 IV fluids
M. pneumoniae
0 Nutritional support
infection
0 Sheepskin or air-fluid bedding
0 Daily saline or Burrow
solution compresses
0 Paraffin gauze or colloidal gel
dressing of denuded areas
• Management similar to that of
severe burns
• Narcotics
• Same as SJS
• Still controversial but
with success: systemic
glucocorticosteroids, !Vig
• Anticonvulsants,
sulfonamides,
• Withdrawal of offending drug
I
minocycline,
• Systemic steroids
allopurinol
• Supportive care
• Also associated with
HHV6 reactivation
• Supportive care
• Most common
• Topical emollients, systemic
implicated etiology:
antihistamines, NSAIDs for
HSV infection
symptomatic relief
• Drug-related EM is
• Opioids for severe mucosa\
less common ( <10%),
disease
associated with
• Prophylactic oral acyclovir
NSAIDs, paracetamol,
to control recurrent HSV-
sulfonamides,
associa tee! EM
antibiotics
Source:KliegmanRM.el al. NelsonTextbookof Pediatrics(21sted).Elsevier:2020
OrimeM. J lmmunolRes:2017
343
ANAPHYLAXIS
I. ETIOPATHOGENESIS
• Serious, life-threatening systemic hypersensitivity reaction
• Triggers:
• Food (most common trigger in children)
0 Stinging insects
• Medications (most commonly antibiotics, NSAIDs,neuromuscular blocking agents)
• Diagnostic agents (radiocontrast media, fluorescein)
• Occupational allergens (bee venom, latex)
• Infants: due to inability to describe symptoms
Age-related factor • Adolescents and youn1J/dults: increased risk-taking behaviors
• Labor and delivery: ris from medications
• Elderly: increased risk of fatality
• Asthma
Concomitant • Allergic rhinitis
diseases • Atopic dermatitis
• Others: cardiovascular /respiratory /psychiatric diseases, mastocytosis
• Exercise, emotional stress
Co-factors • Acute infection
• Disruption of routine
• Premenstrual status

II MANIFESTATIONS
General • Sensation of warmth, weakness, apprehension (sense of doom)
• Generalized urticaria or angioedema
Skin or mucosa!
• Oral, periocular, or cutaneous pruritus
tissue involvement
• Flushing, swollen lips/tongue/uvula
• Dyspnea, wheeze, stridor
Respiratory
• Reduced peak PEF
compromise
• Hypoxemia
Symptoms of end- • Hypotonia/collapse, hypotension
organ dysfunction • Syncope, incontinence
Gastrointestinal • Crampy abdominal pain
symptoms • Nausea and vomiting
CNSsymptoms • Headache, altered mental status, dizziness, confusion

III. CLINICALCRITERIAFOR ANAPHYLAXIS

Any 1 of the following 3 criteria:

1. Acute onset (minutes to several hours) of skin or mucosa I tissue involvement AND at
least 1 of the following:
• Respiratory compromise
• Reduced BP or associated symptoms of end-organ dysfunction
2. Rapid occurrence (minutes to several hours) of at least 2 of the following after exposure
to a likely allergen

• Skin or mucosa! tissue involvement

• Respiratory compromise
• Reduced BP or associated symptoms of end-organ dysfunction
• Persistent gastrointestinal symptoms

3. Reduced BP following exposure to known allergen (minutes to several hours)

• Children: low age-specific systolic BP or >30% drop in systolic BP
• Adolescents and adults: systolic BP <90 mmHg or >30% drop from baseline
Source:SimonsFER,et al. WAOguidelines
for assessmentandmanagement of anaphylaxis.WAOJournal:2011
of Pediatrics(21sted).Elsevier;2020
KliegmanRM.et al. NelsonTextbook

344
IV. DIAGNOSIS

Histamine levels • Optimally obtained within 15 minutes to 1 hour of symptom onset

·Normallevelsof histamine
ortryptase
donotruleoutanaphylaxis

IV. MANAGEMENT
A. Pharmacologic Management
• First-line drug (the only medication that reduces hospitalization
and death)
• Dose: 0.01 mg/kg of a 1:1000 solution (max dose is 0.5 mg for
Epinephrine
adults and 0.3 mg for children <40 kg)
• Route: Intramuscular injection in mid-anterolateral thigh
• May be repeated every 5-15 minutes if necessary
• Diphenhydramine 1 mg/kg IV,max SO mg
Hi-antihistamine
• Chlorpheniramine 2.5-5 mg
• Ranitidine 1 mg/kg IV,max SO mg
HZ-antihistamine
• To decrease flushing, headache
• Potentially relieve protracted anaphylaxis symptoms and
prevent biphasic anaphylaxis
Corticosteroids
• Methylprednisolone 1 mg/kg IV,max SO mg
• Prednisone 1 mg/kg PO, max 75 mg
Beta 2-agonist • Salbutamol nebulization pm for bronchospasm
Source:SimonsFER,et al. WAOguidelines
forassessmentandmanagement of anaphylaxis.WAOJournal; 2011
KliegmanRM,et al. NelsonTextbook
of Pediatrics(21sted).Philadelphia:
2020

B. Non-Pharmacologic Management
Remove exposure to the trigger if possible
Place patient in supine position with elevated lower extremities if hypotensive
Establish IV access and give 20 cc/kg bolus of isotonic fluids (normal saline or lactated

0
Ringer) ifhypotensive
Oxygen support via face mask if with respiratory distress or shock
1=
Monitor patient frequently and provide a written plan for emergency management of
allergic symptoms
Patient education on avoidance of triggers, recognition of early signs of anaphylaxis,
early treatment of allergic symptoms to avoid anaphylaxis
Source:KliegmanRM,et al. NelsonTextbookof Pediatrics(21sted).Elsevier:2020
TschudyMM.et al. TheHarrietLanehandbook:Philadelphia: 2012
Chameides L. et al.AHA:2012
SimonsFER.et al. WAOguidelinesfor assessmentandmanagement of anaphylaxis.WAOJournal:2011

345
 SECTION TWO
IMMUNOLOGY

IMMUNODEFICIENCY
I. EVALUATION OF SUSPECTED IMMUNODEFICIENCY
• Most cost-effective screening test: CBC, manual differential count, ESR
Warning signs for primary immunodeficiency disorders:
;,4 new ear infections within 1 year
;,2 serious sinus infections within 1 year
;,2 months on antibiotics with little effect
;,2 pneumonias within 1 year
Failure of an infant to gain weight or grow normally
Recurrent, deep skin or organ abscesses
0 Persistent thrush in mouth or skin fungal infection
0 Need for intravenous antibiotics to clear infections
0 ;,2 deep-seated infections including septicemia
Family history of primary immunodeficiency disorder

II. CHARACTERISTIC FEATURES OF PRIMARY IMMUNODEFICIENCY

PREDOMINANT PREDOMINANT PHAGOCYTIC COMPLEMENT
T-CELL DEFECT B-CELL DEFECT DEFECT DEFECT

• After maternal
• Early onset (2-6
Onset antibodies diminish • Early onset • Any age
months)
(5-7 months)
• Fungal, viral, • Haemophilus,
• Staphylococcus, • Neisseria,
protozoa!, Streptococcus,
Pathogens Serratia, Haemophilus,
mycobacterial Pneumococcus,
Aspergillus Coccobacilli
(opportunistic) Enterococcus
• Extensive • Autoimmune
• Autoimmunity
mucocutaneous disorders
• Lymphoreticular • Prolonged
candidiasis • Meningitis,
malignancy attachment of
• Failure to thrive arthritis,
Features (lymphoma, umbilical cord
• Protracted diarrhea septicemia,
thymoma) • Poor wound
• Post-vaccination recurrent
• Post-vaccination healing
disseminated BCG sinopulmonary
paralytic polio
or varicella infections

• Serum lgA, lgG, • Absolute • CHSO (total

• Absolute
Screening and IgM neutrophil count hemolytic
lymphocyte count
tests • Isohemagglutinins • Respiratory complement
• Flow cytometry
• Antibody titers burst assay activity)

III. CHARACTERISTIC FEATURES OF PRIMARY IMMUNODEFICIENCY

DIAGNOSIS MANIFESTATIONS MANAGEMENT

1) T-Ce/1 Defect

• Cardiac defects, abnormal facies, thymic

DiGeorge anomaly/ • Thymic tissue
hypoplasia, cleft palate, hypocalcemia,
Thymic hypoplasia transplant
22qll deletions (CATCH22 syndrome)

• Impaired immune response to Candida

Chronic • Candidainfectionin skin & mucous membrane
• Systemic azole
mucocutaneous • Associated with endocrinopathy
candidiasis antifungals
(autoimmune polyendocrinopathy
syndrome type I)

Source:KliegmanRM,el al. NelsonTextbookof Pediatrics(21sled). Elsevier:2020

Woronieckaand Ballow2000
346
2) B-Cell Immunodeficiency Disorders
• Sinusitis, otitis media, pneumonia
X-linked
• Chronic fungal infections
agammaglobulinemia • !Vig administration
• Enteroviral infections: echovirus-
(XLA)/ • Judicious antibiotic use
associated myositis, chronic
Bruton disease
enterovirus encephalitis

Common variable
• Later age of onset of infections • !Vig administration
immunodeficiency
• Infections are less severe • Judicious antibiotic use
(CVID)
• Respiratory, GI, urogenital tract • !Vig is not indicated because
Selective IgA infections lgG production is normal in
deficiency • Infusions with blood products >99%, !Vig administration
containing lgA may cause anaphylaxis may result in anaphylaxis
3) Combined T-Cel/ and B-Cel/ Defect
• X-linked recessive • Bone marrow or cord blood
• Immunodeficiency with transplantation
Wiskott-Aldrich
thrombocytopenia and eczema • Supportive care (nutrition,
syndrome
• Bloody stools, prolonged bleeding !Vig, killed vaccines, platelet
from circumcision, atopic dermatitis transfusion, splenectomy)

• Most severe immunodeficiency

• Recurrent or persistent diarrhea,
pneumonia, otitis media, sepsis, skin
Severe Combined
infections, opportunistic infections • Stem cell transplantation or
Immunodeficiency
within first few months oflife gene therapy
(SCID)
• Untreated, death usually in the first
year of life and almost invariably
before 2 years of age

• Progressive cerebellar ataxia,

Ataxia-
oculocutaneous telangiectasias, chronic • Supportive care
Telangiectasia
sinopulmona,y disease, malignancy

4) Disorders of Phagocyte Function

Leukocyte adhesion
defect

Chediak-Higashi
• Delayed umbilical cord detachment,
leukocytosis, recurrent infection

• Oculocutaneous albinism,
progressive peripheral neuropathy,
increased susceptibility to infections,
• Stem cell transplantation

• High dose ascorbic acid for

stable phase
I
syndrome mild bleeding diathesis, tendency • Stem cell transplantation for
to develop hemophagocytic accelerated phase
lymphohistiocytosis

• Inability to kill catalase-positive

microorganisms due to failure of
Chronic oxidative burst (lack of NADPH
granulomatous oxidase activity] • Stem cell transplantation
disease • Abscesses, suppurative
lymphadenopathy, antral outlet
obstruction, pneumonia, osteomyelitis

5) Complement Deficiencies
• Usually asymptomatic but with risk
CZ Deficiency
of septicemia

• Specific deficiency in CS-C9 • Supportive management

Terminal
• Inability to form membrane-attack
complement
complexes
deficiency
• Systemic disease with Neisseriaspp.
~ource:Kliegman
RM,el al. NelsonTexlbook
ofPediatrics(21sled).Elsevier;2020
Madkaikar
el al. IndianPedialrics;2013
347
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29. Waserman Sand Watson W. (2011). Food allergy. Allergy, Asthma & Clinical Immunology, ?(Suppl 1), S7.
30. Wollenberg A et al. Consensus-based European guidelines for treatment of atopic eczema
in adults and children. JEADV.2018:32:657
31. Woroniecka M and Ballow M. (2000). Office evaluation of children with recurrent infection.
PediatrC\in NorthAm,47.1211-1224.
32. Zuberbier T, et al. The EAACI/GA2LEN/EDG/WAO guideline for the definition, classification,
diagnosis and management of urticaria. Allergy. 2018;73:1393-1414.

348
RH
EUMATO
LOGY
 SECTION ONE
APPROACH TO RHEUMATOLOGIC DISEASES

OVERVIEW OF RHEUMATIC DISEASES

• Rheumatic diseases result from abnormally regulated immune responses which generally
leads to inflammation of target organs
• Overlap syndromes: patients present with partial criteria that evolve over time or with
features of >l rheumatic disease

I. SYMPTOMSSUGGESTIVEOF RHEUMATICDISEASES
• Joint pains, feve1; fatigue, rash
• Joint pains without physical findings of arthritis may indicate other clinical conditions
such as fibromyalgia, growing pains, and benign hypermobility syndrome

II. SIGNS SUGGESTIVEOF A RHEUMATICDISEASE

SIGN RHEUMATIC DISEASE NON-RHEUMATIC DISEASES

• SLE, Juvenile • Sunburn, Parvovirus Bl 9,

Malar rash
Dermatomyositis (JDM) Kawasaki disease

• Herpes simplex infection,

• SLE, Behcet disease
Oral ulcers
(associated with genital ulcers)
• Vasculitis, Wegener
Purpuric rash granulomatosis, Henoch-
Schonlein Purpura (HSP)
Gottron papules • Juvenile Dermatomyositis
• Juvenile Idiopathic
Arthritis ()IA), HSP,
spondyloarthropathy,
Arthritis
vasculitis, mixed connective
tissue disease (MCTDJ,
sclerodenna, reactive arthritis
PFAPA (periodic fever; aphthous
stoma ti tis, pharyngitis, adenitis)
• Meningococcemia,
thrombocytopenia, clotting
disorders
• Psoriasis, eczema
• Postviral arthritis, reactive
arthritis, trauma, infection,
Lyme disease, Kawasaki disease,
malignancy, overuse syndrome

III. FEATURESTHAT SUGGESTA VASCULITICSYNDROME

CLINICAL FEATURES
• Fever, weight loss, fatigue of unknown origin •
LABORATORY FEATURES*
Increased ESR or CRP levels
I
• Skin lesions (palpable purpura, vasculitic • Leukocytosis, anemia, eosinophilia
urticaria, livedo reticularis, nodules, ulcers) • Antineutrophil cytoplasmic antibodies
• Neurologic lesions (headache, • Elevated factor VIII-related antigen (von
mononeuritis multiplex, focal CNS lesions) Willebrand factor)
• Arthralgia or arthritis, myalgia or myositis • Cryoglobulins
• Serositis • Circulating immune complexes
• Hypertension • Hematuria, proteinuria, elevated serum
• Pulmonary infiltrates or hemorrhage creatinine
'Diagnosticmanifestationscan take timeto develop(it is thereforeessential to recognizeclinicalpatterns). There is
no singlediagnostictest to differentiatethe variousrheumatologicdiseases.

351
APPROACH TO PATIENTS WITH MUSCULOSKELETAL PAIN/SWELLING
I. REMINDERS IN EVALUATINGMUSCULOSKELETALPAIN/ SWELLING
Observe patient from front to back and sides
Observe patient walking on his or her heels and on tiptoes
Observe the hands showing straight in front of you
Observe patient pinch index finger and thumb finger together
Ask patient to touch tips of the fingers
Ask patient to put hands together palm to palm and back to back
Observe while patient is asked to reach up while hands are raised
Ask patient to put hands behind his or her neck
Ask patient to try and touch his shoulder with his ear
Ask patient to open his mouth wide and put three fingers in the mouth
Palpate the knee
Ask patient to extend and flex the knee
Perform passive movement of hip
Ask patient to bend forward and touch his or her toes

II. DIFFERENTIAL DIAGNOSIS OF JOINT PAIN OR SWELLING IN CHILDREN

PROMINENT
SINGLE JOINT POLYARTICULAR FEATURES AND PAIN WITHOUT
INVOLVEMENT INVOLVEMENT JOINT PAIN JOINT SWELLING

• Septic arthritis • SLE • Growing pains

• SLE
• Osteomyelitis • Kawasaki syndrome • Fibromyalgia
• Dermatomyositis,
• )IA • Mixed CTD
Sarcoidosis
• Reactive arthritis • HSP
• Mixed CTD
• Trauma • Systemic vasculitis
• HSP,JIA
• Malignancy • Infection
• Reactive arthritis
• Hemophilia
• Malignancy
• Osteomyelitis

Ill. ALGORITHMIC APPROACH TO JOINT PAIN/SWELLING

Joint Pain/ Swelling

Acute Chronic
(< 6 weeks) (> 6 weeks)

Signs of Inflammation?

Inflammatory Arthritis Non-Inflammatory Arthritis

I

M.'.JnoarticularArthritis PolyartlcularArthritis

-------- --- --- ---------

Infection SLE '' Growlngpains
Osteomyelitis Kawasakisyndrome Malignancy
MCTD Osteonecrosls
HenochMSchonlein
purpura Rbromyalgia
Systemicvasculitls
Infection
'
, _ - - - - - - - - - - - - - - - - - - - _1

Source:FoslerH, el al. BestPractResClinRheumatol;2009

FosterHE,et al.ArthritisResearchCampaign;2008
Khubchandani R, et al.TheIndianJournalOfPediatrics;
2002
Kliegman
R, et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia:
Elsevier,
2020

352
 SECTION TWO
DISORDERS IN RHEUMATOLOGY

JUVENILE IDIOPATHIC ARTHRITIS (JIA)

I. ETIOPATHOGENESIS
• Idiopathic synovitis of the peripheral joints with soft tissue swelling and effusion
• Characterized by persistent daily fever (quotidian pattern for systemic onset )IA), rash,
and arthritis
• Peak incidence at 1-3 years old with female predominance

A. Three Principal Types of Onset:

0 Oligoarthritis (pauciarticular disease): 50-60%
Polyarthritis: 30-35%
0 Systemic-onset disease: 10-20%

B. Pathogenesis:
0 Two events are considered necessary for it to occur:
0 lmmunogenetic susceptibility
0 An external environmental trigger (e.g.,rubella, parvovirus, Epstein-Barr virus, antibiotics)
0 HLA types found with increased frequency in affected children
0 HLA-DR4: polyarticular disease
0 HLA-DR8 & -DRS: pauciarticular disease

II. MANIFESTATIONS
MANIFESTATIONS DESCRIPTION
• lntraarticular swelling or the presence of at least 2 of the
following:
Arthritis, morning 0 Limited range of motion
stiffness
0 Tenderness or pain on motion
0 Warmth
• Joints of the lower extremities are commonly affected, associated
Pauciarticuiar
with chronic uveitis
• Involvement of both large & small joints, more severe if extensors

I
Polyarthritis
of elbows and Achilles tendon are involved
• Quotidian fever with daily temperature spikes of 39°C for
Systemic-onset 2 weeks, faint red macular rash over the trunk & proximal
extremities, visceral involvement

III. DIAGNOSIS
A. Diagnostic Criteria
0 Age of onset <16 years
Arthritis in "1 joint
Duration of disease ;;,6 weeks
0 Onset type defined by type of disease in first 6 months:
Polyarthritis: ;;,5 inflamed joints
0 Pauciarticular /oligoarthritis: <5 inflamed joints (usually knees & ankles)
0 Systemic arthritis with characteristic fever
0 Exclusion of other forms of juvenile arthritis

353
 B. Classifications of Childhood Chronic Arthritis
ACR CLASSIFICATION ILAR CLASSIFICATION
PARAMETER
(1977) (1997)
• Juvenile rheumatoid • Juvenile idiopathic
Terminology
arthritis arthritis
Minimum duration • 2e6weeks • 2e6weeks
Age at onset • <16 years • <16 years
• Oligoarthritis
• Persistent: s4 joints for
s4 joints in 1st 6 months
• Pauciarticular course of disease
after presentation
• Extended: >4 joints
after 6 months
• Polyarticular Rheumatoid
>4 joints in 1st 6 months Factor (RF) negative
• Polyarticular
after presentation
• Polya1ticular RF positive
Presence of fever, rash,
• Systemic )RA • Systemic )IA
arthritis
• Psoriatic arthritis
Other categories
• Exclusion of other forms • Enthesitis-related a1thritis
included
• Undifferentiated
Inclusion ofpsoriatic
arthritis, inflammatory
• No • Yes
bowel disease,
ankylosing spondylitis
·ACR:AmericanCollegeof Rheumatology(ACR)
·1LR:InternationalLeagueofAssociationsfor Rheumatology(ILAR)

C. Diagnostics
DIAGNOSTIC FINDINGS/REMARKS
Markers of • Increased ESR & CRP
inflammation • Leukocytosis, thrombocytosis, anemia of chronic disease
Antinuclear antibodies
• Positive in 40-85% with pauci- and polyarticular JRA
(ANA}
• Associated with onset of the disease in an older child
Rheumatoid factor with polyarticular type
• Portends a poor prognosis
• Soft tissue swelling, osteoporosis, periostitis, narrowed
X-ray/MRI
cartilage space

IV. MANAGEMENT
• Depends on subtype, severity, specific manifestations of the illness, and response to therapy
• Mild to moderate disease (non-disabling symptoms): NSAIDs
• Moderate to severe disease (disabling symptoms): immunosuppressive agents and
disease-modifying anti rheumatic agents such as steroids, anakinra, cankinumab,
tocilizumab, and methotrexate
• Physical and occupational therapy

Source:HortonDB,et al. Pediatrics: 2015

LehmannHW.et al.ArthritisRheum:2003
PritchardMH,et al. Br J Rheumalol:1988
SullivanDB,el al.ArthritisRheum:1975
KliegmanR, el al. NelsonTextbook
of Pediatrics(21sted.).Philadelphia:Elsevier;2020

354
SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
I. ETIOPATHOGENESIS
• Autoantibody production against self-antigens resulting in inflammatory damage to organs
• Incidence of31 per 100,000 Asian children with female predominance
• Median age of diagnosis: 11-12 years old
• Fibrinoid deposits found in blood vessel walls of affected organs, skin, joints, kidneys,
blood-forming cells, blood vessels, and CNS
II. DIAGNOSTICCRITERIA FOR SYSTEMICLUPUS ERYTHEMATOSUS(SLE)
ACR CRITERIA FOR SLE
Any 4 of11 criteria are present
(serially or simultaneously, during any interval)
No distinctionis made between clinical&
immunologiccriteria in determiningwhether the
required number has been met
1) Clinical Criteria
CRITERION I DEFINITION
• Fixed e,·ythema, flat or
raised, over the malar
Malar rash eminences, tending to
spare the nasolabial
folds
• Rash from an unusual
reaction to sunlight
Photosensitivity
• By patient history or
clinician observation
Discoid rash
• Erythematosus raised
patches with adherent
keratotic scaling and
follicular plugging
• Atrophic scarring may
occur in older lesions
• Oral or nasopharyngeal
Oral ulcers ulceration, usuallypainless
• Observed by a clinician
• Nonerosive arthritis
involving 2'2
peripheral joints
Arthritis
• Characterized by
tenderness, swelling,
or effusion
I SLICC CRITERIA FOR SLE
4 of 17 criteria, including at least 1 clinical
criterion and 1 immunologic criterion; OR
biopsy-proven lupus nephritis
Criteriaare cumulative& need not be presentlyconcurrently
• Alternatively,
a patientis classifiedas havingSLE ifwith
biopsy-proven nephritisinthe presenceofANAsor anti-dsDNA
I CRITERION I DEFINITION
• Malar rash, bullous
lupus, toxic epidermal
necrolysis variant of
SLE, maculopapular or
Acute photosensitive lupus rash
cutaneous OR
lupus • Subacute cutaneous lupus
(nonindurated psoriaform
and/or annular polycyclic
lesions that resolve
without scarring]
• Classic discoid rash, localized
(above neck) discoid rash,
generalized (above and
below the neck) discoid rash,
hypertrophic (verrucous)
Chronic
cutaneous
lupus
OR
lupus, lupus panniculitis
(profundus ), mucosa!
lupus, lupus erythematosus
tumidus, chilblains lupus
I
• Discoid lupus/lichen planus
overlap
• Diffuse thinning or hair
Nonscarring
fragility with broken hairs
alopecia
(in absence of other causes)
• Palate, buccal, tongue, OR
Oral or nasal
nasal ulcers (in the absence
ulcers
of other causes)
• Synovitis involving 2'2
joints, characterized by
swelling or effusion
Joint disease OR
• Tenderness in 2'2 joints,
2'30 minutes of morning
stiffness
355
 ACR CRITERIA FOR SLE
CRITERION DEFINITION
• Pleuritis: history
of pleuritic pain or
rubbing heard by a
clinician or evidence of
pleural effusion
Serositis
OR
• Pericarditis:
documented by ECG,
rub, or evidence of
pericardia! effusion
SLICC CRITERIA FOR SLE
CRITERION DEFINITION
• Typical pleurisy for more
than one day, pleural
effusions, or pleural rub
OR
• Pericardial pain (pain with
Serositis
recumbency improved
by sitting forward) for> 1
day, pericardial effusion,
pericardia I rub, or
pericarditis by ECG

• Persistent proteinuria • Urine protein-to-creatinine

>500 mg/24 hrs or >3+
Renal disorder OR
• Cellular casts (may be red
cell, hemoglobin, granular,
tubular, or mixed)
ratio (or 24-hour urine
protein) representing 500
Renal mg protein/24 hours
OR
• Red blood cell cast

• Seizures OR psychosis
in the absence of
Neurologic
offending drugs or
disorder
known metabolic
derangements
• Seizures, psychosis,
mononeuritis multiplex,
myelitis, peripheral or
Neurologic
cranial neuropathy, OR
acute confusional state (in
absence of other causes)

• Hemolytic anemia with Hemolytic

• Hemolytic anemia
reticulocytosis anemia
OR • Leukopenia <4000/mm' at
• Leukopenia <4000 / least once
mm 3 on ~2 occasions Leukopenia or
Hematologic OR
OR lymphopenia
disorder • Lymphopenia <1000/mm'
• Lymphopenia <1500/
mm3 on 2::2occasions at least once

OR
• Thrombocytopenia
• Thrombocytopenia Thrombocytopenia
<100,000/nun' at least once
<100,000/mm'

2) Immunologic Criteria
• Abnormal titer, in
the absence of drugs
ANA ANA • Abnormal titer
associated with "drug-
induced lupus"

Anti-dsDNA
• Anti-DNA • Abnormal titer
Anti-Sm
OR
• Anti-Sm • Positive lupus anticoagulant,
false-positive test result
Immunologic OR
for rapid plasma reagin,
disorders • Positive Antiphospholipid
abnormal serum level of
antiphospholipid
anticardiolipin antibody or
antibody (Ab)
anti-beta 2-glycoprotein I
(anticardiolipin
lgG or lgM, lupus Low • Low C3, low C4 OR low
anticoagulant, or false- complement CH50
positive serologic test
for syphilis) • Positive test in the absence
Direct Coombs'
of hemolytic anemia
SLICC:SystemicLupusInternational
Collaborating
Clinics ANA:antinuclear
antibodies
Anti-Sm:
anti-Smith
antibody Anti-dsDNA:
anti-double-stranded
DNA

356
II MNEMONIC FOR REMEMBERING MANIFESTATIONS OF SLE· SOAP BRAIN MD

S erositis B load (hematologic abnormalities) M alar rash

0 ral ulcers Renal D iscoid rash

Arthritis A NA (95-99% are ANA positive)

P hotosensitivity
I mmunologic
N eurologic manifestations
Source:HochbergMC.ACRrevisedcriteriafor SLE;ArthritisRheum1997
PetriM, et al. SLICCclassificationcriteriafor SLE.ArthritisRheum:2012
TanEM,et al. 1982revisedcriteriafor the classification
of SLE.ArthritisRheum1982

Ill. MANAGEMENT
• Depends on the affected target organs & disease severity
• Serologic markers are used as guide
• Most important management tool: meticulous and frequent re-evaluation
CRITERION DEFINITION DEFINITION
• Patients who do not • NSAIDs
Mild disease have renal or other life- • Hydroxycholoroquine *
threatening involvement • Low dose glucocorticoids**

• High dose glucocorticoid** or pulse

therapy with methylprednisolone
• Hydroxycholoroquine*
• Patients with clinically
Moderate- significant organ
• Mycophenolate mofetil
Severe • Cyclophosphamide
involvement to life-
disease • Rituximab
threatening situation
• If persistent disease: methotrexate,
leflunomide, azathioprine to limit
cumulative steroid exposure
'Hydroxychloroquine(5-7 mg/kg/day)treats rash, mildarthritis,and flares, improveslipidprofiles.Potentialside
effects includeretinalpigmentationand impairedcolorvision.
"When complementlevelsrise to withinthe normalrange,steroidsare taperedover2-3 years to the lowesteffectivedose

IV. PROGNOSIS
• With progress in diagnosis and treatment, 5-year survival rate is >90%
• Mortality from infection, complications of glomerulonephritis, central nervous sytem
disease, pulmonary hemorrhage, myocardial infarction
Source:Bader-Meunier B, et al. J Pediatr:2005
HirakiLT,et al. J Pediatr:2008:PinelesD. et al. Lupus;2011 I
REACTIVE AND POST-INFECTIOUS ARTHRITIS
I. ETIOPATHOGENESIS
• Sterile inflammatory reaction in the joints following a recent infection
• Caused by generation and deposition of immune complexes and antibodies or T-cell
mediated cross-reactivity

TERM DEFINITION
• Occurs following enteropathic or urogenital infection (e.g.,
Reactive arthritis Salmonella, Shigella, Yersinia enterocolitica, Campylobacter
jejuni, Chlamydia trachomatis, E.coli, Clostridium difficile)

• Follows infectious illness not classified as reactive arthritis

Post-infectious arthritis
(e.g., group A streptococcus or viruses)

357
II. MANIFESTATIONS
• Symptoms begin 2-4 weeks following infection
• Triad of arthritis, urethritis, and conjunctivitis relatively uncommon in children
Usually oligoarticular, with predilection to the lower extremities
• Dactylitis and enthesitis is common
• Cutaneous manifestations: circinate balanitis, ulcerative vulvitis, oral lesions, erythema
nodosum, keratoderma blennorhagicum
Systemic symptoms: feve1; malaise, fatigue
• Patterns based on etiology
0 Rubella and hepatitis B affect small joints
0 Mumps and varicella affect large joints like the knee

III. DIAGNOSIS
• No specific diagnostic test
CBC,acute phase reactants, metabolic panel and urinalysis to exclude other etiologies
• Imaging findings usually non-specific or normal
Important to rule out other causes of arthritis such as septic arthritis

IV. MANAGEMENT
• Specific treatment is unnecessary for most cases
NSAIDs for pain and functional limitation
• Intra-articular steroid for refractory or severely involved joints [rule out acute infection first)

V. PROGNOSIS
Usually resolves without complications
• Reactive arthritis with C. trachomatis has potential to develop into chronic arthritis
[spondyloarthritis)
Source:KliegmanR, el al. NelsonTextbookof Pediatrics(21sted.).Philadelphia:Elsevier:2020

JUVENILE DERMATOMYOSITIS
I. ETIOPATHOGENESIS
• Most common inflammatory myositis in children
• Defined by proximal muscle weakness and characteristic rash
• Etiology is multifactorial
• HLA88, DRBl *0301, DQAl*0501 and DQAl*0301 are associated with increased susceptibility
• Pathology: inflammatory cell infiltrates result in vascular inflammation

II. MANIFESTATIONS
• Initial presentation is either rash, insidious onset of weakness, or both.
• Lipodystrophy and calcinosis associated with long-standing or untreated disease
RASHES MUSCLE WEAKNESS
• Extreme photosensitivity to ultraviolet
light (shawl sign)
• Erythema over knees and elbows
• Typically symmetric
• Heliotrope rash: blue-violet discoloration
• Proximal muscles are affected more
of eyelids
• Positive for Gower sign (use of hands on
• Facial erythema crossing nasolabial fold
thighs to stand from sitting position)
• Gottron pa pules: bright pink or pale, shiny,
• Esophageal and respiratory muscles may
thickened or atrophic plaques over the
be affected
proximal interphalangeal joints and distal
interphalangeal joints
• Periungual erythema and telangiectasia

358
III. DIAGNOSIS
AB oI1an an d Peter 1agnost1c Cnteria
Classic rash (heliotrope rash, Gottron papules) plus THREE of the following:
1. Weakness: symmetric, proximal

2. Muscle enzyme elevation (~1)

° Creatine kinase
0 Aspartate aminotransferase
0 Lactate dehydrogenase

Aldolase
0

3. Electromyographic changes;
Short, small polyphasic motor unit potentials
0

° Fibrillations
Positive sharp waves
0

Insertional Irritability
0

Bizarre, high-frequency repetitive discharges

0

4. Muscle biopsy
Necrosis
0

Inflammation
0

Source:BohanA, et al. NewEnglandJournalOf Medicine;1975

B. Other Remarks on Diagnosis

Muscle biopsy is indicated when diagnosis is doubtful
0 Amyopathic JDM or dermatomyositis sine myositis: classic rash without muscle
weakness or inflammation
Radiographic studies aid in diagnosis and management (MRI T2-weighted images with
fat suppression)

IV. MANAGEMENT
• Corticosteroids alter the course and lower mortality and morbidity
0Prednisone 2 mg/kg/day (max 60 mg daily)
0 High dose methylprednisolone for more severe cases
• Methotrexate is commonly used as a steroid-sparing agent
• Hydroxychloroquine

V. PROGNOSIS
• Around 1% mortality
• With more aggressive immunosuppressive therapy, period of active symptoms decreases
to <1.5 years
• Up to one third may need long term therapy to control symptoms
I
Source:KliegmanR, et al. Nelsontextbookof pediatrics(21sted.).Philadelphia:
Elsevier;2020
BohanA, el al. NewEnglandJournalOf Medicine;1975

359
VASCULITIS SYNDROMES
• The table below summarizes the common vasculitis syndromes encountered
• Kawasaki disease (a medium-vessel vasculitis) is discussed in Cardiology chapter
PATHOGENESIS MANIFESTATIONS DIAGNOSIS MANAGEMENT
1) Henoch Schonlein Purpura (HSP)

• Hallmark: • Mild and self-

palpable purpura limited: supportive
• Most common • Starts as small lesions • Steroids for
vasculitis of on lower extremities & significant GI
• Diagnosis is clinical
childhood buttocks (dependent involvement or
• Nonspecific
• Characterized by areas) that coalesce life-threatening
findings:
leukocytoclastic • Lesions last 3-10 days manifestations:
leukocytosis,
vasculitis & IgA • Arthritis & arthralgia Prednisone (1 mg/
thrombocytosis,
deposition in the are common (usually kg/day for 1-2
anemia,
small vessels of oligoarticular with weeks, then taper)
predilection to lower high ESR & CRP
skin, joints, GIT & • Arthritis usually
kidneys extremities) self-limited and
• Other manifestations: does not lead to
GI (80%), renal (50%) deformities

2) Polyarteritis Nodosa (PAN)

• Systemic • Mesenteric arteries:

necrotizing weight loss,
abdominal pain • Prednisone:
vasculitis of small
• Renovascular arteries:
mainstay therapy
and medium- • Diagnosis through
hypertension, • Adjunctive therapy:
sized arteries demonstration of
cyclophosphamide
• Biopsy: hematuria vessel involvement
• Others: Plasma
necrotizing • Cutaneous: purpura, on biopsy or
exchange,
vasculitis with livedo reticularis, angiography
methotrexate,
granulocytes & ulcerations, digital • Serologic test
azathioprine,
monocyte ischemia, painful for hepatitis B
mycophenolate
• Granulomatous nodules and C should be
mofetil, !VIG,
inflammation is • CNS: CVD,TIA, performed in all
thalidomide,
not present psychosis patients.
cyclosporine, anti-
• Cause unknown • Myocarditis or TNF
• Variable coronary arteritis
presentation • Arthralgias, arthritis
3) Takayasu Arteritis ("Pulseless Disease")

• Chronic large • Angiographic

vessel vasculitis abnormalities of
of unknown • Early manifestation aorta or its main
etiology are nonspecific branches and
• More common in • Hypertension at least 1 of the
Asians & headache are following:
• Average age common 0 Decreased • Glucocorticoid:
of diagnosis in • Late manifestation: peripheral artery mainstay therapy
adolescence diminished pulses, pulse and/or • Others:
• Characterized asymmetric BP, claudication methotrexate,
by inflammation claudication, 0 BP difference azathioprine
of vessel wall Raynaud between arms or • Cyclophosphamide:
starting in the phenomenon, renal legs >10 mmHg for severe disease
vasa vasorum failure 0 Bruits over the
• Giant cells & • Aortic arch disease aorta/or its
granulomatous may manifest with major branches
inflammation CNS symptoms 0 Hypertension
develop in the 0 Elevated ESR/CRP
media

360
OTHER RHEUMATOLOGIC DISEASES

PATHOGENESIS MANIFESTATIONS DIAGNOSIS MANAGEMENT

1) Ankylosing Spondylitis (AS)
• Arthritis
• Goal is to control
predominantly in • Radiographic
inflammation,
the axial skeleton studies: bamboo
minimize pain,
& hips spine sign in
preserve function
• Enthesitis advanced AS
• Complex disease and prevent
(inflammation at • MRI: gold standard
with genetic ankylosis
the site of tendon/ • Laboratory
predisposition (fusion of bones)
ligament/joint evidence of
(associated with • NSAIDs
capsule attachment inflammation (e.g.,
HLA-B27) • DMARDs (disease
to bone) ESR, CRP, RF and
modifying anti-
• Symptomatic eye ANA are negative
rheumatic drugs)
inflammation except for psoriatic
• Corticosteroids
• Gastrointestinal arthritis)
• Physical therapy
inflammation
2) Juvenile Scleroderma

• Unknown etiology Localized

• Mechanisms: Scleroderma • Based on • Deeper lesions:
vasculopathy, • Linear scleroderma: distribution systemic
autoimmunity, most common in and depth of therapy such as
immune activation childhood characteristic methotrexate &
& fibrosis Systemic Sclerosis lesion steroids
• Range of • Diffuse • Biopsy is helpful • Second line is
conditions unified • Limited (previously • No laboratory Mycophenolate
by presence of known as CREST study is diagnostic mofetil
fibrosis of the skin syndrome)
3) BehcetDisease
• Course is
• Azathioprine for
characterized by
inflammatory eye
exacerbations and
disease
remissions

• Primary variable
vessel vasculitis -
involves any size
• Most frequent
initial symptom is
painful oral ulcer
• Genital ulcers
• Recurrent oral
ulceration PLUS 2
of the following:
Eye lesions
0

Skin lesions
0
• Topical sucralfate
or steroids for
ulcers
• Colchicine
for erythema
I
• Anterior uveitis
and type of vessel Positive pathergy
0 nodosum or
(blurred vision,
• Polygenic test (pustular arthritis
redness, pain and
autoinflammatory reaction 24-48 • Other modalities:
photophobia)
disorder hours after thalidomide,
• Skin lesions:
needle puncture sulfasalazine,
etythema nodosum,
or saline injection) anti tumor
papulopustular
necrosis factor,
acneiform lesions,
cyclophosphamide,
folliculitis, purpura
interferon
and ulcers

361
 PATHOGENESIS MANIFESTATIONS DIAGNOSIS MANAGEMENT
4} Sjogren Syndrome
• Oral: recurrent
parotitis, dry
• Chronic mouth
inflammatory, • Ocular:
• Symptomatic
autoimmune disease xerophthalmia, • No established
treatment
• Progressive recurrent diagnostic criteria
• Artificial tears
lymphocytic conjunctivitis, as of the moment
• Oral lozenges
and plasma cell keratoconjunctivitis • Immunologic tests:
• Corticosteroids,
infiltration of the sicca • Anti-SSA
NSAlDs,
exocrine glands, • Other: recurrent • Anti-SSB
hydroxychloroquine,
especially salivary vaginitis • High titer ANA
methotrexate,
and lacrimal, with • Systemic: feve1; • RF
etanercept
potential for systemic non-inflammatory
manifestation arthralgia,
hypokalemic paralysis,
abdominal pain

Source:KliegmanR.el al. Nelsontextbookof pediatrics(21sted.).Philadelphia:

Elsevier:2020

REFERENCES
1. Kliegman, R., Stanton, B., St. Geme, J., Schor, N., & Behrman, R. (2016). Nelson textbook of pediatrics
(20th ed.). Canada: Elsevier.
2. Foster H, Kimura Y. Ensuring that all paediatricians and rheumatologists recognise significant
rheumatic diseases. Best Pract Res Clin Rheumatol. 2009 Oct:23(5):625-42.
3. Foster HE, Jandial S. pGALS-A Screening Examination of the Musculoskeletal System in School-Aged
Children. Reports on the Rheumatic Diseases (Series SJ, Hands On 15. Arthritis Research Campaign;
2008 June. Copyright© 2008 Arthritis Research Campaign.
4. Khubchandani, R., & D'Souza, S. (2002). Initial evaluation of a child with arthritis-An algorithmic
approach. The Indian Journal Of Pediatrics, 69(10), 875-880. doi: 10.1007 /bf02723712
5. Horton DB, Scott Fl, Haynes K, Putt ME, Rose CD, Lewis JD, Strom BL. Antibiotic Exposure and
juvenile idiopathic Arthritis: A Case-Control Study. Pediatrics. 2015 Aug;136(2):e333-43. Epub
2015 Jul 20.
6. Lehmann HW, Knoll A, Kuster RM, Modrow S. Lehmann HW, Knoll A, Keister RM, Modrow S. Arthritis
Rheum. 2003;48(6):1631.
7. Pritchard MH, Matthews N, Munro). Antibodies to influenza A in a cluster of children with juvenile
chronic arthritis. Br) Rheumatol. 1988;27(3):176.
8. Sullivan DB, Cassidy )T, Petty RE. Pathogenic implications of age of onset in juvenile rheumatoid
arthritis. Arthritis Rheum. 1975;18(3):251.
9. Bader-Meunier B, Armengaud )B, Haddad E, et al. Initial presentation of childhood-onset systemic
lupus erythematosus: a French multicenter study) Pediatr 2005; 146:648.
10.Hiraki LT, Benseler SM, Tyrrell PN, et al. Clinical and laboratory characteristics and long-term
outcome of pediatric systemic lupus erythematosus: a longitudinal study.) Pediatr 2008; 152:550
11.Hochberg MC. Updating the American College of Rheumatology revised criteria for the
classification of systemic lupus erythematosus (letter]. Arthritis Rheum 1997; 40:1725
12.Lupus. 2011 Oct;20(11):1187-92. Epub 2011 Jul 18. Pediatrics. 1989;83(2):235.
13.Petri M, Orbai AM, Alarcon GS, et al. Derivation and validation of the Systemic Lupus International
Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum
2012; 64:2677.
14.Pineles D, Valente A, Warren B, Peterson MG, Lehman T), Moorthy LN. Worldwide incidence and
prevalence of pediatric onset systemic lupus erythematosus. Lupus. 2011 Oct;20(11):1187-92.
Epub 2011 Jul 18.
15.Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus
erythematosus. Arthritis Rheum 1982; 25:1271.
16.Bohan, A., & Peter, J. (1975). Polymyositis and Dermatomyositis. New England journal Of
Medicine, 292, 403-407.
362
CARDIOLOGY
QJ OVERVIEW OF THE CIRCULATORY SYSTEM
I. Fetal Circulation
II. Transition at Delivery

0 APPROACH TO COMMON CARDIAC COMPLAINTS

I. Approach to Chest Pain
II. Approach to Cyanosis
Ill. Approach to Common Pediatric Cardiac Murmurs

~ CONGENITAL HEART DISEASES

I. Overview of Congenital Heart Diseases
II. Acyanotic Congenital Heart Diseases
Ill. Cyanotic Congenital Heart Diseases
IV. Obstructive Lesions

~ ACQUIRED HEART DISEASES

I. Rheumatic Fever
II. Valvular Heart Disease
Ill. Infective Endocarditis
IV. Kawasaki Disease
 SECTION ONE
OVERVIEW OF THE CIROU.LATORY SYSTEM

FETAL CIRCULATION
• Prior to delivery, the fetus depends on the placenta for gas/nutrient exchange
• The low vascular resistance of the placenta and the high vascular resistance of the fetal
lungs result in right-to-left shunts characteristic of the fetal circulation:
Foramen ovale: blood shunted from right atrium (RA) to left atrium (LA)
0 Ductus arteriosus: blood shunted from the pulmonary artery (PA) to the aorta

I. OVERVIEW OF THE FETAL CIRCULATION

NormalFetalCirculation

Pulmon!ryartery\
/ Lungs
i
Rightjventricle Pulmon!ryveins DuctusAjrteriosus
Leftatrium

Ft°ramen
ovare teft ventricl!

Rightatrium Aorta

Inferiorv}na cava j
Ouctusvenosus

Umbilical
t
vein - Placenta- Umbilical
arteries

Flow of blood in the fetus. Blood is first oxygenatedin the placenta,then returnsto the fetus via the um-
bilicalvein. Somebloodgoes into the hepaticcirculation/liver (50%),while the rest bypassthe liver via the
ductusvenosusand entersthe inferiorvena cava (IVC)then the right atrium(RA). Fetalsuperiorvenacava
bloodalso drains into the RA. From the RA, most blood bypassesthe right ventricle(RV) via the foramen
ovaleto enter the LA, then the left ventricle(LV),then the aorta towardsthe fetal tissues(i.e., systemiccir-
culation). From the RA, some bloodmay enter the RV,then the pulmonarytrunk (PT). From the PT, some
blood may enter the lungs, but most would bypassthe lungs via the ductus arteriosus(DA), then to the
aorta & systemiccirculation. Deoxygenatedbloodgoes back to the placentavia the two umbilicalarteries.
I
II. FETAL CARDIAC OUTPUT
• The fetal heart is unable to increase stroke volume when the heart rate (HR) falls because
it has low compliance
• Thus, the fetal cardiac output (CO) depends on HR; when the HR drops, the result is a
serious fall in CO

365
III. STRUCTURES IMPORTANT TO MAINTAIN PARALLEL CIRCULATION
FORAMEN OVALE DUCTUS ARTERIOSUS
DUCTUS VENOSUS
(FO) (DA)
• Precardiac (venous) • Postcardiac (arterial)
Location • lntracardiac shunt
shunt shunt
• Between left • Between pulmonary
Anatomy • Between LA & RA
umbilical vein & IVC artery & aorta
• Pulmonary circuit is
• Blood from IVC
bypassed due to high
directly flows to LA
pulmonary vascular
due to valves in IVC
resistance (PVR)
• Bypasses portal • No mixing of
Physiology • Less oxygenated blood
circulation deoxygenated
in PA flows through the
blood from SVC
open DAto descending
• Most of SVCblood
aorta then to placenta for
goes to the RV
oxygenation
• Within 48 hours of birth
• During umbilical • During 1" breath, (10-15 hours in a normal
Functional cord clamping septum primum neonate)
closure • Removal of placenta is pressed against • Occurs by constriction of
results in its closure septum secundum medial smooth muscles in
the DA
• 15-20 days when • 1 yea1; when there
Anatomic • At 2-3 weeks when there
there is proliferation is fusion of 2
closure is intimal proliferation
of obliterating tissue apposed septa
Remnant • Ligamentum venosum • Fossa ovale • Ligamentum arteriosus

TRANSITION AT DELIVERY
• The neonate must rapidly make physiologic changes when the umbilical cord is clamped
at birth (transition from intrauterine to extra uterine life)
• Successful transition involves:
Alveolar fluid clearance
0 Lung expansion with the first effective breath
Increases in pulmonary perfusion and systemic pressure
° Closure of the right-to-left shunts of the fetal circulation

Changes in the Circulation after Birth:

Primary change after birth is a shift of blood flow for gas exchange from the placenta
to the lungs
Interruption of the umbilical cord results in the following:
0 An increase in systemic vascular resistance due to removal of the low-resistance placenta
° Closure of the ductus venosus as a result of lack of blood return from the placenta
Lung expansion results in the following:
0 Reduction of the PVRleads to an increase in pulmonary blood flow and a fall in PApressure
Functional closure of the foramen ovale occurs due to increased pressure in the LA
Increased arterial oxygen saturation
0 A postnatal increase in 0 2 saturation of the systemic circulation is the strongest
stimulus for constriction of the ductal smooth muscle which leads to closure of the
ductus arteriosus (the patency of the ductus arteriosus is maintained by low 0 2
saturation and high levels ofprostaglandins)
0 The responsiveness of the ductal smooth muscle to 0, is related to the gestational
age of the newborn (ductal tissue ofa premature infant responds less intensively to
oxygen than that of a full-term infant)

SourceFernandesCJ,et al. Physiologictransitionfromintrauterineto extrauterinelife UpToDate;2019

KliegmanR. et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia: Elsevier:2020
Park,M. PediatricCardiologyfor Practitioners
(5thed.).Philadelphia: MosbyElsevier:2008
366
 SECTION TWO
APPROACH TO COMMON CARDIAC COMPLAINTS
APPROACH TO CHEST PAIN
DIFFERENTIALS REMARKS
Cardiac Etiology
• Pleuritic-type of pain relieved by sitting up and referred to the neck,
shoulders, and abdomen
Pericarditis • Unable to assume supine position
• Pericardia! friction rub on supine position
• ECGshows di fuse ST-segment elevation

Arrhythmias • Due to inadequate coronary blood flow

Mitra! valve • Vague anterior chest pain

prolapse • Mid-systolic click and late systolic murmur
• Rare in childhood, usually associated with connective tissue disease
Aortic dissection
• Severe pain, sudden in onset, "tearing" in quality, with radiation to the back

Coronary artery • Extremely rare in pediatric age group

disease • Myocardial ischemia, angina pectoris, myocardial infarction

Non-Cardiac Etiology

• Most common cause of chest pain

Musculoskeletal • Tietze's syndrome (costochondritis)
• Precordial catch syndrome (intercostal muscle cramping)
• Hyperventilation or anxiety
Psychogenic
• Hysterical behavior
• Pleuritic in nature exacerbated by deep inspiration or coughing
Pulmonary • Inflammation or irritation of the pleura
• Pneumonia, pleurodynia, pneumothorax
• GER, esophagitis, gastritis, GI spasm
Gastroesophageal
• Usually described as "burning" pain

APPROACH TO CYANOSIS
• Bluish purple discoloration of tissues due to an increased concentration
hemoglobin in the capillary bed

CENTRAL CYANOSIS
of deoxygenated

• Due to decreased 0 2 saturation
Mechanism
(<85%) or high deoxygenated Hgb
Area involved • Generalized (e.g., lips, conjunctiva)
PERIPHERAL CYANOSIS
I
• Due to diminution of blood flow
• Localized (e.g., distal extremities)

Affected part • Wann • Cold

Response to
• No improvement • With improvement
warming
Clubbing • Usually present • Absent
Capillary refill • < 2 seconds • > 2 seconds

• Cardiac shunts (e.g., TOF) • Cold exposure

Examples • Lung disease • Obstruction to blood flow
• Hemoglobin abnormalities • Low cardiac output

367
APPROACH TO COMMON PEDIATRIC CARDIAC MURMURS
• Murmurs are common in healthy children & most are not pathologic ("innocent murmurs")
• A murmur may also be the sole finding in children with structural heart disease

I. INNOCENT MURMURS
Result from normal patterns of blood flow through the heart and vessels
Seven S's of innocent murmurs:
Sensitive (changes with position or respiration)
Short duration (not holosystolic)
Single (no associated clicks or gallops)
Small (small area and nonradiating)
Soft
Sweet (not harsh)
Systolic (limited to systole)
MURMUR DESCRIPTION
Aortic systolic
• Systolic ejection murmur over the aortic valve
murmur
• High pitched systolic murmur (can extend into diastole) best heard
Mammary
along the anterior chest wall over the breast
artery souffle
• Caused by blood flow in arteries & veins leading to and from the breasts

Pulmonary • Crescendo-decrescendo, systolic murmur peaking in mid-systole

flow murmur • Best heard at the left sternal border between 2nd & 3rd lCS
• Early systolic murmur with a vibratory or musical quality
Still's murmur
• Best heard at the lower left sternal border
• Continuous murmur, accentuated in diastole
Venous hum
• Whining, roaring, or whirring quality, best heard over low anterior neck

II. RED FLAGS THAT INCREASE LIKELIHOOD OF A PATHOLOGICMURMUR

• Holosystolic or diastolic murmur
• Grade 3 or higher murmur
• Harsh quality
• Abnormal S2
• Maximal murmur intensity at the upper left sternal border
• Systolic click
• Increased intensity when patient stands

Systolic Ejection Murmurs

DIFFERENTIALS DESCRIPTION USUAL LOCATION
Atrial septa( • Systolic ejection murmur with • 2"" left intercostal space (ICS)
defect wide split (fixed) Sl with a widely split S2
• Systolic ejection murmur • 2"' left ICSwith radiation to the
Pulmonic stenosis
• Systolicejectionclick may be heard upper back
• Systolic ejection murmur with • 2"' right ICSwith radiation to the
Aortic stenosis
radiation to the carotid arteries upper back
Coarctation of the • Systolic ejection murmur with • 3"'-4'' left ICSwith radiation to
aorta normal Sl and S2 interscapular area

Systolic Regurgitant Murmurs

Ventricular Septa I
• Left lower sternal border (LLSB)
Defect
• Holosystolic murmur
Mitra! • LLSBwith radiation to the left
Regurgitation anterior axillary line (LAAL)
Source:Kliegman
R. et al. NelsonTextbook of Pediatrics
(21sted.).Philadelphia:
Elsevier;2020
Park,M. PediatricCardiologyfor Practitioners
(6thed.).Philadelphia:
MosbyElsevier;2014
FrankJE,et al.AmFamPhysician; 2011
368
 SECTION
CONGENITAL HEART DISEASES
OVERVIEW OF CONGENITAL HEART DISEASES
MANIFESTATIONS
Left to Right Shunts
• Usually asymptomatic
ASD • Widely split and fixed S2
[Secundum) • Systolic ejection
murmur at LUSB
• Holosystolic murmur in
VSD
left parasternal border
• Continuous/machinery
PDA
murn1ur
Right to Left Shunts
• Single & loud SZ
• No murmur if with
TGA
intact ventricular
septum
• Systolic ejection
TOF murmurat 2nd LUSB
• Loud & single SZ
• Systolic regurgitant
Tricuspid
murmur at left lower
Atresia
sternal border LLSB
• Systolic murmur at LSB
TAPVR
• Murmurs may be absent
Obstructive Lesions
• Weak femoral pulses
Coarctation
• BP in arms higher than
of the aorta
in the legs
• Systolic ejection
Pulmonic
murmur at LUSB with
stenosis
radiation to upper back
Aortic • Systolic ejection
stenosis murmur at RUSB
ASD:Atrialseptaldefect VSD:Ventricular
PDA:Patentductusarteriosus TGA:Transposition
TOF:Tetralogyof Fallo! TAPVR:Totalanomalous pulmonaryvenousrelurn
Source:Kliegman
R, et al. NelsonTextbook of Pediatrics
Park.M.PediatricCardiology for Practitioners
THREE
DIAGNOSTICS
• RA & RV enlargement on
CXR (chest radiograph)
and ECG
• LVHor biventricular
enlargement on CXR& ECG
• Prominent PA.enlarged LA
and LV on CXR
• LVH or biventricular
hypertrophy on ECG
• Egg-shaped heart on CXR
Boot-shaped heart on CXR
[couer en sabot)
• Hypoplastic RV
• LAD & LVH in ECG
• Snowman sign or figure of
8 on CXR
• Rib notching on CXR
• Uplifting of apex and
normal or decreased
pulmonary vascularity
on CXR
• Prominent ascending
aorta with a normal aortic
knob on CXR
septaldefect
of thegreatarteries
(6thed.).Philadelphia:
369
MANAGEMENT
• Depends on type of
shunt, its symptoms
and/or size
• Transcatheter closure
generally preferred
for ASD secundum,
muscular VSD, and PDA
• Rashkind procedure
[palliative)
• Jateneprocedure
(arterial switch)
• Atrial switch [e.g.,
Senning, Mustard)
• Blalock:raussig
procedure [palliative)
• Complete repair oFTOF
• Palliative [e.g., Rashkind,
PDA stcnt)
• Corrective repair: Glenn
shunt followed by Fontan
procedure
• Van Praagh procedure
• Primary re-anastomosis
I
or a patch aortoplasty
• Depends on symptoms &
severity of stenosis
• Balloon valvuloplasty
• Valvotomy [Brock
procedure)
• Depends on symptoms &
severity of stenosis
• Balloon valvuloplasty
• Ross procedure (valve
translocation)
RNLA:RighUlett
atrium
RVILV:RighVlett
ventricle
PA:Pulmonaryartery
(21sted.).Philadelphia:
Elsevier:
2020
MosbyElsevier;2014
ACYANOTIC CONGENITAL HEART DISEASES
I. ATRIALSEPTALDEFECT(ASD)
A. Etiopathogenesis
0 ASDs can occur in any portion of the atrial septum (secundum, primum, or sinus venosus)
More common in females
1. Effects of an ASD in the heart
In large defects, oxygenated blood flows from LA to RA, which is added to the usual
venous return to the RA and is pumped by the RVto the lungs
Enlargement of the right sided chambers of the heart (RA, RV,PA)
LA is usually not enlarged
Pulmonary vascular resistance may begin to increase in adulthood

2. Types of ASD
TYPE REMARKS
Ostium • Most common
secundum • Defect present at the site of fossa ovalis
• Defect situated in the lower portion of the atrial septum and overlies
Ostium the mitral & tricuspid valves
primum • AVsepta! defect (AVcanal defect or endocardial cushion defect): consists of
contiguous atrial and VSDs with markedly abnormal AVvalves
Sinus • Defect situated in the upper part of the atrial septum close to the
venosus entry of the SVCor IVC(more common at SVC)

Sinus venosus (SVC type)

Septum Secundum ASD

Sinus venosus (IVC type)

B. Manifestations of ASD Secundum

• Usually asymptomatic (especially if small ASD secundum)
• Subtle failure to thrive may be present for more significant shunt
Symptoms
• Eisenmenger physiology (reversal of flow) may occur for uncorrected
large ASDs
• Left precordial bulge and RVsystolic lift may be present
• Widely split S2: characteristic finding in ASD
Signs
• Systolic ejection murmur at the left middle and upper sternal border
due to increased flow across RV outflow tract into the PA

• Rate of spontaneous closure 87% (those >8 mm rarely close)

• Untreated large defect leads to congestive heart failure (CHF) and
Natural
pulmonary hypertension in adults
Course
• Eisenmenger syndrome: a long-standing left-to-right shunt causes
pulmonary hypertension & reversal into a right-to-left (cyanotic) shunt

370
 Chest • Enlarged RV,RA, and PA
Radiograph • Increased pulmonary vascularity
• Volume overload of the RV
ECG
• Normal or right axis deviation of the QRS
• RVoverload:
0 Increased RVend-diastolic dimension
° Flattening or reversed (anterior) motion of the ventricular septum
2D-echo
• Location and size of atrial defect seen
• Transesophaeal echocardiogram: recommended imaging modality
to delineate more accurate measurement/size of the defect

Cardiac • Confirms the presence of the defect

Catheterization • For measurement of shunt ratio & pulmonary pressure/resistance
Cardiac MRI • Can be used to determine the presence, dimensions & margins &
(CMR) degree of shunting and effects on right heart volume & cardiac function

D. Management of ASD Secundum Type:

Percutaneous catheter device closure using an atrial septa! occlusion device
Open heart surgery to patch large defects (if not amenable for percutaneous closure)
Timing of closure: may proceed after 1 year old if symptomatic or before entry to school
Indications for closure:
0 Symptomatic patients
0 Asymptomatic patient with Qp:Qs ratio (pulmonary vs. systemic blood flow) of at
least 2:1 or if with RVenlargement
In small secundum ASDs and minimal left-to-right shunts without RVenlargement,
closure is not required

II. VENTRICULAR SEPTAL DEFECT (VSD)

A. Etiopathogenesis
0 Most common congenital heart disease (approximately 25% of congenital heart diseases)
May occur in any portion of the ventricular septum, but most are of the membranous type

1. Effects of a VSD on the Heart

Because the shunt occurs mainly during systole when the RValso contracts, the
shunted blood goes directly to the PA rather than remaining in the RV cavity

I
Enlargement of the LA, LV,and main PA
Size of the VSD is a major determinant of the magnitude of the left-to-right shunt

TYPE REMARKS
• Small communication is present (usually <5 mm)
Restrictive VSD
• RVpressure is normal
Large • Usually >10 mm
nonrestrictive VSD • Right and left ventricular pressures are equalized

371
 2 T ofVSD
TYPE REMARKS
• Beneath the aortic valve
Membranous
• Most common type is perimembranous VSD (70% of all VSDs)
Outlet • 5%-7% ofVSDs
(infundibular • Defect located within the outlet (canal) septum
or conal) • The aortic and pulmona1y annulus forms part of its rim
• 5%-8% ofVSDs
Inlet (AV canal) • Located posterior and inferior to the perimembranous defect,
beneath the septa I leaflet of the tricuspid valve

Trabecular • 5%-20% ofVSDs

(muscular) • Usually multiple defects

Outlet/Supracristal/Subpulmonic/
lnfundibular/Juxta-arterial VSD

Perimembranous/Paramembranous/
Conoventricular VSD

Inlet/AV Canal type VSD

B. Manifestations
• Small VSD (most common): asymptomatic with normal growth &
development
Symptoms
• Moderate to large VSD: delayed growth & development, decreased
exercise tolerance, repeated pulmonary infections, heart failure
• Systolic regurgitant murmur at the LLSB
Signs • Intensity of P2 is increased with a large shunt
• S2 is loud and single in pulmonary hypertension
• Spontaneous closure occurs in 40-50% of membranous and 90% in
Natural
muscular VSD (especially with small defects)
Course
• Eisenmenger syndrome

372
C. Diagnosis
DIAGNOSTIC SMALL VSD LARGEVSD
• Usually normal
• Cardiomegaly with prominence of both
• May have minimal
Chest ventricles, LA,and pulmonary artery
cardiomegaly &
Radiograph • Increased pulmonary vascular markings
borderline increase in
• Frank pulmonary edema may be present
pulmonary vasculature
• Biventricular hypertrophy
• Generally normal, but • Notched or peaked P waves
ECG
may suggest LVH • Presence of RVHsuggests a larger VSD
with pulmonary hypertension
• Shows the position and size of the VSD
2D Echo • Measures degree of volume overload of the LAand LV
• Estimated pulmonary artery pressure may be obtained
• Normal right heart • Equal or nearly equal pulmonary and
Cardiac
pressures & pulmonary systemic systolic pressure
Catheterization
vascular resistance • Elevated pulmonary vascular resistance
• Visualizes flow, defect position & size, ventricular volume &
Cardiac MRI
function, and net shunt quantification (arterial flow)

D. Management ofVSD
l. Management Depending on the Size
• Parents are reassured of the relatively benign nature of the VSD
Small VSD • Surgical repair is not recommended
• Infective endocarditis prophylaxis
• Management goals: control symptoms of heart failure and
prevent development of pulmonary vascular disease
LargeVSD
• Severe pulmonary vascular disease nonresponsive to pulmonary
vasodilators is a contraindication to repair an uncorrected large VSD

2. Treatment Modalities
• Indications for surgical closure:
0 Patients at any age with large defects when symptoms & failure
to thrive cannot be controlled medically
Surgery
0 Infants 6-12 months of age with large VSD& pulmonary
hypertension, even if symptoms are controlled by medication

Transcatheter
Closure
0 Patients >24 months with a Qp:Qs ratio >2:1
• Most successful for muscular VSDs
• Small sized perimembranous type VSDs with no associated aortic
valvar pathology
I

373
III. PATENTDUCTUSARTERI0SUS (PDA)
A. Etiopathogenesis
0 Persistent patency of a normal fetal structure between left PA & descending aorta
° Common in premature infants
More common in females
Effects of a PDA on the heart:
Blood shunts left-to-right through the ductus due to a higher aortic pressure
0 Enlarged LA, LV,main PA
Enlarged aorta, which also handles an increased amount of blood flow

B. Manifestations
• Small PDA: usually asymptomatic
Symptoms
• Large PDA: heart failure, growth retardation
• Small PDA: normal peripheral pulses, normal sized heart
• Large PDA: bounding peripheral arterial pulse, wide pulse
Signs pressure, cardiomegaly, prominent apical impulse
• Tachycardia & dyspnea due to volume overload on LA & LV
• Continuous machinery-like murmur at 2"• left ICS or LSB
• Spontaneous closure of ductus after infancy is rare
Natural Course • Small PDA: few complications, but late manifestations may occur
• Large PDA: heart failure occurs early in infancy

C. Diagnosis
DIAGNOSTICS REMARKS/FINDINGS
• Prominent PA with increased pulmonary vasculature
Chest • Enlarged LA and LV(depending on degree of the shunt)
Radiograph
• Aortic knob may be normal or prominent
• Small PDA: normal ECG
ECG
• Large PDA: LVHor biventricular hypertrophy
• Small PDA: normal sized cardiac chambers
20
Echocardiography • Large PDA: enlarged LA and LV
• Direct visualization of PDA

Cardiac • May demonstrate normal or increased pressures in the RV and PA

Catheterization ( depending on the size of the PDA)
Cardiac CT • For those PDA with tortuosity
Cardiac MRI • Can give information regarding volumes and flow

D. Management: Surgical or Transcatheter Closure

0 Rationale for closure in small PDA: to prevent bacterial endarteritis or other late
complications
Indication for closure in larger PDA: to treat heart failure and prevent pulmonary
vascular disease

R.et al.NelsonTextbook
Source:Kliegman of Pediatrics
(21sted.).Philadelphia:
Elsevier;
2020
Park,M. PediatricCardiology for Practitioners
(6thed.).Philadelphia:
MosbyElsevier;2014
M. Fogelet al. Principles
andPracticeof CMRin CHOForm.Function. andFlow:2010

374
CYANOTIC CONGENITAL HEART DISEASES
DECREASED PULMONARY BLOOD FLOW INCREASED PULMONARY BLOOD FLOW
• Tetra logy of Fallot (TOF) • D-Transposition of the great arteries
• Pulmonary atresia • Total anomalous pulmonary venous return
• Tricuspid atresia • Truncus arteriosus

I. D-TRANSPOSITION OF THE GREAT ARTERIES

A. Etiopathogenesis
Systemic veins return normally to the RA, pulmonary veins return to the LA
° Connections between atria and ventricles are also normal (atrioventricular concordance)

• Aorta arises from the right ventricle (canying desaturated blood to the body)
Abnormality
• Pulmona1y a1te1y arises from the LV(canying oxygenated blood to the lungs)
• Complete separation of pulmonary & systemic circulations, leading to
hypoxemic blood circulating throughout the body & hyperoxemic blood
Effect circulating in the pulmonary circuit
• Defects that permit mixing of the two circulations (e.g., ASD, VSD, PDA)
are needed for survival

B. Manifestations
• Cyanosis from birth
Symptoms
• Signs ofCHF with dyspnea & feeding difficulties during newborn period
• Single & loud SZ
• No hea1t murmur is heard in infants with an intact ventricular septum
Signs
• Severe arterial hypoxemia with or without acidosis (hypoxemia
unresponsive to oxygen inhalation)

C. Diagnosis
DIAGNOSTICS REMARKS/FINDINGS
Chest • Egg-shaped cardiac silhouette with a narrow, superior mediastinum
Radiograph • May show cardiomegaly & normal to increased pulmonary blood flow
ECG • Usually normal, showing the neonatal right-sided dominant pattern
Echocardiography • Diagnostic and confirmatory
Hyperoxia test • Arterial PO2 is low & does not rise after breathing of 100% oxygen
• Indicated if noninvasive imaging is diagnostically inconclusive, if
Cardiac
Catheterization

D. Management
unusual coronary artery anomaly is suspected, or if emergency
balloon atrial septostomy (Rashkind procedure) is required
I
ASPECT MANAGEMENT
• Prostaglandin El infusion: to maintain patency of the ductus
arteriosus
Palliative
• Create interatrial communication by balloon atrial septostomy
(Rashkind procedure) or atrial septostomy (Blalock-Hanlon procedure)
• Surgically switch right- & left-sided blood at three levels:
0 Atrial level (Senning or Mustard)
Definitive
0 Ventricular level (Rastelli)
0 Great artery level (latene, the surgical treatment of choice)

Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Philadelphia:

Elsevier;2020
Park,M. PediatricCardiologyfor Praclitioners
(6thed.).Philadelphia:
MosbyElsevier:2014

375
II. TETRALOGY OF FALLOT(TOF)
A. Etiopathogenesis
1. Anatomic Findings Characteristic ofTOF [mnemonic: PROVe)
Pulmonary stenosis (PS) or obstruction to RV outflow
Right ventricular hypertrophy
Overriding aorta (deviation of the origin of the aorta to the right)
Ventricular septa! defect

2. Pathophysiology
• Primary defect: the infundibular septum (the muscular septum that separates the
aortic & pulmonary outflow tracts) is anteriorly & superiorly deviated, resulting in:
Defect in the ventricular septum
0 Pulmonary stenosis by blocking flow to the PA,which leads to increased pressure
on the right side of the heart & RVH
Right to left shunting is increased when pressures on the left are decreased
There is absence of pulmonary congestion due to severe PS and reduced pulmonary
venous return to the left side of the heart

8. Manifestations
• Varying degrees of cyanosis, tachypnea, clubbing
• Cyanosis is not often present at birth, but with increasing RVhypertrophy
Symptoms in the infundibulum, cyanosis occurs later in the 1" year of life
• Timing of onset of symptoms, severity of cyanosis, and the degree of
RVHis determined by the degree of RVoutflow obstruction
• RVtap along the left sternal border
Signs • Systolic thrill along the left sternal border in the 3••-4•hparasternal spaces
• Loud systolic ejection murmur at mid- & ULSBwith radiation to the back
• Activities that suddenly lower the systemic vascular resistance (e.g., crying,
feeding, or defecation) initiate the spell, leading to an increase in cyanosis
Hypoxic
• Sudden onset of tachycardia or hypovolemia can cause the spell
or
TetSpells • Paroxysm of hyperpnea, irritability, prolonged crying, & increasing cyanosis
• Decreased intensity of the murmur because flow across the right
ventricular outflow tract (RVOT)diminishes
• Polycythemia develops
Natural • Growth retardation if cyanosis is severe
Course • Brain abscess & cerebrovascular accident (due to cerebral thrombosis)
• Coagulopathy is a late complication

:
DIAGNOSTIC REMARKS/FINDINGS
Chest • Decreased pulmonary vascular markings
Radiograph • Concave main PAwith an upturned apex (boot-shaped heart)
• RAD,RVH,and RAE (tall and peaked P wave)
ECG
• Dominant R wave in the right precordial chest leads
Echocardiography • Establishes the main anatomy
• Helpful for coronary anomalies
Cardiac CT • Advantage is the ability to delineate major aortopulmonary
collateral arteries difficult to visualize in echocardiogram
Cardiac MRI • Highly reproducible quantification of RVvolumes
• Necessary in TOF patients with pulmonary atresia to image the
Cardiac
source of blood supply to and size of each lung segment
Catheterization
• Markedly decreased PA pressure
Source:KliegmanRM.et al. NelsonTextbook of Pediatrics(21sted);2020
Park,M. PediatricCardiologyfor Practitioners
(6thed.).Philadelphia:
MosbyElsevier:2014
ChelliahA, et al. CurrentCardiovascularImagingReports;2019
376
 D Management
• Maintain good dental hygiene & antibiotic prophylaxis against
General subacute bacterial endocarditis (SBE)
Management
• Phlebotomy & volume replacement for symptomatic polycythemic patients

• Addressing tet spells should be done in sequence:

° Knee-chest position
0 Oxygenation

0 Morphine sulfate at 0.2 mg/kg/dose subcutaneously suppresses the

respiratory center & abolishes hyperpnea
Management 0 NaHC0
3 1 mEq/kg IV (for metabolic acidosis)
of Tet Spells
• Other medications
0 Phenylephrine (0.02 mg/kg IV) to raise the SVR, or
0 Propranolol (0.01-0.25 mg/kg slow IV) may stabilize vascular reactivity of

the systemic arteries preventing a sudden decrease in SVR,or

° Ketamine (1-3 mg/kg IV over 60 secs) increases SVR
Source:KliegmanR, el al. NelsonTextbookof Pediatrics(21sted.).Philadelphia:Elsevier:2020
Park,M. PediatricCardiologyfor Practitioners(6th ed.).Philadelphia:MosbyElsevier:2014

E. Corrective Management for TOF

• Blalock-Taussig shunt: systemic-to-pulmonary artery shunt to
Palliative augment pulmonary artery blood flow
Procedure • RVOT stenting for severe infundibular stenosis/narrowing
• PDA stenting

Corrective • Consists of surgical procedures focused to relieve the extent of RVOT

Surgery obstruction and to close the large VSD by patch

OTHER CYANOTIC CONGENITAL HEART DISEASES

PATHOGENESIS MANIFESTATION MANAGEMENT
1) Truncus Arteriosus {TA)
• TAis the emb,yologic precursor to • Highpulmona1y flow:increases • Rastelli Repair
the aorta & pulmonary artery a1terial 0 2 saturation, which ° Closure ofVSD
• Both ventricles eject blood into giveslesscyanosis 0Pulmona1yarte1ies
a common vessel • Low pulmona1y flow: mixing are separated from the
• A VSDis always present of R & L ventricular outflow, truncus
• Type I (main pulmona1y is present leading to central cyanosis & ° Continuity is
& bifurcates) is most common earlier CHF established between
• Manifestations depend on • Single S2 produced by the the RVand the PAwith
amount of pulmonary flow truncal valve
2) Total Anomalous Pulmonary Venous Return {TAPVR)

• All four pulmonary veins drain

a conduit

to the RA
• Supracardiac type (drains to a
vertical vein connected to the
SVCdraining to the RA) is most
common
3) Single Ventricle (Double-Inlet Ventricle, Univentricular Heart)
• Timing of surge1y
determined by presence
I
• RVvolume overload
or absence of pulmona1y
• "Snowman sign" on CXR(for
venous obstruction
supracardiac type TAVR)
• Common pulmonaty
venous trunk anastomosed
directly to the LA

• Depends on associated • Palliative management

• Both atria empty through a
intracardiac anomaly includes Blalock-Taussig
common atrioventricular valve
• If with RVOTobstruction, aortopulmonary shunt
or via 2 separate valves into a
manifestation similar to TOF or pulmonary arterial
single ventricular chamber
• If RVOTis not obstructed, banding if pulmonary
• Total mixing of systemic and
similar to transposition of outfiow is unrestricted
pulmonary blood
great vessels with VSD • Corrective surgery

377
 4) Hypoplastic Left Heart Syndrome
• Related anomalies that present
with underdevelopment of the
left side of the heart
• Inadequate maintenance of
systemic circulation
5) Tricuspid Atresia {TA)
• No outlet from the RA to the
RVis present (i.e., atretic or
missing tricuspid valve)
• Entire systemic venous return
leaves RAand enters the
left side of the heart via the
fora men ova le or an ASD
Park,M. PediatricCardiology
• Early cyanosis or grayish- • Prostaglandin infusion to
blue color of skin because of maintain patency of a1terial
cyanosis and poor perfusion duct before the surgery
• Signs of poor systemic • Staged surgical
perfusion and shock reconstruction
• Cyanosis at birth • For severely cyanotic
• Holosystolic murmur at the neonates, prostaglandin
left sternal border infusion is done until
• Single S2 surgical aortopulmonary
• LADand LVHon ECG shunt can be done
( combination of cyanosis & • Surgicalmanagement:
LADis highly suggestive) bidirectional Glennshunt or
• 20 echo: hypoplastic RV modified Fontan operation
Source:KliegmanRM,et al. NelsonTextbook of Pediatrics(21sted);2020
for Practitioners(6thed.).Philadelphia:
MosbyElsevier;2014
ChelliahA, et al. CurrentCardiovascular
ImagingReports:2019
JonasR. Comprehensive Surgical Management forCongenitalHeartDiseases. 2004

OBSTRUCTIVE LESIONS
I. COARCTATION OF THE AORTA (CoA)
A. Etiopathogenesis
1. Epidemiology
More common in males and most are associated with bicuspid aortic valve
Chromosomal abnormality associated with this condition: Turner's syndrome ( 45 XO)
Lesion is in the descending aorta in 98% of cases ( distal to the origin of the left
subclavian artery)
2. Effects in the Heart
Pressure build-up in the proximal aorta and LV
• Disease course depends on the degree of obstruction, presence of collateral
circulation, and associated cardiac anomalies
B. Manifestations
Asymptomatic for mild CoA; symptoms ofCHF if with more significant narrowing
° Classic sign: disparity in pulsation and blood pressure in arms & legs (BP is elevated in
vessels proximal to the coarctation; i.e., higher in the arms than in the legs)
0 Weak, delayed, or absent femoral pulses

Co· :
DIAGNOSTIC REMARKS/FINDINGS
• Rib notching: due to pressure erosion from increased blood flow through
Chest interthoracic & intercostal vessels which serve as collateral circulation
Radiograph
• Cardiac enlargement and pulmonary congestion in severe coarctation
• Normal in young children
ECG
• LVHin older patients
• Can visualize the segment of coarctation
Echocardiography • Not sufficient for the quantification & morphologic characterization
of coarctation
• Preferred non-invasive technique to evaluate the anatomy of the
Cardiac CT
entire aorta

• First line assessment in the follow-up of CoA

Cardiac MRI • Can identify the arch geometry and morphology (residual stenosis
or aneurysm formation)

378
 D. Management
• Prostaglandin El infusion for neonates with severe coarctation to
In Neonates reopen the ductus and reestablish adequate lower extremity blood flow
• Surgery is done once diagnosis is confirmed
• Surgical repair: treatment of choice
Older Children • Balloon angioplasty/stent placement in selected cases (usually
recurrent CoAand adolescent/adult)
Source:KliegmanRM,et at NelsonTextbook of Pediatrics(21sted);2020
Park,M. PediatricCardiologyfor Practitioners
(6thed.).Philadelphia:
MosbyElsevier;2014

II. PULMONICSTENOSIS
A. Etiopathogenesis
° Causes RVOTobstruction
0 Associated with congenital rubella, Noonan & William syndrome

1 E I 'd :
TYPE REMARKS/FINDINGS
Valvar PS • Isolated valvular PS is the most common
Subvalvular
• Caused by muscular or fibrous obstruction in the RVOT
(infundibular) PS
Supra valvular • Constrictions along the major branches of pulmonary arteries
or peripheral PS • High incidence in congenital rubella syndrome

2. Effects in the
Obstruction
• RV pressure
• PA pressure
Heart
of flow from RVOTto PA results in increased RV systolic pressure & RVH
may be higher than systemic arterial pressure in severe cases of PS
( distal to obstruction) is normal or decreased
I
B. Manifestations
Symptoms • Asymptomatic, unless severe
• RV heave/tap
• Ejection click and systolic thrill
Signs • Systolic ejection murmur at the LUSBwith radiation to the back
• Soft P,
• Signs of RV failure in severe PS (e.g., hepatomegaly, edema)
• Asymptomatic; progression unlikely
Natural Course • Easy fatigability & CHF if severe
• Others: chest pain, syncope, sudden death, arrhythmias, endocarditis

379
 Co· :
DIAGNOSTICS REMARKS/FINDINGS
• Normal or RV cardiomegaly (uplifting of the apex)
Chest
• Pulmonary vascularity may be normal or decreased
Radiograph
• If post-stenotic dilatation, normal or dilated MPA
• Right axis deviation
• Tall, spiked P wave
ECG
• RVH (pure Rand upright Tin Vl)
• RBBB may be seen
• Valvar PS: thickened pulmonic valve with restricted systolic motion
Echocardiogram • lnfundibular PS: hypertrophy of the RVOTclose to pulmonic valve
or well below it

Cardiac • Not generally required

Catheterization • Undertaken as part of a balloon valvuloplasty in valvar PS
• Useful to evaluate coronary anomalies and major aortopulmonary
Cardiac CT
collateral arteries
• RV outflow tract morphology
• Pulmonary artery morphology
Cardiac MR
• Valvular function with flow assessment
• Ventricular volume & function

OM :
TYPE MANAGEMENT
• Balloon valvuloplasty (catheterization) for moderate/severe
isolated PS as the initial treatment of choice
Valvar PS
• Surgical valvotomy for severely thickened pulmonic valves (e.g.,
Noonan syndrome)
Infundibular PS • Surgical resection of hypertrophied infundibular muscle in RVOT
Peripheral PS • Surgery or catheter balloon dilation
Source:KliegmanR, et al. NelsonTextbooko Pediatrics(21st ed.). Philadelphia:Elsevier:2019
Park.M. PediatricCardiologyfor Practitioners(6th ed.). Philadelphia:MosbyElsevier:2014

III. AORTIC STENOSIS (AS)

A. Etiopathogenesis
Causes left ventricular outflow tract (LVOT) obstruction
0 More frequent in males

1. Types of AS
TYPE REMARKS
• Most common form
• Leaflets are thickened and commissures are fused
Valvular AS
• Valve is usually thickened and bicuspid with fused commissmes
and eccentric orifice
Subvalvular
• Discrete fibromuscular shelf below the aortic valve
(Subaortic) AS
Supravalvular AS • Least common type

2. Effects in the Heart

Rise in LVpressure due to LVOTobstruction
LVHand high intracavitary pressure may lead to inadequate coronary artery filling
Reduced compliance of LVleads to diastolic dysfunction

380
B. Manifestations
• Usually asymptomatic until the LVfails (symptoms depend on
Symptoms severity of AS)
• Syncope and sudden death may occur with exercise
• Mild AS: pulses, heart size, and apical impulse are normal
• More severe AS: diminished pulses, enlarged heart, LVapical
Signs thrust, diminished Sl, paradoxical splitting of S2, presence of S4
from decreased LVcompliance
• Early systolic ejection click at the apex and left sternal edge
• Neonates with critical AS: severe heart failure with rapid
deterioration from a low-output shock state (emergency surgery
or balloon valvuloplasty is lifesaving)
Natural Course
• AS is one of the causes of sudden cardiac death in pediatrics
• Older children with isolated bicuspid AV:increased risk for
developing dilation of ascending aorta

C. Diagnosis
DIAGNOSTICS REMARKS/FINDINGS
Chest • Prominent ascending aorta with a normal aortic knob
Radiograph • Normal heart size
• May be normal
ECG
• LVHand strain (inverted T waves in left precordial leads)
• Defines the site and severity of AS
Echocardiogram
• Number of valve leaflets and morphology will be defined
Cardiac • Performed in conjunction with aortic balloon valvuloplasty
Catheterization • Demonstrates magnitude of pressure gradient from LVto the aorta
Source:KliegmanRM.et al. NelsonTextbookof Pediatrics(21sted);2020
Park,M. PediatricCardiologyfor Practitioners
(6thed.).Philadelphia:
MosbyElsevier;2014

D. Management
TYPE MANAGEMENT
• Balloon valvuloplasty (treatment of choice) indicated for
moderate-severe valvular AS
Valvular AS
• Surgery reserved for dysplastic aortic valves that are not
amenable to balloon therapy
Subvalvar AS
Supravalvular AS
• Surgical resection of discrete subaortic AS
• Surge1y
Source:KliegmanR, et al. NelsonTextbooko PedIatncs(21sted.).Philadelphia:
Park,M. PediatricCardiologyfor Practitioners
(6thed.).Philadelphia:
Elsevier;2019
MosbyElsevier;2014
I

381
 SECTION FOUR
ACQUIRED HEART DISEASES

RHEUMATIC FEVER (RF)

I.ETJOPATHOGENESIS
• Inflammatory lesion found mainly in the
• Valvular damage: involves (in descending
and pulmonary valve
• Aschoff bodies in the atrial myocardium:
fragmentation of collagen fibers, altered
heart, brain, joints & skin
order of frequency) the mitral, aortic, tricuspid,
inflammatory lesions with swelling,
staining characteristics of connective tissue

II. MANIFESTATIONS
History of streptococcal pharyngitis 1-4 weeks before the onset of symptoms
• Other non-specific manifestations: pal lot; malaise, easy fatigability, abdominal pain

A. Major Manifestations
MANIFESTATIONS REMARKS
• Low-risk*: migratory polyarthritis
• Moderate/high risk: monoarthritis, polyarthritis, or
polyarthralgia
Arthritis
• Most often asymmetric and affecting large joints (ankles, wrists,
(70%)
knees, elbows)
• Highly responsive to salicylates and NSAIDs
• Generally runs a self-limited course lasting approximately 4 weeks
• Clinical OR subclinical carditis
• May be pancarditis involving the pericardium, myocardium, and
endocardium
0 Pericarditis: audible friction rub, pericardia! effusion on
echocardiography
Carditis 0 Myocarditis: unexplained heart failure (gallop, soft heart
(50%) sounds), or cardiomegaly
0 Endocarditis/valvulitis: apical holosystolic murmur of mitral
regurgitation or basal early diastolic murmur
• Hallmark is valvular damage (only valvulitisleads to pe1111anentdamage)
• Characteristic manifestation is mitral regurgitation
• Single most important prognostic factor in RF
• Evanescent, pink rash seen with pale centers and rounded
serpiginous margins
Erythema • Most prominent on the trunk and inner proximal portions of the
marginatum
extremities
(<10%)
• Does not occur in the face
• Disappears on exposure to cold and reappear after a hot shower

• Round, hard, painless, nonpruritic, freely movable swellings

Subcutaneous • Found symmetrically, singly or in clusters on the extensor surfaces
nodules
(2-10%) of large & small joints, over the scalp or along the spine
• May last for weeks
• Purposeless, involuntary, non-stereotypical movements of the
Sydenham chorea
trunk or extremities
(15%)
• Often with muscle weakness and emotional !ability
'Low riskpopulation:ARFincidence,<;2per 100,000school-agedchildrenor $1 per 1,000population

Source:GewitzMH,el al.AHARevisionof theJonescriteria;2015

382
 B.Minor Manifestations
ASPECT MANIFESTATIONS
I

• Arthralgia (joint pains): not considered a minor manifestation if

arthritis is present
0 Low risk: polyarthralgia
Clinical 0 Moderate/high risk: monoarthralgia
• Fever
0 Low risk: 2e38.S°C
0 Moderate/high risk: 2e38°C
• Elevated acute phase reactants
Laboratory 0 Low risk: ESR 2'60 mm/hand/or CRP 2'3.0 mg/dL
findings 0 Moderate/high risk: ESR 2'30 mm/hand/or CRP 2'3.0 mg/dL
• Prolonged PR interval on ECG
Gewitz
Source: oftheJonescriteria;
MH,etal.AHARevision 2015
MH,etal.Revision
Gewitz Circulation:
oftheJonesCriteria. 2015

C. Evidence of Antecedent Group-A Streptococcal Infection within the Last 45 Days:

, A history of sore throat/scarlet fever unsubstantiated by laboratory data is inadequate
evidence of recent infection
Any 1 of the following can serve as evidence of preceding infection:'

• Elevated or rising anti-streptolysin-Ob or other streptococcal antibody

• Positive throat culture
• Rapid antigen test for group-A streptococcus
• Recent scarlet fever
' Streptococcalantibodytests are the mostreliablelaboratoryevidence.Theonsetof the clinicalmanifestations
coincidewiththe peakof the streptococcalantibodyresponse
O (ASO)titeris betterevidencethana singletiterresult
'A rise inAntistreptolysin
Gewitz
Source: oftheJonescriteria;
MH,el al.AHARevision 2015

III. DIAGNOSIS

The Revised Jones Criteria for the Diagnosis of RF and Rheumatic Heart Disease (RHO)
DIAGNOSIS CRITERIA
• Evidence of preceding group-A streptococcal infection; PLUS:
Initial Rheumatic 0 2 major criteria, or
Fever
0 1 major+ 2 minor criteria

Recurrent Rheumatic
Fever
• With a reliable past history of ARF or established RHO,and in
the face of documented GASinfection:
0 2 major criteria, or;
• 1 major+ 2 minor, or;
I
• 3 minor manifestations

ofRFcanbe madewithout
whereina diagnosis
Threecircumstances strictadherencetoJonescriteria:
• Whenchoreaistheonlymajormanifestation
carditis
• Whenindolent uponfirstcheckup,monthsaftertheapparentonsetofARF
istheonlymanifestation
• ApparentARF numberofpatientswithrecurrences
ina limited populations
high-risk
ofARFinparticularty

Gewitz
Source: MH,el al.AHARevisionoftheJonescriteria;
2015
Bisno Rheumatic
Consultation:
A,etal.WHOExpert heartdisease;
feverandrheumatic 2004

383
IV. MANAGEMENT
A.Antibiotic Therapy
Once the diagnosis of acute RF has been made, give 10 days of oral Penicillin or
Erythromycin or a single IM injection ofbenzathine Penicillin to eradicate GASfrom
the upper respiratory tract
• After this initial course of antibiotics, patient should be started on long-term antibiotic
prophylaxis

Indications for Antibiotic Therapy

INDICATION RATIONALE ANTIBIOTICS
• Penicillin VK 200-500 mg QID
x 10 days
Primary • To treat streptococcal pharyngitis
• Benzathine Penicillin G 0.6 - 1.2
prevention and eradicate Streptococcus
(prophylaxis) million units IM as single dose
• To prevent first episode of RF
• Erythromycin 250 mg TIO
x 10 days
• To prevent recurrences of RF
Secondary • Penicillin VK 250 mg BID
• To prevent colonization and/or
prevention • Benzathine Penicillin 0.6-1.2 M
(prophylaxis) infection in patients who have
units every 21 days IM
had a previous attack of RF
Source:GewitzMH,et al.AHARevision
of theJonescriteria;2015

Duration of Prophylaxis for Patients who have had Acute RF

CATEGORY
• RFwithout carditis
• RF with carditis, but no residual valvular
disease (no clinical or echocardiographic
evidence of valvular disease)
• RF with carditis and persistent residual
valvular disease
DURATION OF PROPHYLAXIS
• 5 years after last attack or until 21 years
old (whichever is longer)
• 10 years after last attack or until 21
years old (whichever is longer)
• 10 years after last attack or until 40 years old
(whichever is longer, sometimes lifetime)

Source:AHAScientific
Slalemenl.
AcuteStreptococcal 2009
Pharyngitis:
GewitzMH,el al.AHARevision
of theJonescriteria;2015

B.Anti-lnflammatory and Other Agents for Manifestations of RF

ASPECT MANAGEMENT
Arthritis/ • Aspirin 100 mg/kg/day in 4-6 divided doses up to 2 weeks
Mild Carditis • Dose can be decreased to 60-70 mg/kg/day for an additional 3-6 weeks
Moderate-Severe • May add prednisone 1-2 mg/kg/day up to maximum of 3 weeks
Carditis (then dose decreased and tapered by 20-25% each week)
Severe Chorea • Carbamazepine or valproic acid
• Complete bed rest and oxygen
• Morphine 0.2 mg/kg at 4-hour interval for severe CHF with
respiratory distress
Heart Failure
• Restriction of sodium and fluid intake
• Furosemide 1 mg/kg/dose every 6-12 hours
• Others: digoxin, inotropes
• Hospital admission to confirm diagnosis and institute treatment
General Measures
• Bed rest and monitoring for onset of carditis
Source:AHASc1enl1ficStatement
Prevention Fever;2009
of Rheumatic
BisnoA, el al.WHOExpertConsullalion:
RFandRHO;2004
384
VALVULAR HEART DISEASE (VHD)
May be congenital or acquired (almost all acquired VHDs are rheumatic in origin)
• Mitra! valve disease make up majority cases (next is the aortic valve)
• Tricuspid valve involvement is rare, while pulmona1y valve involvement almost never occurs
• Aortic and pulmonic stenosis are discussed above (under Congenital Heart Diseases)

PATHOPHYSIOLOGY MANIFESTATIONS MANAGEMENT

1) Mitra/ Stenosis (MS)

• Most common valvular
involvement in adults
• Thickening of leaflets and
fusion of the commissures
results in calcification with
immobility of the valve
• LA and right-sided heart
chambers become dilated
and hypertrophied
2) Mitra/ Regurgitation (MR)
• Asymptomatic if mild
• Dyspnea, 01thopnea, nocturnal
dyspnea in severe cases
• Increased RV impulse along • Dental hygiene & antibiotic
left parasternal border prophylaxis
• Opening snap followed by • Closed mitral
a low-frequency diastolic commissurotomy for those
rumble at the apex without calcification
• CXR:enlarged LA& RV, • Valve replacement if valves
prominent main pulmonary are calcified
a1te1y (MPA)and lung fields
show pulmona1yvenous
congestion (with Kerley 8 lines)

• Most common valvular
involvement in RHO
• Shortened leaflets due to
fibrosis
• Dilated LA & LVwith dilated
MV ring
3) Mitra/ Valve Prolapse (MVP)
• Asymptomatic in childhood
• Hyperdynamic apical impulse
is palpable in severe MR
• S1 is normal or diminished
• Systolic regurgitant
murmur at the apex with
transmission to the left axilla
• Short, low-frequency
diastolic rumble at the apex
• Dental hygiene & antibiotic
prophylaxis
• Afterload-reducing agents
to maintain forward cardiac
output
• Diuretics & digoxin for CHF
• MV repair or replacement

• Identified by auscultation
in asymptomatic patients or
incidentally through 2D echo
• Major complications include: • Most common
• Asymptomatic patients
endocarditis, severe MR, complaint: palpitation
do not need treatment or
cerebrovascular ischemic from supraventricular
restriction of activity
events arrhythmias
• Systolic click: due to sudden

4) Aortic Regurgitation (AR)

tensing of the MV as leaflets
prolapse into LA in systole
I
• Asymptomatic if mild
• Reduced exercise tolerance
in severe AR or if with CHF • Dental hygiene & antibiotic
• Hyperdynamic precordium prophylaxis
• Semilunar cusps are
• Wide pulse pressure and a • ACE inhibitor to reduce the
deformed & shortened
bounding water-hammer dilatation of LV
• Dilated valve ring so that the
pulse in severe AR • Digoxin, diuretics, afterload-
cusps fail to appose tightly
• High-pitched diastolic reducing agents
• Most have associated mitral
murmur heard best at the • AV replacement before
valve disease
3rd-4th left JCS (hallmark) irreversible dilatation of LV
• CXR:LVE,dilated ascending develops
aorta & prominent aortic
knob

Source:KliegmanR.et al. NelsonTextbookof Pediatrics

(21sted.).Philadelphia:
Elsevier;2019
Park,M. PediatricCardiologyforPractitioners
(61hed.).Philadelphia:
MosbyElsevier;2014
385
INFECTIVE ENDOCARDITIS (IE}
I.ETIOPATHOGENESIS
• Infection of the endocardial surface of the heart, which usually includes the heart valves
• Prototypic lesion: vegetation

A. Etiology
0 Viridans-type streptococci (alpha-hemolytic strep) and Staphylococcus aureus
0 Other common causes: group D strep, Enterococws, S. bovis, S. faeca/is, etc.
0 ~6% of cases: blood cultures are negative

B. Pathophysiology and Risks

0 Vegetations form at the site of the endocardial or intimal erosion that results from the
turbulent flow
0 At high risk: VSD,AS,TOF, PDA,MVP,children who underwent valve replacement
0 Identification of IE is most often based on a high index of suspicion during evaluation
of an infection in a child with an underlying contributory factor

II. MANIFESTATIONS
A. Acute versus Subacute Endocarditis

ACUTE BACTERIAL IE SUBACUTE BACTERIAL IE

• S. aureus (sometimes)
• S. aureus • Streptococcus viridans
Usual • B-hemolytic streptococci • Enterococci
Etiology • Pneumococci • HACEK*
• Aerobic gram-negative bacilli • Coagulase-negative staphylococcus
(prosthetic valves)
• High-grade fever (39.4-4O°C) • Low-grade fever (rarely >39.4°C)
Clinical • Acute/decompensated heart • Weight loss, abdominal symptoms,
failure is common pleurisy
• Rapid
• Indolent, subtle, and non-specific
• Hematogenously seeds to
Course symptom
extracardiac sites
• Rarely metastasizes
• Complications are common
Prognosis • Poor (if untreated) • Better prognosis
• HACEK:Haemophifus
sp.. Aclinobaciffus
actinomycetemcomitans,
Cardiobacterium
hominis,Eikeneffa
corrodens,
Kingelfasp.

B. Manifestations of IE
MANIFESTATION DESCRIPTION
Cardiac • Murmur is common, which may be indicative of valvular damage in IE
manifestations • Others: gallop, arrhythmias, pericardia! rub
Janeway lesions • Non-tender, slightly raised hemorrhages on the palms and soles
Osier's nodes • Tender; raised nodules on the pads of the fingers or toes
Splinter • Painless dark red linear lesions in the proximal nail bed that
hemorrhages may coalesce
Roth spots • Retinal hemorrhages with small, clear centers

386
Ill. DIAGNOSIS
A Major and Minor Criteria
MAJOR CRITERIA
1) Positive blood culture
• Typical organisms for IE from ;;;:2cultures:
• OR, Persistently positive cultures,
defined as:
0 At least 2 blood cultures drawn >12
hours apart, or
0 All of 3 or a majority of ;;,4 separate
cultures with first and last drawn at
least 1 hour apart
• OR, Single positive blood culture for
Coxiella burnetii or phase 1 lgG antibody
titer of ;el:800
Note: If cultures remain negative after
48-72 hours, 2-3 additional blood culture
sets should be obtained. Antimicrobial
pretreatment reduces the yield of blood
culture to 50-60%
2) Evidence of endocardial involvement:
• Positive echocardiogram for IE*:
0Oscillating intracardiac mass on valves
or supporting structures
0Abscess, or
0New dehiscence of prosthetic valve
0New valvular regurgitation**
MINOR CRITERIA
• Predisposing heart condition (valvular
disease with stenos is or regurgitation,
prosthetic valves, congenital heart
defects, prior endocarditis, hypertrophic
cardiomyopathy) or injection drug use
• Fever ;,3B.0°C
• Vascular phenomena: major arterial
emboli, septic pulmona1y infarcts,
mycotic aneurysms, intracranial
hemorrhage, conjunctiva! hemorrhages,
Janeway lesions
• Immunologic phenomena:
glomerulonephritis, Osier's nodes, Roth's
spots, rheumatoid factor
• Microbiologic evidence: positive blood
culture but not meeting major criterion or
serologic evidence of active infection with
organism consistent with IE

Note: Absence of vegetations does not

exclude endocarditis
'Transesophageal echocardiography (TEE)or transthoracic echo(TTE).TEEis recommended for prosthetic
valves,ratedat leastpossibleIE by clinicalcriteria,or complicated
IE (e.g.paravalvular
abscess)
"Worseningor changingor pre-existing murmuris notsufficient
Source:Baltimore
RS,et al. Infectiveendocarditis
in childhood.
AHACirculation:
2015

I
B.Duke Clinical Criteria for Diagnosis
DIAGNOSIS CLINICAL DEFINITION
• Two major criteria; OR
Definite
• One major criterion and three minor criteria; OR
Endocarditis
• Five minor criteria
Possible • 1 major and 1 minor criterion, OR
Endocarditis • 3 minor criteria

• Alternative diagnosis is established OR

• Symptoms resolve & do not recur withs 4 days of antibiotic
Diagnosis oflE is therapy OR
Rejected • Surgery or autopsy after s4 days of antimicrobial therapy yields no
histologic evidence of endocarditis OR
• Does not meet criteria for possible IE, as above
Source:JS Li et al:ClinInfectDis2000
Baltimore
RS,el al. Infectiveendocarditis
in childhood.
AHACirculation; 2015

387
IV. MANAGEMENT
• Treatment is generally 4-6 weeks (referral to infectious disease specialist is recommended)
• Longer theapy may be required for recurrent endocarditis, prosthetic valve endocarditis,
and uncommon organisms
• Empirical therapy for patients without a prosthetic valve and at high risk of S. aureus,
enterococcus, and viridans-type streptococci: Vancomycin plus Gentamicin
ORGANISM REGIMEN
Unknown Agent
• Ampicillin/sulbactam plus gentamicin
Native valve or "late" prosthetic valve
• With or without vancomycin
infection (>l year after surgery)
• Add rifampin for prosthetic valve endocarditis
Nosocomial endocarditis associated • Vancomycin PLUS gentamicin
with vascular cannulae or "early"
• With or without rifampin if with prosthetic material
prostethic valve endocarditis
('.SIyear after surgery) • PLUS cefepime or ceftazidime

Streptococcus
Highly susceptible to penicillin G • Penicillin G or ceftriaxone
• Penicillin G (or ampicillin) PLUS gentamicin
Relatively resistant to penicillin
(for first 2 weeks, or whole course for enterococci)
Staphylococcus (S.aureus or Coagulase-Negative Staphylococci)
Susceptible to penicillin G (rare) • Penicillin G
Resistant to 0.1 ug/mL of penicillin G • Oxacillin OR nafcillin ± gentamicin x 3-5 days

MRSA • Vancomycin
Vancomycin resistant or intolerant • Daptomycin
If prosthetic material is present • PLUS rifampin PLUS gentamicin (for first 2 weeks)
Other Specific Therapy
• Ceftazidime, cefepime, cefotaxime, or ceftriaxone
Gram-negative enteric bacilli plus gentamicin (or tobramycin or amikacin,
depending on susceptibility)
• Ceftriaxone OR Cefotaxime OR
HACEK
• Ampicillin-sulbactam
Fungi: Candidaspp, Aspergillusspp • Amphotericin B or Flucytosine

V. ANTIBIOTIC PROPHYLAXIS FOR IE

• Considered for those patients at highest risk for IE
, With prosthetic valves [including transcatheter valve)
'With prosthetic material for cardiac valve repair
' With previous episode of IE
• The following subpopulation with congenital heart disease (CHD) are
Prophylaxis is
also high risk for IE, and must be given prophylaxis
given to whom?
0 Unrepaired cyanotic CHD (includes palliative shunts or conduits)
, Repaired CHD with a prosthetic material, especially during the 6
months after repair
'Incompletely repaired CHD with residual defects adjacent to
prosthetic material
Prophylaxis is
• Dental procedures requiring manipulation of the gingival or periapical
given for which
procedures? region of the teeth or perforation of the oral mucosa

Source:BaddourLM.et al.AHAGuidelineson IE:Circulation:

2015
Taskforce
for the Managementof IE of the ESC2015:EuropeanHeartJournal2015

388
KAWASAKI DISEASE (KD)
• Acute non-specific systemic vasculitis (medium-sized arteries)
• Vessels lose its structural integrity and weakens, resulting in dilatation and saccular or
fusiform aneurysm formation

I. ETIOPATHOGENESIS
A. Epidemiology
Most affected children are Asians although it occurs worldwide
0 More common in boys (1.5:1)
0 80% of patients are <5 years old
0 Unknown cause

B. Pathology
0 Arteritis is frequently observed in the coronary arteries and iliac arteries
0 Interstitial myocarditis, pericarditis, inflammation of the SA node and atrioventricular
conduction system, endocarditis and valvulitis may also be present

II. MANIFESTATIONS
PHASE DESCRIPTION
Acute febrile • Last 1-2 weeks
phase • Fever and other acute signs of illness
• Begins when fever & other acute signs have abated
Subacute • Associated with desquamation, thrombocytosis, coronary aneurysms
phase (highest risk of sudden death in those who have developed aneurysms)
• Lasts until the 4th week
Convalescent • Begins when all clinical signs have disappeared
phase • Continues until ESR & CRP return to normal ~6-8 weeks after onset

III. DIAGNOSIS
A. Diagnostic Criteria

Fever (spiking up to 40°C and persisting for 5 days or more, remittent, unresponsive
to antibiotics), plus presence of at least 4 features:

• Bilateral bulbar conjunctiva! injection (without exudates)

• Erythema of the oral & pharyngeal mucosa with strawberry tongue and dry, cracked
lips without ulceration
• Nonsuppurative cervical lymphadenopathy (>1.5 cm in diameter), usually unilateral
• Edema & erythema of the hands & feet
• Polymorphous exanthema: maculopapular, diffuse erythroderma, or erythema
multiforme-like (nonvesicular and non-petechial, with accentuation in groin area)

Fever plus less than four out of five remaining criteria or the presence of coronary lesions
(proven by echocardiogram or angiography) may still be diagnostic. In the presence of four
or more principal clinical features, especially when redness & swelling of the hands and feet
are present, the diagnosis can be made with 4 days of fever.
I
Incomplete or Atypical Kawasaki Disease is considered in any infant or child with prolonged
unexplained fever with fewer than 4 of the principal clinical features AND compatible
laboratory or echocardiographic findings

B. Diagnostics
DIAGNOSTIC REMARKS/FINDINGS
• Anemia and leukocytosis may be present
• Platelet count usually normal in the 1" week of illness but
Laboratory Tests
rapidly increases in the 2"'-3'' week
• Elevated ESR, CRP for 4-6 weeks
• Should be performed when diagnosis of KD is considered
Echocardiography
• Should be repeated within 1-2 weeks & 4-6 weeks after treatment
389
IV. MANAGEMENT
PHASE MANAGEMENT
• !VIG2 g/kg as single IV infusion within 10 days of illness onset but as
soon as possible after diagnosis
• May give !VIGto children after the 10th day of illness if they have either:
0Persistent fever without other explanation. or
Acute phase
° Coronary artery abnormalities with elevated ESR or CRP
• Aspirin as moderate- (30-50 mg/kg/day) to high-dose (80-100 mg/kg/
day) every 6 hours is reasonable until patient is afebrile (but there is no
evidence that it reduces coronary artery aneurysms)
• When high-dose ASA is discontinued, low-dose ASA (3-5 mg/kg/day)
Convalescent
is begun and continued until the patient has no evidence of coronary
phase
changes by 6-8 weeks after onset of illness
Long-term • For children who develop coronary abnormalities, ASA may be
therapy for
continued indefinitely
coronary
abnormalities • Aspirin 3-5 mg/kg once daily +/-clopidogrel 1 mg/kg/day (max 75 mg/day)

REFERENCES
I. Baddour LM, Wilson WR, Bayer AS, Fowler VG Jr, Tleyjeh IM, Rybak MJ, et al. AHA Scientific Statement. Infective
Endocarditis in Adults: Oi;1gnosis, Antimicrobial Therapy. and Management of Complications: A Scientific Statement
for Healthcare Professionc1ls from the American Heart Association. Circulation. 2015;132(15): 1435·86.
2. Baltimore RS,Gewitz M,Baddour LM,Beerman LB.JacksonMA Lockhar1PB, Pahl E,Schutze GE,Shulman Sl'.Willoughby RJr:
on behalf of the American Hea1tAssociation Rheumatic Feve1;Enclocarditis,and Ka1,,vasaki
Disease Committee of the Council
on Cardiovascular Disease in the Young and the Council on Cardiovascular and Stroke Nursing. Infective endocarditis in
childhood: 2015 update: a scientific statement from the American Heait Association. Circulation.2015; 132: 1487-1515.
3. Bisno A. Butchart EG, Ganguly NK, Ghebrehiwer T, Lue HC, Kaplan EL, et al. World Health Organization Expert
Consultation: Rheumatic fever and rheumatic heart disease. WHO Library Cataloguing-in-Publication Data; 2004.
4. Chelliah A, et al. Cardiovascular CT in Cyanotic Congenital Heart Disease. Current Cardiovascular Imaging Repmts. July 2019.
5. Crain EF.Gershel]Cand Cunningham SJ(eds). ClinicalManual of Emergency Pediatrics 5th ed. 2011.Camb,idge UniversityPress
6. Fernandes CJ.Weisman LE, Kim MS.Physiologic transition from intrauterine to extrauterine life UpToDate: 2019
7. FogelM(eel).Principlesand Praa::iceof
Ca,tliacMagneticResonanceinCongenitalHea1tDise.:1.Se
Fonn,Function.andFlow2010,Wiley-Black\vell
8. Frank JE,Jacobe KM.Evaluation and management of heart murmurs in children. Am Fam Physician 84(7):793-800; 20 I 1
9. Gerber M. Baltimore RS, Eaton CB,Gewitz M, Rowley AH, Shulman ST, et al. Ame1ican Hea,t Association scientific stalemem:
Preventionof rheumatic feverand diagnosisand treatment of acute streptococcalphmyngitis.Circulation.2009; 119( 11):1541-155I.
CA,Shulmans-r,
I 0. GewitzMH,BaltimoreRS,Tani LY.Sable Carapetisj, RemenyiB,Taube1tKA,BolgerAF,Beennan L,MayosiBM,Beaton
A.Pandian NG,Kaplan EL;on behalfof the Ame1icanHea1tAssociationCommitteeon Rheumatic Fever,Endocarditis,and Km-vasaki
Diseaseofthe Councilon CardiovascularDiseasein the Young.Revisionof the Jonesaitetia for the diagnosisofacute rheumatic feverin
the era of Dopplerechocardiography:a scientificst,llement from the Ame1icanHea,t Association.Circulation.2015;131:1806-·1818
11. Gewitz MH,Baltimore RS,Tani LYet al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the eraof
doppler echocardiography: a scientific statement from the american hea1t association. Circulation 2015;131:1806-18.
12. Habib G. Lanccllotti, P,J\ntuncs, M et al.2015 ESCGuidelines for the management of infective endocarditis: The Task
Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC) Endorsed by:
European Association for Cardio•Thoracic Surgery (EJ\CTS), the European Association of Nuclear Medicine (EANM).
European Heart Journal, Volume 36, Issue 44, 21 November 20 I 5. Pages 3075-3128
13. jonas R. Comprehensive Surgical Management for Congenital Heart Diseases.2nd ed. 2004. CRCPress
14. Kliegman RM,Stanton BF,St Geme JW,Schor NF.Nelson Textbook of Pediatrics 21st ed. 2020. Philadelphia, PA:Elsevie1:
l 5. Li JS, Sexton DJ, Mick N, Nettles R, Fowler VG J1~Ryan T, et al. Proposed modifications to the Duke criteria for the
diagnosis of infective endocarditis. Clin Infect Dis. 2000;30( 4):633-638
I 6. McCrindle, 8.W. et al. Diagnosis, Treatment. and Long-Term Management of
Kawasaki Disease. A Scientific Statement for Health Professionals from the American Heart Association. 2017.
17. Ntsinjana H, Hughes, M, and Taylor A. The Role of Cardiovascular Magnetic Resonance.
Cardiovasc Magn Reson.2011; 13{1): 51.
18. Park, M. Pediatric Cardiology for Practitioners 6th ed. 2014. Philadelphia

390
NEPHROLOGY
 SECTION ONE
APPROACH TO HEMATURIA

HEMATURIA
I. DEFINITION
• General definition of hematuria is the presence of at least 5 RBC per high-power field in
urine microscopy
• 10-50 RBCs/uL in microscopy may suggest underlying pathology

II. OVERVIEW OF DISEASES COMMONLYPRESENTING AS HEMATURIA

!GA GOODPASTURE IDIOPATHIC
PARAMETERS PSGN
NEPHROPATHY SYNDROME RPGN
• All ages with
• 10-35 years • 15-30 years
Age mean of 7 • Adults
old old
years old
Sex
predilection • 2:1 • 2:1 • 6:1 • 2:1
{M:F)
Acute nephritic
• 90% • 50% • 90% • 90%
syndrome
Asymptomatic
• Occasionally • 50% • Rare • Rare
hematuria
Nephrotic
• 5-10% • Rare • Rare • 10-20%
syndrome
Hypertension • 70% • 30-50% • Rare • 25%
Acute renal • 50%
• Very rare • 50% • 60%
failure [transient)
• Pulmonary
• Follows throat
• Follows viral hemorrhage
Others infection or • None
syndrome • Iron deficiency
pyoderma
anemia
• HLA-B12; D • HLA-Bw35, • None
lmmunogenetics • HLA-DR2
"EN" DR4 established
• Focal to diffuse
Light • Diffuse • Focal
proliferation • Crescentic GN
microscopy proliferation proliferation
with crescents
lmmuno
fluorescence
Electron
microscopy
• Granular lgG,
C3
• Subepithelial
humps
• Diffuse
mesangial lgA
• Mesangial
deposits
• Linear lgG, C3

. No deposits
• No immune
deposits

• No deposits
I
• 95% resolve • Slow • 75% stabilize . 75% stabilize
Prognosis spontaneously progression in or improve if or improve if
• 5% RPGN 25-50% treated early treated early
PSGN:Poststreptococcal
glomerulonephritis
RPGN:
Rapidly progressive
glomerulonephritis
Source:Kliegman
R,et al.NelsonTextbook
ofPediatrics
(21sted).Elsevier:
2020
PhadkeK,et al.Manual
ofPediatric
Nephrology.Springer:
2014

393
III. CAUSES OF RED URINE
ORIGIN CHARACTERISTICS

Upper urinary tract: • Glomerular: brown/cola/tea-colored, RBCcasts, deformed

within the nephron
(giomeruius, tubular
system, interstitium)
Lower urinary tract:
pelvocalyceal system,
ureter, bladder, or
urethra
RBC (acanthocytes)
• Proteinuria >100 mg/dL via dipstick
• Tubular system: with or without leukocytes or renal tubular cast
• Gross hematuria, bright red/pink, terminal hematuria (at
end of urine stream), blood clots, normal RBC morphology
• Minimal proteinuria <100 mg/dL via dipstick

Heme • Hemoglobinuria, myoglobinuria

positive
• Drugs: chloroquine, deferoxamine, ibuprofen, iron
Other Heme sorbitol, metronidazole, nitrofurantoin, phenazopyridine,
causes negative phenolphthalein, phenothiazines, rifampicin, salicylates,
(negative sulfasalazine
dipstick • Dyes: beets, blackberries, food coloring, rhubarb
for blood) • Metabolites: homogentisic acid, melanin, methemoglobin,
porphyrin, tyrosinosis, urates
Source:KliegrnanR, et al. NelsonTextbookof Pediatrics(21sted}.Elsevier;2019

GLOMERULONEPHRITIS
I. MANIFESTATIONS OF GLOMERULONEPHRITIS
• Tea- or cola-colored urine
• Facial or body edema
• Hypertension
• Oliguria

II. COMMONCAUSES OF GLOMERULONEPHRITIS

MANIFESTATIONS DIAGNOSTICS RENAL BIOPSY MANAGEMENT
1) Post Infectious Glomerulonephritis
• Acute onset • Urinalysis shows • Enlarged, • PenicillinGx 10 days
ofhematuria, hematuria bloodless • Fluid limitation
periorbital edema, • C3 is decreased glomeruli, diffuse • Sodium restriction
hypertension, • Documentation mesangial cell • Diuresis
renal insufficiency of streptococcal proliferation, (Furosemide)
• With a history of infection (e.g., ASO increased • Antihypertensives
infection (usually titers for throat mesangial matrix
streptococcal infections, anti- • lmmunoflourescence
pyoderma or deoxyribonuclease (IF): "lumpy-
pharyngitis) B for pyoderma) bumpy" deposits
of lg and
complement on
the GBMand in the
mesangium
• Electron
microscopy (EM):
electron dense
deposits "humps",
observed on the
epithelial side of
the GBM

394
MANIFESTATIONS DIAGNOSTICS RENAL BIOPSY MANAGEMENT
2) IgA Nephropathy or Berger Disease

• Gross or • Urinalysis shows • Focal and • BP control

microscopic hematuria segmental • Management of
hematuria and/ • Proteinuria <l,000 mesangial proteinuria
or proteinuria, mg/24h proliferation • Medications:
may present • C3 is normal and increased unclear but may
with nephritic mesangial matrix include ACEi,
or nephrotic in the glomerulus ARBs, fish oil,
syndrome • lgA deposits in the corticosteroids
• Onset within 1-2 mesangium with
days of viral URTI C3 complement
3) Thin Basement Membrane Disease/ Benign Familial Hematuria

• Intermittent • Urinalysis: • EM: isolated • Monitoring

microscopic persistent thinning of the
hematuria hematuria glomerular
• Episodic gross basement
hematuria after membrane
respiratmy infection
4) A/port Syndrome (Hereditary Nephritis)

• Asymptomatic • Urinalysis shows • Glomeruli • No specific

microscopic hematuria mesangial treatment
hematuria • Proteinuria proliferation & • ACEi or ARBs
• Gross hematuria >lg/24h capillary wall may slow down
thickening,
1-2 days after • Audiogram progression
progressive
URTI • Ophthalmologic glomerular • End-stage renal
• Bilateral examination sclerosis; tubular disease in most
sensorineural • Genetic atrophy, interstitial patients
hearing loss examination inflammation and • Renal transplant
• Ocular • Anterior fibrosis, foam cells
abnormalities: lenticonus is (lipid containing
anterior pathognomonic tubular or
interstitial cells)
lenticonus,
• EM: diffuse
macular flecks, thickening &
corneal erosions thinning (basket
• With family weaving), splitting
history & layering of
• End-stage renal glomerular &
disease tubular basement
membrane
SJRapidly Progressive (Crescentic) Glomerulonephritis (RPGN)

• Acute nephritis
with proteinuria
or nephrotic
• Identify cause of
RPGN
• Crescents in 50%
or more in the
glomeruli; no
• High-dose
corticosteroid and
cyclophosphamide
I
syndrome immune deposits • Poor prognosis if
• Rapid loss of renal • IF and pattern with a majority of
function of deposits to crescents on renal
• Renal failure/ identify underlying biopsy
insufficiency cause • End-stage renal
• May be primary or • Remember, disease in most
secondary to other "crescent" is an patients
conditions ( e.g., ominous sign
lgA nephropathy,
HSP nephritis,
PSGN, SLE)

395
MANIFESTATIONS DIAGNOSTICS RENAL BIOPSY MANAGEMENT
6) Henoch-Schonlein Purpura (HSP) or IgA vasculitis
• Idiopathic • 50% have renal • Deposition of • Urinalysis done
systemic immune manifestations: polymeric lgA in weekly during
complex-mediated asymptomatic the glomeruli the period of
vasculitis with lgA microscopic • Glomerular active disease
deposits in small hematuria to findings and then once a
blood vessel walls severe progressive indistinguishable month for up to 6
• Preceded by URTI glomerulonephritis from lgA months (if all are
• Any mucosa! • Skin biopsy: Nephropathy normal, nephritis
infection or leukocytoclastic is unlikely to
food antigen vascultis with develop)
may trigger lgA C3, and fibrin • Mild disease:
the increased deposition resolves
production of spontaneously
pathogenic lgA 1 • Moderate to severe
• Tetrad of HSP: disease: steroids
0 Palpable (e.g., prednisone
purpuric 1 mg/kg/day for
rashes on the 3 months with
dependent parts ACE inhibitors)
of the body followed by
0 Arthritis or azathioprine or
arthralgia mycophenolate if
0 Abdominal pain severity persists
0 Renal disease • Consult a specialist
if proteinuria,
renal insufficiency,
or hypertension
develops
• 2-5% risk of
developing CKD
7) Hemolytic-Uremic Syndrome (HUS)
• Triad of HUS: • PBS results: • Renal biopsy • Supportive therapy
0 Microangiopathic microangiopathic rarely performed & hydration
hemolytic anemia hemolytic anemia • Glomerular • PRBC transfusion
0TimJmbocytopenia • CBC results: thickening of • Correction
0 Renal anemia, capillary walls of electrolyte
insufficiency thrombocytopenia • Narrowing of abnormalities
• History of bloody (20,000-100,000) capillary lumen • Control of
gastroenteritis • Coombs test • Platelet-fibrin hypertension
within the usually negative thrombi in • Dialysis
preceding 3 weeks • Renal: elevation glomerular • Platelet
• Most common ofBUN and capillaries transfusion
form is due to creatinine, acute • Thrombi in generally not
Shiga toxin- renal failure afferent arterioles administered
producing and small arteries • Antibiotics may
Escherichia coli with fibrinoid precipitate toxin
0157:H7 (STEC) necrosis release
'Indications
forRenalBiopsy
inG!omerulonephritis:
Acuterenalfailure,Nephrolic
syndrome,Absence
ofevidence
ofstreptococcal
infection,
Normalcomplementlevel,Hematuria
andproteinuria,
Diminished renalfunction,
LowC3 >2 months

Source:KliegmanR, et al. NelsonTextbook of Pediatrics

(21sted).Elsevier:2020
PhadkeK, et al. Manualof PediatricNephrology. Springer:2014
WeeningJ, et al. Journalof TheAmericanSocietyof Nephrology: 2004
Kumar,Vet al. RobbinsPathology10thedition:2017

396
EVALUATING A PATIENT WITH GROSS HEMATURIA

Glomerular Hematuria No
(:!: hypertension, oliguria and edema) Non-glomerula r Hematuria
(work up as indicated)

Urinary Tract Infection

Meatal Stenosis
With Other Systemic Perinea I Irritation
Manifestations Trauma
Urolithiasis
Coagulopathy
Decreased C3 Hypercalciuria
Tumor
SickleCell
Food (e.g., beet)

Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted).Elsevier;2020

PhadkeK, et al. Manualof PediatricNephrology. Springer;2014

397
 SECTION TWO
APPROACH TO PROTEINURIA

PROTEINURIA
I. DIFFERENTIALS FOR PROTEINURIA
REMARKS POSSIBLE ETIOLOGIES
• Negative evaluation on repeated • Fever >38.3 °c
Transient dipstick • Exercise/stress, dehydration
proteinuria • Usually not >l-2+ on dipstick • Cold exposure
• Normal quantified urinary protein • Heart failure, seizures
• Upright position increases proteinuria
10-fold up to 1,000 mg/24h
• Normal or minimal proteinuria in
the supine position
Orthostatic • No hematuria, hypertension, edema, • It is the most common cause of
or Postural hypoalbuminemia, & renal dysfunction persistent proteinuria in school
proteinuria • Confirmation: absence of proteinuria age children
in 3 consecutive first morning void
0 Bladder is emptied before sleeping
0 Dipstick is negative or trace
0 UPCR <0.2
• Glomerular proteinuria
0MCD, FSGS, MPGN, MN
0Diabetic nephropathy
• Definition (on 3 separate occasions): 0Others: PSGN, lgA nephropathy,
Fixed 0 ;,+l on dipstick with SG >l.015, SLE, HSP,Alport syndrome
Proteinuria or
0 UPCR;, 0.2 • Tubular proteinuria
° Fanconi Syndrome
0 X-Linked tubular syndrome
0 Dent disease

II. GENERALWORK-UP FOR PROTEINURIA

INDICATIONS INTERPRETATION REMARKS
1) Urine Dipstick Test

• Routine • Normal should be negative • False positive:

screening or trace if specific gravity 0 Alkaline urine (pH>7.0) or very

in selected (SG) ;el.020 concentrated urine (SG >l.025)

population • Nephrotic range usually 0 Presence of blood, pyuria,
+3 or +4 prolonged dipstick immersion
• Dipstick interpretation: • False negative:
0 Trace: 10-29 mg/dL 0 Low urine pH ( <4.5)

0 1 +: 30-100 mg/dL 0 Dilute urine

0 2+: 100-300 mg/dL 0 If albumin is not the urinary

0 3+: 300-1000 mg/dL
protein
0 4+: >1000 mg/dL
• Normal SG: 1.015-1.025
2) 24-hr Urine Protein and Creatinine Excretion

• Quantitation of • Normal: <100 mg/m 2/24h • More accurate

proteinuria or <150 mg/24hr
• Nephrotic range: 3.5 g/24h
or> 40 mg/1112/hr

398
 3) Spot Urine for Protein/Creatinine Ratio (UPCR}
• Semiquantitative • Normal: • Easier collection
assessment of 0 <0.2 mg protein/mg creatinine in >2 yrs old • Use first morning void
proteinuria 0 <0.5 mg protein/mg creatinine in 6-24 mos • Less accurate than
• Nephrotic range: >2 mg protein/mg creatinine 24hr urine collection
• Not readily available
4) Microalbuminuria
• Assess risk of • Normal: <30 mg of urine albumin per gram of creatinine on first
progressive morning void
glomerulopathy
Source:KliegmanR.et al. NelsonTextbookof Pediatrics(21sted). Elsevier:2020

NEPHROTIC SYNDROME (NS)

• Suspect when there is nephrotic range proteinuria (>3.5 g/24 hours or UPCR >2) PLUS
the clinical triad of nephrotic syndrome:
0 Hypoalbuminemia (,; 2.5 g/dL)
0 Edema
0 Hyperlipidemia (cholesterol >200 mg/dL)
• Mnemonic: "EPAL" - Edema, Proteinuria, Albuminemia(hypo), Lipidemia(hyper)

I. ETIOPATHOGENESIS
• More common in males
• Most common cause in children is minimal change disease & most respond to steroids
• Among those nonresponsive to corticosteroids, 80% would be FSGS

A. Hallmarks of Nephrotic Syndrome:

HALLMARK FINDINGS/REMARKS
Heavy Proteinuria
• Due to increased permeability of the glomerular capillary wall
(40 mg/m 2 /hour)
Hypoalbuminemia
• Due to urinary protein loss
(,;2.5 g/dL)
• Underfill hypothesis: proteinuria leads to a decrease in plasma
protein & low intravascular oncotic pressure, resulting in
leakage of plasma water into interstitium, leading to edema
Edema
• Overfill hypothesis: nephrotic syndrome is associated with
urinary sodium retention leading to volume expansion and
leakage of excess fluid into the interstitium
• Low albumin stimulates generalized hepatic protein synthesis
Hyperlipidemia • Increased urinary loss of liproprotein lipase causes decreased
lipid catabolism thus elevating serum lipid levels

8. Types of Nephrotic Syndrome

10% have secondary NS
I
0 90% have idiopathic form:
Minimal change disease (85%): around 95% respond to steroids
° Focal segmental glomerulosclerosis (10%): only 20% respond to steroids
Mesangial proliferation (5%): around 50% respond to steroids

II. MANIFESTATIONS
• Generalized edema (including genitals), anorexia, irritability, abdominal pain
• Hypertension and gross hematuria are uncommon

399
 I~-,
Ill. OVERVIEW OF THE TYPES OF NEPHROTIC SYNDROME
I I Membranoproliferative Glomerulonephritis
Focal Segmental I
Minimal Change
Glomerulosclerosis I Membranous Nephropathy I (MPGN)
-- ---~---
Disease (MCD) (MN) 1~· ~-

i
I I
(FSGS) I
I I
-,.y--;e Type II

• Sex (M:F) • 2:1 • 1.3:1 • 2:1 • 1:1 • 1:1

Manifestations
• Nephrotic Syndrome • 100% • 90% • 80% • ~60% • ~60%
• Asymptomatic
• 0 • 10% • 20% • 40% • 40%
proteinuria

• Hentaturia • 10-20% • 60-80% • 60% • 80% • 80%

• Hypertension • 10% • ~20% • Uncommon • 35% • 35%
• Rate of progression to
• Does not progress • 10 years • 50% in 10-20 years • 10-20 years • 5-15 years
renal failure
• HIV,heroin use,sick1ecell • Renalveinthrombosis,drugs,SLE,
• Associated conditions • None • None • Partial lipodystrophy
disease, reflux nephropathy hepatitis8 and C,lymphoma,tumors
• Increased SUN • Increased BUN • Low complement • Normal Cl, C4
• Laboratory findings • Normal complement levels
• Normal complement • Normal complement levels; Cl, C4, C3-C9 • Low C3-C9
• Thickened GBM,
• Light Microscopy • Normal • Focal sclerotic lesions • Thickened GBM, spikes • Lobulation
proliferation
• lmmunotluorescence • Negative • lgM, C3 in lesions • Fine granular lgG, C3 • Granular lgG, C3 • C3 only
• Mesangialand
• Electron Microscopy • Fusion of foot processes • Foot process fusion • Subepithelial deposits • Dense deposits
subendotl,elialdeposits
• Remission after oral • Not established/ • Not established/
• 90% • 15-20% • Resistant
corticosteroid therapy resistant resistant
Source:KliegmanR.et al.Nelson
Textbook
ofPediatrics
(21sted).Elsevier:
2020
PhadkeK,et al.Manual
ofPediatric
Nephrology.Springer:
2014
IV. DIAGNOSIS
DIAGNOSTICS FINDINGS/REMARKS
• 3+ to 4+ proteinuria
Urinalysis • Urinary protein excretion >40 mg/m 2/hr
• UPCR >2
• Serum creatinine usually normal
Blood Chemistry • Serum albumin <2.5 g/dL
and Immunology • Elevated serum cholesterol & triglycerides
• C3 & C4 levels are normal
• Gross hematuria
• Hypertension
Indications for
• Renal insufficiency
Renal Biopsy in NS
• Hypocomplementemia
• Age <l year old or> 12 years old

V. MANAGEMENT
A. Steroids
If onset at 1-8 years old: likely steroid-responsive minimal change form (steroids may
be initiated without renal biopsy)
0 Prednisone 60 mg/m 2/day OR 2 mg/kg/day (max dose 60 mg daily) for 4-6 weeks
Taper prednisone after 4 weeks to alternate day therapy (starting at 40 mg/m' /day or
1.5 mg/kg/day) for 8 weeks to 5 months
DESCRIPTION
Response • Remission attained during first 4 weeks of steroid therapy
Remission • UPCR <0.2 or dipstick <l for 3 consecutive days
Relapse • UPCR >2 or dipstick ;,3+ for 3 consecutive days
Steroid • Two consecutive relapses during corticosteroid therapy or within 14
Dependent days of ceasing therapy
Steroid • Failure to achieve complete remission after 8 weeks of steroid therapy
Resistant • Requires renal biopsy and referral to specialist

B. Other Aspects in Management

ASPECT MANAGEMENT
• First episode with mild-moderate edema: can be treated as outpatient
General • Severe symptomatic edema (effusions, ascites) should be admitted
• Tuberculosis must be ruled out prior to starting steroid therapy

Fluid
Balance
•
•
•
•
Fluid restriction and low sodium diet
Diuretics: furosemide 1 mg/kg/dose IV
Intravenous 25% human albumin 0.5 g/kg
Monitor volume status, serum electrolytes, renal function
I
• Pneumococcal vaccine (ideally when in remission and off daily steroids)
• Varicella vaccine (defer live vaccines until the prednisone dose is below
Vaccination
either 1 mg/kg daily or 2 mg/kg on alternate days)
• Influenza vaccine

VI. COMPLICATIONS
• Infection :major complication of NS (e.g., spontaneous bacterial peritonitis/SBP, bacteremia)
• Organisms: Streptococcus pneumoniae (most common in children) and E.coli
• Peritoneal leukocyte counts of>250 cells/uL highly suggestive ofSBP

VII. PROGNOSIS
• If steroid-resistant, FSGSis the usual form and has a much poorer prognosis
• If in remission, they are considered normal and may have unrestricted diet and activity
Source:KDOQIclinicalpracticeguidelines for CKD:AmJ Kidney:2002
KliegmanR, et al. NelsonTextbookof Pediatrics(21sted).Elsevier:2020
401
 SECTION THREE
APPROACH TO AZOTEMIA

GLOMERULAR FILTRATION RATE (GFR)

I. COMPUTATIONOF ESTIMATEDGFR (eGFR)
• There are several formulas used to compute for the eGFR
• In pediatric patients >1 year old, the most commonly used and recommended formula
is the Modified Schwartz

Modified Schwartz Formula (for patients >1 year old)

Height in cm x 36.5
eGFR=
Creatinine in mmol/l

OR

Height in cm x 0.413
eGFR=
Creatinine in mg/dl

Source:MilikuK, el al.AmericanJournalof Nephrology:

2017
AvnerE, el al. PediatricNephrology
(7thed).Springer:2016

RENAL FAILURE
I. ACUTE KIDNEYINJURY
• Abrupt loss of kidney function which leads to a rapid decline in the GFR, accumulation
of waste products, and dysregulation of extracellular volume and electrolyte homeostasis

A. Pediatric-Modified RIFLE (pRIFLE) Criteria

CRITERIA CREATININE eGFR URINE OUTPUT

• <0.5 ml/kg/hr for
Risk • Increase > l.Sx • Decreased by ;,25%
;,8 hours
0 Decreased by • <0.5 ml/kg/hr for
Injury • Increase ;, 2x
;,SO% e,16 hours
• Increase ;,3x or
• Decreased by ;,75% • <0.3 ml/kg/hr for
>4 mg/dL with an
Failure or <35 ml/ e,24 hours or anuric
acute rise of >0.5
min/l.73m 2 for ;,12 hours
mg/dL
Loss • Persistent failure >4 weeks
End-stage • Persistent failure >3 months

Source:KDIGOClinicalPracticeGuidelineforAcuteKidneyInjury.Kidneyinter.,Suppl:2012

402
 B. Indications for Dialysis in Acute Kidney Injury:
Anuria/oliguria
Volume overload with hypertension/pulmonary edema refractory to diuretics
Persistent hyperkalemia
Severe metabolic acidosis unresponsive to therapy
Uremia (encephalopathy, pericarditis, neuropathy)
Blood urea nitrogen >100-150 mg/dL (or lower if rapidly rising)
Calcium:Phosphorus imbalance, with tetany that cannot be controlled
0 Inability to provide adequate nutrition due to severe fluid restriction

C. Common Causes of Acute Kidney Injury

PRERENAL INTRINSIC RENAL POSTRENAL

• Decreased effectivecirculating
• Renal parenchymal
arterial volume leading to • Obstruction of the
damage with sustained
inadequate renal perfusion urinary tract
hypoperfusion and ischemia
and a decreased GFR
• Glomerulonephritis
• Dehydration, • Posterior urethral valves
• HUS
gastroenteritis • Urolithiasis
• Acute tubular necrosis
• Burns, sepsis, hemorrhage, • Tumors
• Renal vein thrombosis
capillary leak • Ureteropelvic and
• Acute interstitial nephritis
• Hypoalbuminemia ureterovesicular
• Rhabdomyolysis
• Cardiac failure junction obstruction
• Tumor infiltration
• Anaphylaxis • Urethral strictures
• Drugs and toxins
• Cirrhosis • Hemorrhagic cystitis
• Tumor lysis syndrome
• Abdominal compartment • Neurogenic bladder
• Vasculitis
syndrome • Anticholinergic drugs
• Cortical necrosis
Source:KliegmanR, et al. Nelsontextbookof pediatrics(21sted.).Philadelphia:
Elsevier;2020

II. CHRONICKIDNEYDISEASE (CKD)

A. Criteria for Definition of CKD

1. Kidney damage for ;;,3 months. Defined by structural or functional abnormalities of

the kidney, with or without decreased GFR, manifested by 1 or more of the following:
Abnormalities in the composition of the blood or urine
0

Abnormalities in imaging tests

0

Abnormalities on kidney biopsy

0

2. GFR <60 ml/min/1.73111 2 for ;;,3 months, with or without the other signs of kidney
damage described above

B. Stages of Chronic Kidney Disease

I
STAGE DESCRIPTION GFR (ml/min/1.73m 2)

1 • Kidney damage with normal or increased GFR • >90

z • Kidney damage with mild decrease in GFR • 60-89
3 • Moderate decrease in GFR • 30-59
4 • Severe decrease in GFR • 5-29
5 • Kidney failure • <15 or on dialysis
Source:KDOQIclinicalpracticeguidelines
for CKD:AmJ Kidney;2002

403
 SECTION FOUR
SPECIFIC DISORDERS IN NEPHROLOGY

ACUTE POSTSTREPTOCOCCAL GLOMERULONEPHRITIS (PSGN)

I. ETIOPATHOGENESIS
• Follows infection of the throat (serotypes 1, 4, 25, and some strains of 12) or skin (type 49)
by nephritogenic strains of group A 8-hemolytic streptococci (Strep pyogenes)
• Common in 5-12 years old; uncommon before 3 years old
• Exemplifies immune complex mediated reaction
0Involves activation of classic and alternative complement pathways
0 Localized in the glomeruli due to negatively charged capillary wall, mesangial trapping,
hydrodynamic forces

II. MANIFESTATIONS
• Latency period: 1-2 weeks a~er throat infection and 3-6 weeks after pyodenna
• Nonspecific symptoms: malaise, lethargy, abdominal pain, or flank pain are common
• Gross hematuria, periorbital edema, hypertension, oliguria
• Complications:
0Encephalopathy (considered if with severe headache, altered mental status, or new seizures)
0Heart failure due to hypertension or hypervolemia
• Complete recovery is expected in >95% of cases

III. DIAGNOSIS
DIAGNOSTIC REMARKS/FINDINGS
Urinalysis • RBCs, often with RBC casts, proteinuria, and PMN leukocytes
CBC • May have mild normochromic anemia
• Low serum C3 in >90% of patients. C3 characteristically normalizes
Serum C3
in 6-8 weeks
• ASO for throat infection
Documentation
• DNAse-B Antigen for pyoderma
of GABHS infection
• Rising antibody titers to streptococcus-antigen
Renal biopsy • See findings at table under common causes of glomerulonephritis

IV. MANAGEMENT
ASPECT REMARKS
• Early systemic antibiotics do not eliminate the risk of GN
Antibiotics • Penicillin G x 10 days is recommended to limit spread of
nephritogenic strains
• Sodium restriction
• Diuresis (Furosemide 1-2 mg/kg/dose IV q 6-12 hours)
• Antihypertensives: ACE inhibitors, or CCBs,or vasodilators
Fluids
• Fluid restriction: 400 mL/BSA + urine output in 24 hours
• Strictly monitor input and output, weigh daily. Re-adjust fluid limit daily.
• Titrate diuretics accordingly based on the clinical status of the patient
• Microscopic hematuria can persist for 12-24 months after the initial
presentation
Monitoring • Hypertension usually normalizes 4-6 weeks after onset
• Acute phase generally resolves within 6-8 weeks
• C3 should normalize after 8 weeks

404
lgA NEPHROPATHY OR BERGER DISEASE
I. ETIOPATHOGENESIS
A. Epidemiology
0 Most common chronic glomerular disease worldwide
0 More common in males
B. Pathology
Immune complex disease that appears to be caused by abnormalities in the lgA
immune system
Predominance of lgA within mesangial deposits of the glomerulus in the absence of
systemic diseases

II. MANIFESTATIONS
• Gross and/or microscopic hematuria, associated with URTl or GI infection 1-2 days prior to
its onset
• May present as nephritic syndrome, nephrotic syndrome, or a combined nephritic-
nephrotic syndrome

III. DIAGNOSIS
• Normal serum C3 level
• Serum lgA has no diagnostic value (increased in only 15% of patients)

IV. MANAGEMENT
ASPECT REMARKS
ACE inhibitors
• Effective in managing hypertension and reducing proteinuria
&ARBs
Fish oil (omega-3
• May decrease the rate of renal progression
fatty acids)
• May be beneficial in some patients, if ACE inhibitors and ARBs are
Steroids
ineffective

V. PROGNOSIS
• Does not lead to significant kidney damage in most children
• Progressive disease in 20-30% of children at 15-20 years after onset
• Most children will not show progressive renal dysfunction until adulthood
• Poor prognosis if: persistent hypertension, renal dysfunction, heavy /prolonged proteinuria

HEMOLYTIC-UREMIC SYNDROME (HUS)

I. ETIOPATHOGENESIS
• One of the most common causes of acute renal failure in young children
• Triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency
I-:
• Usually occurs in preschool and school aged children

A E.t10Iogy
Infection • Most common form of HUS is due to toxin-producing E.coli

Diseases with • SLE

microvascular • HELLP syndrome (hemolytic anemia, elevated liver enzymes, low
injury platelet count syndrome)
• Genetics
Others • Drugs (calcineurin inhibitors, cytotoxic agents, clopidogrel and
ticlopidine, quinine]

B. Pathology
Toxin initiates endothelial cell injury which leads to microvascular injury
Microangiopathic anemia is due to mechanical damage to RBCs as they pass through
the altered vasculature
Thrombocytopenia is due to intra-renal platelet adhesion or damage
405
II. MANIFESTATIONS
• Most common in <4 years old
• Onset is usually preceded by gastroenteritis
• Sudden onset of pallor, irritability, weakness, lethargy, and oliguria
• Usually occurs 5-10 days after the initial illness
• Physical examination: dehydration, petechiae, hepatosplenomegaly, and marked irritability

III. DIAGNOSIS
A. Laboratory Criteria for Diagnosis of HUS

The following are both present at some time during the illness:
• Anemia (acute onset) with microangiopathic changes (schistocytes, burr cells, helmet cells)
on peripheral blood smear
• Acute renal injury: hematuria, proteinuria, elevated creatinine level (2el mg/dL in
<13 years old or >1.5 mg/dL in >13 years old or 2'50% increase over baseline)

B. Case Classification
CLASSIFICATION DESCRIPTION
• Meets the lab criteria but with no clear history of diarrhea in
the preceding 3 weeks, or
Probable
• Onset within 3 weeks of diarrhea, and meets the lab criteria
except that microangiopathic changes are not confirmed
Confirmed • Meets the laboratory criteria ANDbegan within 3 weeks of dian-hea

C 0th o· .
DIAGNOSTIC FINDINGS/REMARKS
• Leukocytosis, thrombocytopenia, anemia
CBCand peripheral
• Increased reticulocyte count
blood smear
• PBS findings: Helmet cells, burr cells, fragmented RBCs
• Negative Coombs test
• Hematuria & proteinuria
Others
• Normal PT & PTT
• Stool culture is often negative

IV. MANAGEMENT
• Correction of fluids & electrolytes derangements
• Aggressive nutrition
Supportive care • Early institution of peritoneal dialysis to decrease mortality
• BP control
• Red cell transfusion
• Not generally administered regardless of platelet count
Platelet
• Almost immediately consumed by active coagulation & can worsen
transfusion
the clinical course
Antibiotic
• No antibiotic therapy because this may lead to increased toxin release
therapy
• Complications (hypertension, chronic renal insufficiency, proteinuria)
Prognosis
may not be apparent for up to 20 years, long-term follow-up needed

406
AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE (ARPKD)
I. ETIOPATHOGENESIS
• Infantile polycystic disease (1:10,000 - 1:40,000)
• Autosomal recessive
• The gene for autosomal recessive PKD (PKHDl) encodes fibrocystin, a large protein
• Both kidneys are markedly enlarged, grossly showing innumerable cysts
• Progressive interstitial fibrosis and tubular atrophy eventually leads to renal failure

II. MANIFESTATIONS
• Bilateral flank masses during the neonatal period or early infancy
• Associated with oligohydramnios, pulmonary hypoplasia, respiratory distress,
spontaneous pneumothorax in the neonatal period
• Oligohydramnios complex or Potter facies:
0 Low-set ears, micrognathia, flat nose, limb-positioning defects, growth deficiency
0 Hypertension, hyponatremia, impaired renal function, hepatic fibrosis

III. DIAGNOSIS
• Strongly suggested by:
° Clinical findings (bilateral palpable flank masses in an infant with pulmonary
hypoplasia, oligohydramnios, hypertension), with
0 Normal renal ultrasound of parents
• Renal ultrasound: enlarged and hyper-echogenic kidneys with poor cortico-medullary distinction

IV. MANAGEMENT
• Supportive: ventilatory support, control BP, fluid and electrolyte correction, osteopenia
management, management of clinical manifestations of renal insufficiency

URINARY TRACT INFECTIONS (UTI)

I. ETIOPATHOGENESIS
A. Epidemiology
Prevalence during the 1st year oflife: M>F (ratio of 2.8-5.4:1)
0 Beyond 1-2 years old: M<F (ratio of 1:10)

B. Pathogenesis
0 Most UTls are ascending infections: bacteria arise from fecal flora and colonize the
perineum, and then enters the bladder via the urethra
Voiding dysfunction is a risk factor for UTI
C. Etiology
Caused mainly by colonic bacteria
0 In girls, 75-90% are due to Escherichia coli, followed by Klebsiellaand Protet1s
° For both sexes, Staphy/ococct1ssaprophyticus and Enterococcus

II. CLINICALFORMSOF UTI

Clinical
FORM DESCRIPTION
• Characterized by (any or all) abdominal, flank, or back pain, fever
I
Pyelonephritis (may be the only manifestation), malaise, nausea/vomiting
• Indicates bladder involvement (more localized urinary signs and
symptoms)
• Gross hematuria and dysuria
Cystitis • Urgency, frequency, malodorous urine, incontinence, supra pubic pain
• May occur in response to chemical toxins (penicillins, dyes,
insecticides, cyclophosphamide ), viruses, radiation, idiopathic
• Usually resolves within 1 week
• (+)urine culture without any manifestations of infection
Asymptomatic
• Most common in girls; incidence declines with increasing age
Bacteriuria
• Benign and does not cause renal injury except in pregnant women

407
III. DIAGNOSIS
DIAGNOSTICS* DESCRIPTION
• Necessary for confirmation and appropriate therapy
• Positive culture:
0Suprapubic or cathterized sample: >50,000 colonies of a single
Urine culture
pathogen OR 10,000 colonies and the child is symptomatic
0In a bag/midstream urine sample: single organism cultured with
a colony count >100,000 AND (+)urinalysis AND symptomatic
• Pyuria (leukocytes in the urine) suggests infection, but may be
Urinalysis present without UTI ••
• Nitrites and leukocyte esterase are usually positive in infected urine
Imaging Studies
• To assess kidney size, detect hydronephrosis and ureteral dilation
• To evaluate bladder anatomy
Sonogram of • Indicated for:
kidneys and
bladder ° First UTI <6 months old
0 No response to antibiotic therapy within 24-48 hours
• If ultrasound is abnormal, DMSAor VCUGmay be done
Dimercaptosuccinic • To assess renal scarring and identify areas of acute pyelonephritic
acid (DMSA) scan involvement
Voiding
cystourethrogram • To assess for reflux
(VCUG)

'Note on obtainingappropriateurinesamples:
In toilet-trained
children.a midstream
sampleis usuallysatisfactory
Childrenwho are not toilet-trained:catheterizedurinesampleshouldbe obtained

.. Sterilepyuria(positiveleukocytes.negativeculture)occursin partiallytreatedUTls,viral infections,renalTB, renalabscess,urinary

obstruction,urethritisdue to STls. inflammationnearthe ureteror bladder.and interstitialnephritis

Source: NationalInstitutefor Healthand CareExcellence.UTI in under16s:2007

CPGfor the Diagnosis& Managementof the InitialUTIin FebrileInfantsand Children2 to 24 Months:Pediatrics;2011
AmmentiA, et al. Acta Paediatr2012

IV. MANAGEMENT
TYPE DESCRIPTION
• <2 months: Cefotaxime + Amikacin for 10-14 days
• >2 months to 18 years old: Coamoxiclav, Cefuroxime, Ampicillin-
Sulbactam for 7-14 days
Acute • Nitrofurantoin can be given for adolescents (but ONLYfor acute
uncomplicated UTI:
Acute cystitis and cystitis)
pyelonephritis • Oral therapy is equally effective to IV therapy
• IV therapy preferred if seriously ill, cannot tolerate oral therapy
• Switch to oral therapy once afebrile for 24 hours and able to
tolerate oral therapy
Complicated UT!
• Refer to IDS, nephrologist and urologist
(catheter-related
or with co-morbids) • Ceftriaxone +/-Amikacin

Source:KliegmanR, el al. NelsonTextbookof Pediatrics(21sted). Elsevier;2020

NationalAntibioticGuidelinesfor UTI.2017
CPGfor the Diagnosis& Managementof the InitialUTI in FebrileInfantsand Children2 to 24 Months:Pediatrics:2011
Avner,E, et al. Pediatricnephrology;2016

408
RENAL TUBULAR ACIDOSIS
• Characterized by normal anion gap hyperchloremic metabolic acidosis in the setting of a
normal or near-normal GFR

I. ETIOPATHOGENESIS
TYPES PATHOGENESIS
• Inherited and persistent from birth or
occur transiently during infancy
• Usually occurs as a component of
Proximal (type
global proximal tubular dysfunction
II)RTA
(Fanconi syndrome: proteinuria,
glycosuria, phosphaturia,
aminoaciduria, and proximal RTA)
• Sporadic or inherited
• Can occur as a complication of
inherited or acquired diseases of the
Distal (type I)
distal tubules
RTA
• Results from impaired W ion
excretion, so urine pH cannot be
reduced to <5.5
• Due to impaired aldosterone production
(hypoaldosteronism) or impaired
renal responsiveness to aldosterone
(pseudohypoaldosteron ism)
Hyperkalemic
• Aldosterone has a direct effect on
(type IV) RTA
hydrogen secretion & stimulates K"
secretion in the collecting tubule
• Lack of aldosterone results in
acidosis and hyperkalemia
• Very rare, autosomal recessive
Combined • Due to inherited carbonic anhydrase
proximal and
2 deficiency
distal type
(type Ill) • Features of both proximal & distal RTA
CLINICAL MANIFESTATIONS
• Growth failure in the 1st year
of life, polyuria, hyponatremic
dehydration, anorexia,
vomiting, constipation,
hypotonia, hypokalemia
• Growth failure, hypokalemia,
nephrocalcinosis and
hypercalciuria
• Absent phosphate and
bicarbonate wasting
• Growth failure, hyperkalemia
• Polyuria and dehydration from
salt wasting are common
• Urine may be alkaline or acidic
• High urinary sodium levels
with inappropriately low
urinary K"
• Associated with osteopetrosis
or marble bone disease,
cerebral calcification, and
mental retardation
• Growth failure, bone fractures,
facial dysmorphism, conductive
hearing loss & blindness

II. DIAGNOSIS

FINDING

Urine pH with
PROXIMAL RTA

• <5.5
CLASSIC
DISTAL RTA

• >5.5
GENERALIZED
DISTAL
DYSFUNCTION

• <5.5 or >5.5
I
acidosis
Urine net charge • Negative • Positive • Positive
Fanconi lesion • Present • Absent • Absent
Fractional • >10-15% during
bicarbonate • 2-5% • 5-10%
excretion alkali therapy

Response to
• Least responsive • Responsive • Less responsive
therapy
Associated disease • Fanconi syndrome • Nephrocalcinosis • Renal insufficiency
Source:Kliegman
R,et al.NelsonTextbook
of Pedialrics(21sted).Elsevier;2020

409
III. MANAGEMENT& PROGNOSIS
• Bicarbonate replacement is the mainstay of therapy in all forms of RTA
0 Proximal RTAoften require bicarbonate ofup to 20 mEq/kg/day (e.g., sodium
bicarbonate or sodium citrate solution)
0 Distal RTAbase requirement is generally in the range of 2-4 mEq/kg/day and
requires monitoring for the development of hypercalciuria. Those with symptomatic
hypercalciuria, nephrocalcinosis, or nephrolithiasis may require thiazides to decrease
urine calcium excretion.
• Patients with Fanconi syndrome usually require phosphate supplementation
• Patients with type IV RTAcan require chronic treatment for hyperkalemia with sodium-
potassium exchange resin
• Prognosis of RTAlargely depends on the nature of any existing underlying disorder
• Those with treated isolated proximal or distal RTAgenerally show improvement in
growth as long as bicarbonate levels are maintained in the normal range

REFERENCES
1. Ammenti A, Cataldi L, Chimenz R, Fanos V, La Manna A, Marra G, et al., Italian Society of
Pediatric Nephrology. Febrile urinary tract infections in young children: recommendations for
the diagnosis, treatment and follow-up. Acta Paediatr 2012;101(5):45 le7.
2. Avner E, et al. Pediatric Nephrology 7th ed. 2016 Springer USA
3. Engorn, B., and Flerlage, j. The Harriet Lane Handbook 20th ed. 2015. Philadelphia: Elsevier.
4. Hemolytic Uremic Syndrome, Post-diarrheal I 1996 Case Definition. 2018. Available from
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case-definition/1996/
5. Kidney Disease: Improving Global Outcomes (KDIGO) Acute Kidney Injury Work Group.
KDIGO Clinical Practice Guideline for Acute Kidney Injury. Kidney inter., Suppl. 2012; 2 :
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6. Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group. KDIGO
Clinical Practice Guideline for Glomerulonephritis. Kidney inter., Suppl. 2012; 2: 139-274.
7. Kliegman, R., Stanton, 8., St. Geme, j., Schor, N., and Behrman, R.. Nelson Textbook of Pediatrics
21st ed. 2020. Canada: Elsevier.
B. Kuma1; Vinay, Abu! K. Abbas, and Jon C.Aster. Robbins and Cotran Pathologic Basis of Disease.
Ninth edition. 2017 Philadelphia, PA: Elsevier/Saunders
9. Miliku, K.,Bakker, H., Dorresteijn, E., Crans berg, K., Franco, 0., Felix, j., and jaddoe, V.Childhood
Estimates ofGlomerular Filtration Rate Based on Creatinineand Cystatin C: Importance ofBody
Composition.American journal ofNephrology, 2017 45( 4), 320-326. doi: 10.1159/000463395
10.National Institute for Health and Care Excellence. Urinary tract infection in under 16s:
diagnosis and management. 2007. Available from: https://www.nice.org.uk/guidance/
cg54/ evidence/full-guideline-pdf-196566877
11.National Kidney Foundation: KDOQI clinical practice guidelines for chronic kidney disease:
evaluation, classification, and stratification, Am j Kidney Dis 39(2 Suppl 1):sl-s266, 2002.
12.Phadke, K., Goodyer, P., and Bitzan, M. (2014). Manual of Pediatric Nephrology. London:
Springer.
13.Consensus Statements on Parenteral Fluid Therapy in Infants, Children and Adolescents.
Task Force on Fluid and Electrolyte Therapy. Philippine Pediatric Society, Inc. 2017
14.Urinary Tract Infection: Clinical Practice Guideline for the Diagnosis and Management of
the Initial UTI in Febrile Infants and Children 2 to 24 Months .. PEDIATRICS, 2011, 128(3),
595-610. doi: 10.1542/peds.2011-1330
15.Weening, j. The Classification of Glomerulonephritis in Systemic Lupus Erythematosus
Revisited. journal of The American Society of Nephrology, 2004, 15(2), 241-250. doi:
10.1097 /Ol.asn.0000108969.21691.5d
16.Welch, T. An Approach to the Child with Acute Glomerulonephritis. International journal of
Pediatrics, 2012, 1-3. doi: 10.1155/2012/426192

410
ENDOCRINOLOGY
 SECTION ONE
OISORDER'S OF THE HVPOTH·ALAMl)S & PITUITARY GLAND

OVERVIEW OF THE PITUITARY HORMONES

I. ANTERIOR PITUITARY HORMONES

HORMONES DESCRIPTION

• Causes linear & soft tissue growth, increase in bone thickness,

Growth hormone (GH) protein synthesis, fatty acid release from adipose tissue, insulin
resistance, and increase in blood glucose
..
Pr<ilactin (PRL) • For milk PRODUCTION

Thyroid-stimulating • Stimulates release of thyroxine (T4) and triiodothyronine (T3)

hormone (TSH) from the thyroid gland
Adrenocorticotropic
• Stimulates cortisol synthesis and secretion from the adrenal cortex
hormone (ACTH)

Luteinizing hormone • Promotes luteinization of the ovary and Leydig cell function of
(LH) the testis
Follicle-stimulating • Stimulates follicular development in the ovary and
hormone (FSH) gametogenesis in the testis

II. POSTERIOR PITUITARY HORMONES

HORMONES DESCRIPTION

Antidiuretic hormone • Increases water reabsorption via insertion of aquaporin-2 in

(ADH) the renal collecting duct
• For milk SECRETION
Oxytocin
• Stimulates uterine contractions during labor and delivery
Source:KliegmanRM,et al. NelsonTextbook
of Pediatrics(21sted)Elsevier;2020
KoeppenM.,et al. Berne& LevyPhysiology. Mosby/Elsevier;
2010

HYPOPITUITARISM
I. ETIOPATHOGENESIS
• Deficiency of growth hormone with or without a deficiency of other pituitary hormones
• Congenital: if with severe defect of GH production or action, height will fall >4 SD below
the mean for length by 1 year old
• Acquired: child is normal initially and manifestations gradually appear and progress

II. MANIFESTATIONS
• Severe growth failure, tendency for hypoglycemia, micropenis, prolonged neonatal jaundice
• Short & broad face, prominent frontal bone, depressed nasal bridge, saddle-shaped nose,
underdeveloped mandible, short neck, high-pitched voice, well-proportioned extremities
I
but small hands & feet, delayed or absent sexual maturity
• Acquired: atrophy of adrenal cortex, thyroid & gonads result in weight loss, asthenia,
sensitivity to cold, and absence of sweating

413
 DIFFERENTIALS FOR
SHORT STATURE DESCRIPTION

• Height is initially within normal range that starts falling off the
height curve over time
Pathologic Short • Delayed bone age
Stature • Possible causes: Prenatal onset (maternal infections and
undernutrition, chromosome defects) & postnatal onset (nutritional
deficiency, chronic systemic disease, psychosocial deprivation)
• Height is sustained at lower percentiles during childhood
• Hallmark finding: delayed pubertal growth spurt
Constitutional • Eventual normal final adult height is reached (catch-up growth
Growth Delay occurs late adolescence)
• Often with history of similar growth pattern with family members
• Delayed bone age (chronologic age > bone age)
• Stays parallel to the growth curve
Familial Short
• Significant number of family members who are short
Stature
• Bone age is not delayed (chronologic age = bone age)
Source:KliegmanRM,et al. NelsonTextbook of Pediatrics(21sted)Elsevier;2020
LifshitzF,el al. (5thEd.).PediatricEndocrinology.
lnformaHealthcare;2007

III. DIAGNOSIS
A. Examination
Diagnosis is suspected with severe postnatal growth failure defined as any of the following:
0 Height< 1st percentile for age and sex
0 Height> 2 SD below sex-adjusted mid-parental height
Height velocity is low relative to sex- and bone age-matched peers
See Growth and Development Chapter for proper height determination

B. Diagnostic Tests
• Absent or low levels of GH in response to stimulation or
provocative test (e.g., rapid administration of insulin, arginine,
clonidine, levodopa, or glucagon)
Definitive
Diagnosis • Always assess thyroid function prior to provocative GH test as it
is a prerequisite for normal GH synthesis
• Necessary to examine other pituitary functions (TSH, T3, T4,
cortisol, ACTH,gonadotropins, gonadal steroids)
• Delayed skeletal maturation / bone age
Radiologic
Findings • Small anterior pituitary gland
• Suprasellar calcification (if associated with craniopharyngioma)
Source:KliegmanRM,el al. NelsonTextbook
of Pediatrics
(21sted)Elsevier;2020
RogolAD,el al. J Pediatr;2014

IV. MANAGEMENT
Human Growth • Should be started as soon as diagnosis is made
Hormone (hGH) • Maximal response occurs in the 1" year of therapy with growth
Treatment Guide velocity usually above the 9S'h percentile for age
• Decision by the patient that he/she is tall enough
Criteria for stopping
treatment • Growth rate <l inch/year
• Bone age >14 years in girls & >16 years in boys
Source:RogolAD,et al. J Pediatr;2014
KliegmanRM,et al. NelsonTextbook
of Pediatrics
(21sted) Elsevier;2020

414
HYPERPITUITARISM
I. ETIOPATHOGENESIS
• Overproduction of GH leads to:
Gigantism if occurring among young patients with open epiphyses
0 Acromegaly if with closed epiphyses

II. MANIFESTATIONS
GlGANTlSM ACROMEGALY

• Longitudinal growth acceleration (cardinal • Coarse facial features

feature) • Increased skull circumference
• Coarse facial features • Broad nose
• Enlarged hands & feet • Enlarged tongue and jaw
• Behavioral problems • Separation of teeth
• Visual field defects, loss of visual acuity • Enlargement of distal parts of the body
• Headache • Visual field defects
• Visceromegaly • Dorsal kyphosis
Source:KliegmanRM,el al. NelsonTextbookof Pediatrics(21sted) Elsevier;2020
LifshitzF,et al. (5th Ed.).PediatricEndocnnology.
lnformaHealthcare;2007

III. DIAGNOSIS OF GH EXCESS

• Elevated insulin-like growth factor binding protein 3 (IGFBP-3) or IGF-1
Screening
(serum somatomedin CJ
• Diagnosis is confirmed by oral glucose-suppression test: serum GH
Confirmatory
levels are not suppressed by glucose load

IV. MANAGEMENT
• If with well-circumscribed pituitary adenomas: transsphenoidal surgery (complete
removal of the tumor)
• Pituitary irradiation if with local invasion, involvement of optic chiasm/nerve, or
unsuccessful surgery
• Octreotide (somatostatin analog) for GH suppression, pegvisomant [GH receptor antagonist)
Source:KliegmanRM,et al. NelsonTextbookof Pediatrics(21sted) Elsevier;2020
RootAW.PediatricEndocrinology; 2007

DIABETES INSIPIDUS (DI)

I. ETIOPATHOGENESIS
• Results from vasopressin deficiency [central DI) or vasopressin receptor insensitivity
(nephrogenic DI)
• Vasopressin is the principal regulator of tonicity (secreted in the posterior pituitary
gland) which has both anti diuretic & vascular pressor activity

II. MANIFESTATIONS& DIAGNOSIS

I
• Cardinal features: polyuria and polydipsia (exceeding 2 L/m 2 /24hr)
Request for the following:
, Serum osmolality
BUN, creatinine, Na', K·, Ca2 •
Glucose
Urine osmolality, specific gravity, urine glucose
• DI is established if serum osmolality is >300 mOsm/kg & urine osmolality <300 mOsm/kg
• For patients with serum osmolality >270 but <300 mOsm/kg, perform water deprivation
test to establish diagnosis and differentiate central from nephrogenic DI

415
III. DIFFERENTIAL DIAGNOSIS
SYNDROMEOF
INAPPAPROPRIATE
CEREBRAL
SALT NEPHROGENIC
PARAMETER ANTIDIURETIC CENTRAL DI
HORMONE
WASTING DI
SECRETION(SIADH)
(CSW)

Serum Na' Low Low High High

Serum
Low Low High High
osmolality
Urine Na' High Very high Low Low

Urine output Normal or low High High High

lntravascular
High Low Low Low
volume status
Vasopressin
High Low Low High
level
Thiazides +/-
Limit oral fluid NaCl & H2 O Fluid therapy
Management indomethacin or
intake replacement Vasopressin
amiloride

IV. MANAGEMENTBASED ON ETIOLOGY

CENTRAL/NEUROGENIC DI NEPHROGENIC DI

• Congenital
• Congenital
• Hypercalcemia, hypokalemia
• Trauma (deceleration injury)
• Renal disease (polycystic kidney
• Tumors (craniopharyngioma,
disease, chronic renal failure,
lymphoma, leukemia)
acute tubular necrosis, obstructive
• Autoimmune
uropathy)
Etiology • Infection (meningitis, TB)
• Drugs (lithium, cisplatin,
• Drugs (ethanol, phenytoin,
methoxyflurane, demeclocycline)
opiate antagonists, alpha-
• Infiltrating lesions
adrenergic agents)
• Vascular (sickle cell anemia)
• Vascular (bleed or infarction)
• Solute diuresis (glucose, mannitol, Na.,
• Idiopathic in 10%
radiocontrast dyes)
• Treat underlying disorder
• Fluid therapy
• Thiazides (decrease overall urine
• Long-acting vasopressin
output by enhancing Na' excretion
analog DDAVP
at the expense of water & by causing
• Acute onset after
Management decrease in GFR)
neurosurgery: vasopressin
• Indomethacin and amiloride +/-
with total fluid intake
thiazides to further reduce polyuria
limited to 1 L/m 2/day during
• Ensure intake of adequate calories for
antidiuresis
growth & to avoid severe dehydration
Source:KliegmanRM,et al. NelsonTextbook of Pediatrics(21sted)Elsevier;2020
LifshitzF.et al. (5thEd.).PediatricEndocrinology.
lnformaHealthcare;
2007
JohnCA,et al. GritCareNurse;2012

416
PRECOCIOUS PUBERTY
I. ETIOPATHOGENESIS
• Onset of secondary sexual characteristics (breast development or testicular development)
before 8 years old in girls & 9 years old in boys
• Classification based on primary source of hormonal production:

CENTRAL PRECOCIOUS PERIPHERAL PRECOCIOUS

PUBERTY
CLASSIFICATION
(Gonadotropin Dependent or
True Precocious Puberty)
• Idiopathic (most frequent cause
among females), brain lesions
(e.g., hypothalamic hamartoma,
Etiology
tumor, hydrocephalus, trauma,
myelomeningocoele, abscess,
radiation), untreated hypothyroidism
• Elevated LH, FSH
• Serial blood samples during sleep
reveals pulsatile LH secretion
• Pubertal levels of testosterone or
Laboratory
estradiol
findings
• GnRH or GnRH agonist (leuprolide)
stimulation test leads to "pubertal"
LH response or pubertal levels of
estradiol
• Advanced osseous maturation
• Pelvic ultrasonography in girls:
progressive enlargement of the
Imaging ovaries & uterus
• MRI: physiologic enlargement of
pituitary gland as seen in normal
puberty
Treatment • GnRH agonist
PUBERTY
(Gonadotropin-lndependent
or Pseudo-Precocious Puberty)
• Ovarian tumors, Leydig cell
tumo,; adrenal tumo,; congenital
adrenal hyperplasia, teratoma,
McCune-Albright syndrome, etc.
• Suppressed LH, FSH
• Elevated testosterone or
estradiol
• No response to GnRH or
leuprolide stimulation test
• Advanced osseous maturation
• Polyostotic fibrous dysplasia in
McCune-Albright syndrome
• Aromatase inhibitors: letrozole,
testolactone
• Anti-estrogens: tamoxifen
• Anti-androgens: spironolactone,
flutamide, bicalutamide
• Inhibitor of testosterone
synthesis: ketoconazole

II. INCOMPLETE(PARTIAL) PRECOCIOUSPUBERTY

• Isolated manifestations of puberty without development of other pubertal signs

• Transient isolated breast development without other pubertal changes

Premature • Most often occurs in the first 2 years of life
thelarche • Menarche occurs at expected age, reproduction is normal

I
• Infrequently may progress to precocious puberty
• Early appearance of pubic hair
Premature • Early increase in adrenal androgen production
pubarche/ • Increased prepubertal growth velocity but decreased pubertal
adrenarche growth, so adult height is not affected
• May herald disorders of androgen synthesis

Premature • Majority will have only 1-3 episodes of bleeding

menarche (rare) • Puberty occurs at usual time
Source:Kliegman RM,et al. NelsonTextbook of Pediatrics 2020
(21sted) Elsevier:
lnformaHealthcare;
LifshitzF,et al.(5thEd.).PediatricEndocrinology. 2007
BrookCGD,et al. Blackwell 2008
Publishing;
LeePA,et a. PediatricEndocrinology;2007
417
 SECTION TWO
DISORDERS OF THE THYROID GLAND

OVERVIEW OF THE THYROID, PARATHYROID AND RELATED HORMONES

HORMONES DESCRIPTION
• Increases oxygen consumption and basal metabolism
• Affects growth, differentiation, carbohydrate and lipid metabolism
Thyroid • Three forms:
hormones , Thyroxine (T4): prohormone
0 Triiodothyronine (T3): active form
, Reverse T3 (rT3): inactive form
• Secreted by the thyroid gland
Thyroxine (T4)
• ~70% is bound to thyroxine-binding globulin (TBG)
• Active form of thyroid hormone (3-4x more potent than T4)
Triiodothyronine
• Only 20% is secreted by the thyroid gland, majority is produced by
(T3)
deiodination ofT4 in the peripheral tissues
• Synthesized by the chief cells of the parathyroid gland
Parathyroid
• Stimulated by hypocalcemia
hormone (PTH)
• Increases serum Ca2 • and lowers serum PO4
• Promotes calcium & phosphate absorption from the small intestines,
VitaminD reabsorption from the kidneys and deposition to the bones
• Increases serum Ca2 ' and serum PO4
• Synthesized by the parafollicular cells of the thyroid gland
Calcitonin • Inhibits osteoclast activityleading to decreased resorption of Ca2' from bone
• Calcitonin levels increase in response to hypercalcemia
Source:KliegmanRM,et al. NelsonTextbookof Pediatrics(21sted) Elsevier:2020
KoeppenBM,Berne& LevyPhysiology. Mosby/Elsevier;2010

HYPERTHYROIDISM
• Excessive secretion of thyroid hormone
• During childhood, the most common cause is Graves disease (at least 95% of cases)

I. GRAVES DISEASE
A. Etiopathogenesis
0 Autoimmune disorder: production of thyrotropin receptor-stimulating antibody (TRSAb)
Females> males (5:1 ratio)
Most common cause of pediatric hyperthyroidism

B, Manifestations
SYMPTOMS SIGNS

• Earliest possible sign: emotional disturbance with

• Finger tremors
• Nervousness, headaches,
difficulties with sleep, poor
concentration
• Voracious appetite with loss
or no increase in weight
• Flushed skin with excessive
sweating, heat intolerance
motor hyperactivity
• Diffuse enlarged thyroid (goiter)
• Exophthalmos (due to antibodies against antigens
shared by the thyroid & eye muscle)
• Retraction of the upper eyelid (Dalrymple sign),
lagging of upper eyelid on downward gaze, infrequent
blinking, impairment of convergence
• Tachycardia, weight loss, palpitations, dyspnea,
increased blood pressure, widened pulse pressure

418
C. Diagnosis
DIAGNOSTIC FINDINGS/REMARKS

Thyroid Function • Increased T4 and T3

Tests • LowTSH
Thyroid Antibodies • Thyroid Receptor Stimulating antibody (TRSAb)confirms diagnosis
• Diffusely enlarged thyroid gland
Thyroid • Often homogeneous with normal or hypoechogenicity
Ultrasound and
Scintigraphy • RAI uptake measured in scintigraphy if history, PE, & laboratory
tests cannot establish the diagnosis

D.Management
1 !
, ,
METHIMAZOLE /
DRUG PROPYLTHIOURACIL (PTU)
CARBIMAZOLE

• Inhibit iodine organification

• Blocks the organification of
Mechanism of and coupling in the thyroid
iodide needed to synthesize the
Action • Also inhibits peripheral
thyroid hormone
conversion ofT4 to T3
• More potent than PTU
• Heavily protein bound, less
• Carbimazole is the prod rug of
able to cross placenta &
methimazole
pass into breastmilk
Properties • Consensus is to use methimazole
• Preferred drug during
for children
pregnancy and lactation
• Clinical response is evident
within 3-4 months
• Initial dose of0.5-1 mg/kg/day
Administration • Administered 3x daily
given OD or BID(max: 40 mg/day)
• Transient granulocytopenia
• Transient urticaria! rashes
Adverse effects
• Agranulocytosis
• Hepatitis, lupus-like polyarthritis syndrome, glomerulonephritis

2. Radioactive Iodine Ablation or Thyroidectomy

Indications:
Inadequate cooperation for medical management
• Medical management failed to result in permanent remission
• Severe side effects preclude use of antithyroid drugs
Patients should be in a euthyroid state prior to radiofrequency ablation or surgery
0

Thyroidectomy is useful when a coexisting suspicious nodule is present

0

° Consequence: permanent hypothyroidism

3. Complications of Therapy
Radiofrequency ablation: might worsen ophthalmopathy
Thyroidectomy: recurrent laryngeal nerve damage, hypoparathyroidism
0

Source:KliegmanRM.et al. NelsonTextbookof Pediatrics(21sted) Elsevier;2020

LifshitzF,et al. (5th Ed.).PediatricEndocrinology.lnformaHealthcare;2007
2014
LegerJ, et al. PaediatricThyroidology;
I
AbrahamP,et al. EurJ Endocrinol;2005

419
II. THYROID STORM
• Acute-life-threatening surge of thyroid hormone in the blood usually precipitated by
surgery, trauma, infection. or radioactive iodine treatment
• Fever, tachycardia, high output cardiac failure, gastrointestinal dysfunction (vomiting,
diarrhea, jaundice), neurologic changes (confusion, agitation, obtundation)
• With rapid progression to delirium, coma, and death
GOAL MANAGEMENT
• Propylthiouracil
Inhibition of thyroid • Saturated solution of potassium iodide (used only after
hormone formation and
secretion PTU has been administered to avoid potential increase in
thyroid hormone synthesis)

Sympathetic blockade • Propranolol

Therapy for relative

• Prednisone
glucocorticoid insufficiency

HYPOTHYROIDISM
• Due to deficient production of hormone or a defect in hormonal receptor activity
• May be congenital or acquired
I. CONGENITALHYPOTHYROIDISM
A. Etiopathogenesis
Thyroid dysgenesis (aplasia, hypoplasia, ectopia): most common cause of congenital
hypothyroidism
0 Dyshormonogenesis (defect in thyroid hormone synthesis)
TSH unresponsiveness
Endemic goiter (iodine deficiency)
0 Maternal antibodies: thyrotropin receptor-blocking antibody (TRBAb)
0 Maternal medications (e.g., PTU, methimazole, iodide, amiodarone, radioiodine)
B. Manifestations
SYMPTOMS SIGNS

• Usually asymptomatic at birth, and • Earliest sign: prolonged physiologic jaundice

birth weight & length are normal
(requires newborn screening tests
for early identification)
• Feeding difficulties, sluggishness,
lack of interest, somnolence
• Respiratory difficulties due to large
tongue
• Constipationunresponsive to treatment
• Progressive retardation of physical
and mental development
• Large abdomen, usually with umbilical hernia
• Hypothermic: cold and mottled skin
• Dry and scaly skin
• Edema of the genitals and extremities
• Stunted growth, short extremities
• Depressed nasal bridge, narrow palpebral
fissures, swollen eyelids, delayed dentition,
short and thick neck, deposits of fat above the
clavicles & between the neck & shoulders
• Coarse, brittle, scanty hair
• Hoarse voice, hypotonic
• Delayed sexual maturation

C. Diagnosis
DIAGNOSTIC FINDINGS/REMARKS
• Elevated serum TSH
Serum TSH
• Used in screening tests
• Low serum T4 and free T4
SerumT4
• No need to measure T3 (normal in many patients with hypothyroidism)
• Thyroid scintigraphy can determine underlying cause and show
ectopic gland (this establishes need for lifelong treatment)
Thyroid
• Thyroid ultrasound documents absence or presence of an
imaging
anatomically normal gland
• Rarely needed, treatment should not be delayed to request for imaging
420
 D.Management
• Treatment of choice: oral levothyroxine (10-15 mcg/kg/day once daily for 0-3 months old)
Normalization of thyroid function ideally within 2 weeks is vital in achieving optimal
neurodevelopmental outcome
• Regular monitoring of hormone levels: every 1-2 months in the 1st 6 months of life
then every 2-4 months between 6 months and 3 years of age

Source:KliegmanRM,el al. NelsonTextbook of Pediatrics(21sted)Elsevier;2020

lnformaHealthcare;
LifshitzF,el al. (5thEd.).PediatricEndocrinology. 2007

II. CHRONICLYMPHOCYTIC THYROIDITIS(Hashimoto Thyroiditis, Autoimmune Thyroiditis)

Most common cause of acquired hypothyroidism & thyroid disease in children/adolescents
• May be euthyroid, hypothyroid, or thyrotoxic (due to leakage of preformed thyroid
hormone into the circulation from the damaged gland lasting for up to 60 days)

A. Etiopathogenesis
• Organ-specific autoimmune disease characterized histologically by lymphocytic
infiltration between the thyroid follicles and presence of lymphoid germinal centers
• Thyroid anti-peroxidase antibodies (TPO-Abs) & thyroglobulin antibodies (Tg-Abs) in 95%
More common in girls

8. Manifestations
• Most common manifestation: goiter (diffusely enlarged, firm, nontender thyroid) and
growth deceleration
• Most of the affected children are clinically euthyroid & asymptomatic
Variable clinical course

C. Diagnosis
DIAGNOSTIC FINDINGS/REMARKS

• Often normal (euthyroid state)

FT4, TSH • May have subclinical hypothyroidism: elevated TSH, normal FT4
• May have overt hypothyroidism: elevated TSH, low FT4

Immunology • Presence of serum antibody titers to TPOAbs, Tg-ABS

• Not usually needed

Thyroid scan • Thyroid scan: irregular & patchy distribution with decreased
and ultrasound uptake of radioisotope
• Thyroid UTZ: heterogeneous echogenicity
• Definitive diagnosis
Biopsy • Done only for prominent nodules that persist despite therapy to
identify possibility of cancer

D.Management
May be self-limited (spontaneous regression) or may persist for years while the
patient remains euthyroid
If with thyrotoxicosis, address the thyroid tenderness with NSAIDs or a short course of
steroids if with severe pain; beta-blockers to alleviate palpitations I·-,_
0 Periodic re-evaluation every 6-8 weeks for early detection & treatment of hypothyroidism

CONDITION MANAGEMENT

If with evidence of
• Give levothyroxine ( age-specific dose)
hypothyroidism

If with subclinical • May give levothyroxine until growth and puberty are complete
hypothyroidism (still controversial)

Source:KliegmanRM,et al. NelsonTextbook of Pediatrics(21sted) Elsevier;2020

lnformaHealthcare;
LifshitzF,et al. (5thEd.).PediatricEndocrinology. 2007
HuangSA.PediatricEndocrinology; 2007

421
 SECTION THREE
DISORDERS OF THE ADRENAL GLAND AND GONADS
CONGENITAL ADRENAL HYPERPLASIA (CAH)
I. ETIOPATHOGENESIS
• Autosomal recessive disorders of cortisol synthesis leading to increased secretion of ACTH
(corticotropin) causing adrenocortical hyperplasia & overproduction of metabolites
• Deficiency of 21-hydroxylase accounts for 90% of affected patients
0 Lackof 21-hydroxylase causes excess substrates shunted towards synthesis of sex ho1111ones

II. MANIFESTATIONS

TYPES OF
21-HYDROXYLASE MANIFESTATIONS
DEFICIENCY
• 70% of patients with classic 21-hydroxylase deficiency
• Weight loss, anorexia, nausea, vomiting, weakness, salt craving
Salt-losing, classic • Dehydration, hypotension, skin hyperpigmentation
form • Hypoglycemia, hyponatremia, hyperkalemia, metabolic acidosis
• Symptoms appear at 10-14 days of age
• Signs of virilization (see below)
• 30% of patients with classic 21-hydroxylase deficiency
• Signs of virilization:
° Female infants: Ambiguous genitalia/masculinized external
Simple virilizing, genitalia (clitoral enlargement, labial fusion, urogenital sinus)
classic form 0Male infants: appear normal at birth. Postnatal signs of androgen
excess include rapid somatic growth, accelerated skeletal
maturation, stunting, pubic & axillary hair, acne, deep voice,
enlarged penis, scrotum & prostate but prepubertal testes
• Attenuated, late-onset form of adrenal hyperplasia
Non-classic form • May be asymptomatic, or may have precocious adrenarche,
hirsutism, acne, menstrual irregularity, infertility

III. DIAGNOSIS
DIAGNOSTIC FINDINGS/REMARKS

• Most reliable to establish diagnosis of 21-hydroxylase deficiency:

increased serum 17-OHP
Blood Chemistry • Low serum cortisol
• Elevated ACTH and renin
• Aldosterone is inappropriately low for the renin level
Pelvic ultrasound • To visualize presence of uterus in female pseudo-hermaphroditism
Rapid karyotype • To determine genetic sex

IV. MANAGEMENT
• Glucocorticoids inhibit excessive production of androgens & prevent progressive virilization
Hydrocortisone 15-20 mg/m2/day orally in 3 divided doses
0 Double or triple doses during periods of stress (surgery, infection)
Continued indefinitely in all patients with classic 21-hydroxylase deficiency
Monitor growth & hormonal levels
• Fludrocortisone as mineralocorticoid replacement+ NaClsupplementation for salt-losing disease
• Surgery for ambiguous genitals

Source:KliegmanRM.et al. NelsonTextbook of Pediatncs (21sted)Elsevier:2020

LifshitzF.et al. (5thEd.).PediatncEndocnnology.lnformaHealthcare: 2007
Charmandan E, et al. Lancet:2014
NewMl. et al. PediatricEndocrinology:2007
422
ADDISON DISEASE
I. ETIOPATHOGENESIS
Acquired primary adrenal insufficiency
• Etiology:
Most common: autoimmune destruction of adrenal glands, usually sparing the medulla
Infection (most common: meningococcemia causing Waterhouse-Friderichsen
syndrome)
Drugs (ketoconazole, mitotane, etomidate)
Hemorrhage from child abuse/anticoagulant use
• Isolated cortisol deficiency early in the disease
• In advanced disease, all adrenocortical function is lost

II. MANIFESTATIONS
• Muscular weakness, malaise, anorexia, nausea, vomiting, weight loss, orthostatic
hypotension, salt craving
• Increased skin pigmentation more prominent in skin creases, mucosa and scars
• Acute decompensation (adrenal crisis) during relatively minor infectious illnesses

DIAGNOSTIC FINDINGS/REMARKS

• Low serum Na·, increased K·

• Hypoglycemia, ketosis, acidosis
Blood • High ACTH with low or inappropriately normal cortisol & aldosterone
Chemistry • Most definitive test: measurement of serum levels of cortisol before & after
administration of ACTH ( cortisol is low & does not increase normally after
ACTH administration)

Urine • Elevated urinary excretion of Na• & CJ·

Electrolytes • Low urinary excretion of K•

Ill. MANAGEMENT
• D5 0.9 NSS IV:to correct hypoglycemia, hypovolemia, hyponatremia
• Hydrocortisone succinate:
For Acute 0 IVbolus, then divided doses every 6 hours for 1" 24 hours (SOmg/m 2 IV bolus
Adrenal then 25-100 mg/m 2/day as IVinfusion or in divided doses eve1y 6 hours), or
Insufficiency 0 As much as 10 mg for infants, 25 mg for toddlers, 50 mg for older children,
and 100 mg for adolescents administered as bolus, and similar amount in
divided doses every 6 hours for the first 24 hours
• Hydrocortisone 10 mg/m 2 /day in 3 divided doses, or equivalent doses of
prednisone twice daily
-. Fludrocortisone if with aldosterone insufficiency
Chronic
• Monitor ACTHlevels
Replacement
• Prevention of adrenal crisis:
Therapy
0 Increase hydrocortisone dose 2-3 fold during stress (infection/surgery)

0Advise patients to visit emergency room if with persistent vomiting and

unable to tolerate oral hydrocortisone treatment

Source:KliegmanRM.et al. NelsonTextbookof Pediatrics(21sted) Elsevier;2020 1=-,

LifshitzF,et al. (5th Ed.).PediatricEndocrinology.
lnformaHealthcare;2007
TschudyMM,et al. The Harrie!Lanehandbook(19thEd).MosbyElsevier;2012
Charmandari E, et al. Lancet;2014

423
CUSHING SYNDROME
I. ETIOPATHOGENESIS
• Result of abnormally high blood levels of cortisol or other glucocorticoids
• Most common cause: prolonged exogenous administration of glucocorticoid hormones
• In children >6 years old, endogenous Cushing syndrome is most commonly caused by
ACTH-secreting pituitary tumor (known as Cushing disease)
• In children <6 years old, adrenal causes are more common causes

II. MANIFESTATIONS
COMMON MANIFESTATIONS IN OLDER CHILDREN
• Rounded face
• Purplish striae on hips, abdomen & thighs
• Prominent cheeks
• Delayed puberty
• Moon facies
• Emotional !ability
• Buffalo hump
• Weakness
• Generalized obesity
• Headache
• Abnormal masculinization
• Hyperglycemia which may progress to diabetes
• Impaired growth
• Osteoporosis
• Hypertension
• Susceptibility to infection

Ill. DIAGNOSIS
DIAGNOSTIC FINDINGS/REMARKS
• Loss of circadian rhythm of cortisol (normally, serum cortisol levels are
highest at 8 am & decrease to <50% by midnight)
Cortisol Levels • Salivary cortisol screening test is a good outpatient screening test
0 Salivary cortisol levels reflect the unbound, biologically active serum cortisol
• Midnight cortisol levels >4.4 mcg/dL suggests diagnosis of Cushing syndrome

Urinary Cortisol • Increased urinary excretion of free cortisol

• Dexamethasone 25-30 mcg/kg (max: 2 mg) given at 11 pm then determine

plasma cortisol levels at Barn
• Normal response: dexamethasone provides negative feedback to the pituitary
Single-Dose
gland to suppress secretion of ACTH,leading to low cortisol levels
Dexamethasone
• Cushing syndrome: patients have persistently high cortisol levels despite
Suppression
dexamethasone administration
Test
• Interpretation:
• Normal: <5 mcg/dL
• Cushing syndrome: >5 mcg/dL
• To determine whether Cushing syndrome is caused by a/an:
• Pituitary adenoma
0 Ectopic ACTH-secreting tumor
2-Step ° Cortisol-secreting adrenal tumor
Dexamethasone • Dexamethasone low dose (30 mcg/kg/day q6) and high dose (120 mcg/kg/
Suppression day q6) are administered on consecutive days
Test • Responses
0 Pituitary adenoma: high dose dexamethasone suppresses cortisol levels
• Ectopic ACTH-secreting tumor or cortisol-secreting adrenal tumor: cortisol
levels remain elevated despite low & high dose dexamethasone

CT Scan or MRI • To detect adrenal tumors, pituitary adenomas

Bilateral
Petrosal Sinus • To localize small pituitary adenomas not visible on MRI
Sampling
Source:KliegmanRM,et al.NelsonTextbook of Pediatrics
(21sted)Elsevier;
2020
LifshitzF,et al.(5thEd.).PediatricEndocrinology.
lnformaHealthcare:
2007

424
IV. MANAGEMENT
MODALITY REMARKS
Transsphenoidal
• Treatment of choice in Cushing disease
Pituitary Microsurgery

Adrenalectomy
.•• For pituitary adenomaunresponsive to treatment, aldosterone-
producing adenoma and ectopic ACTH-secreting metastatic tumor

Ketonoconazole, • Inhibitors of adrenal steroidogenesis

Metyrapone, • Medical therapy serves as adjunctive therapy after unsuccessful
Aminoglutethimide, pituitary surgery
Etomidate • May be used preoperatively to normalize circulating cortisol
• Adequate preoperative & postoperative replacement therapy
with corticosteroids
Supportive Care • Post-operative catch-up growth, pubertal progress, and
increased bone density occur; however, bone density remains
abnormal with compromised adult height
Source:Kliegman
RM,et al. NelsonTextbook
of Pediatrics
(21sted) Elsevier;
2020
LodishMB.et al. EndocrinolMetabClinNAm:2018

PHEOCHROMOCYTOMA
I. ETIOPATHOGENESIS
• Catecholamine-secreting tumor arising from chromaffin cells
• Most common site of origin: adrenal medulla
• Tumors may develop anywhere along the abdominal sympathetic chain
• Tumors found more often on the right side, but bilateral in >20% affected children
• May be associated with genetic syndromes such as von Hippel-Lindau disease, neurofibromatosis,
MEN2Aand MEN28 syndromes, tuberous sclerosis, Sturge-Weber syndrome, ataxia-telangiectasia

II. MANIFESTATIONS
• Hypertension (more often sustained than paroxysmal in children)
• Headache, palpitations, abdominal pain, dizziness, pal lot; vomiting, sweating, convulsions
• May be symptom-free in-between attacks of hypertension
• Polyuria, polydipsia, growth failure
Severe disease: precordial pain radiating into arms, pulmona1y edema, cardiomegaly, hepatomegaly

III. DIAGNOSIS
DIAGNOSTIC FINDINGS/REMARKS
• Elevatedblood or rnina1y levels of catecholamines (e.g.,dopamine, norepinephrine,
epinephrine) & metabolites (e.g., normetanephrine, metanephrine)
• Predominant catecholamine excreted in urine among children is norepinephrine
Catecholamine
• Best sensitivity & specificity: measurement of plasma normetanephrine
levels
• Urinary excretion ofvanillylmandelic acid (VMA) is increased, but no
longer routinely used due to false-positive results from consumption of

I
vanilla-containing food
• CT,MRI, 123 !-metaiodobenzylguanidine: to localize tumor
Imaging • 85% of tumors are found in adrenal glands, 95% are within the abdomen
and pelvis

IV. MANAGEMENT
• Surgical removal of tumors
• Preoperative a & ~-adrenergic blockade and fluid loading
• Transabdominal exploration of all the usual sites because tumors are often multiple

Source:KliegmanRM,et al. NelsonTextbookof Pediatrics(21sted) Elsevier:2020

TsirlinA. et al. Maturitas:2014
425
GENETIC CAUSES OF HYPERTROPHIC HYPOGONADISM

NOONAN KLINEFELTER
PARAMETER TURNER SYNDROME
SYNDROME SYNDROME

Affectation • Males and females • Males • Females

• Normal • 47 XXY(majority) • 45,X

• 48XXXY
Karyotype
• 48XXYY
• 49 XXXYY
• Short stature, • Diagnosis rarely • Recognizable at birth:
webbing of the made before puberty edema of the dorsa
neck, low hairline, due to paucity of of the hands & feet,
pectus carinatum/ signs and symptoms loose skin folds at the
excavatum, right- in childhood nape, low birth weight,
sided congenital • Behavioral or decreased length
heart disease psychiatric disorders • Short stature (cardinal
(mainly valvular (anxious, immature, finding),webbed neck,low
pulmonic stenosis), or excessively shy), posterior hairline, small
cardiomyopathy, learning disabilities, mandible, high arched
cubitus valgus, verbal cognitive palate, prominent ears,
hypertelorism, defects, deficits in epicanthal folds, broad
downward slanted executive function chest with illusion
palpebral fissures, • Tall, slim, long legs of widely spaced
Clinical
ptosis, micrognathia disproportionate to nipples, cubitus valgus,
• Subnormal IQ, the arms hyperconvex fingernails
high-frequency • Small testes & phallus, • Cardiac defects (mainly
sensorineural cryptorchidism, bicuspid aortic valve),
hearing loss low testosterone, renal malformations,
• Cryptorchidism, delayed puberty, inflammatory bowel
small testes, delayed gynecomastia, disease
puberty sparse facial hair; • Nearly all oocytes are
azoospermia, gone by 2 years (ovaries
infertility described as "streaks")
• Increased incidence of • Breast development
metabolic syndrome, fails to occur, but
breast cancer, adrenarche normally
leukemia, lymphoma present
• Human growth • Replacement therapy • Human growth hormone
Management hormone with testosterone • Estrogen replacement
preparation therapy
Source:KliegmanRM.et al.NelsonTextbook
of Pediatrics
(21sted)Elsevier:
2020
LifshitzF, et al. (5th Ed.). PediatricEndocrinology.
lnformaHealthcare;2007

426
 SECTION FOUR
DIABETES MELLITUS

DIABETES MELLITUS (DM)

I. ETIOPATHOGENESIS
• Chronic metabolic disease characterized by hyperglycemia as a cardinal biochemical feature
• Main types of DM seen in children:

MATURITY-ONSET
TYPE TYPE 1 DM TYPE2 DM DIABETES OF THE
YOUNG (MODY)
• Adult-onset DM
Formerly • Insulin-dependent DM
• Non-insulin-
called • juvenile diabetes
dependent DM
• Insulin resistance
• Defect in insulin
atthe level of
secretion from
skeletal muscle,
• Autoimmune genetic mutations
liver, adipose tissue
Etiology destruction of involving
• Obesity is the most
pancreatic islet cells pancreatic/liver
important lifestyle
glucokinase or
factor associated
trans~ription factors
with T2DM
• Most common
endoc1ine-metabolic • Acanthosis • Autosomal
disorder in children nigricans (dark dominant
• Low or absent levels of pigmentation of inheritance
Characteristics
endogenous insulin skin crease at • Ketoacidosis is
• Dependence on nape) is a sign of rare
exogenous insulin to insulin resistance
prevent ketoacidosis
Family history • infrequent • Strong familyhistory • Strong familyhistory
• Peaks at 5-7 years old, • Incidence • Occurs before 25
Age at onset
puberty increases with age years of age
• Insulin therapy • Metformin: the
• Glucose monitoring only FDA approved
• Nutritional: 55% CHO, oral agent for • Depends on
I' 30% fat, 15% protein T2DM in children subtype
• Limit intake of sucrose • Lifestyle: low- • MODY3
& highly refined sugars calorie, low-fat (most common):
Management • Polyunsaturated: diet, physical sulfonylurea
saturated fat ratio ofl.2:1 activity 30-60 • MODY2(second
• <10% of calories from minutes, at least 5 most common):
1,

1,
saturated fats, up to 10%
from polyunsaturated
fats, the rest from
monounsaturated fats
times/week, and
limit screen time
1-2 hours/day
no treatment or
low dose insulin
I
II. MANIFESTATIONS
• Polydipsia, polyuria, nocturia
• Unexplained weight loss with glucosuria and ketonuria
• Monilial vaginitis among female patients from chronic glycosuria
Hyperphagia
• Weight loss
• Symptoms ofketoacidosis (see DKAsection below)
427
III. DIAGNOSIS
IMPAIRED FASTING
GLUCOSE (IFG)/ IMPAIRED DIABETES MELLITUS
GLUCOSE TOLERANCE (IGT)
• 100-125 mg/dL (5.6-7.0
Fasting Glucose • 2'126 mg/dL (7.0 mmol/L}
mmol/L}
2-hour Plasma • ;,140 mg/dL but <200 mg/dL
• 2'200 mg/dL (2'11.l mmol/L)
Glucose (OGTT) (2'7.8 but <11.1 mmol/L)

Hemoglobin Ale • 5.7-6.4%* • 2'6.5%

• Symptoms of DM + random
Others blood glucose 2'200 mg/dL
(2'11.1 mmol/L)
'Thesevaluesareusedtoclassify
prediabetes
amongadults.Itsutilityindiagnosing
prediabetes
amongchildren
isstillbeingstudied

Source:KliegmanRM.et al. NelsonTextbookof Pediatrics(21sted) Elsevier:2020

American
DiabetesAssociation
Standards of MedicalCarein Diabetes. DiabetesCare;2016

IV. GLYCEMICCONTROL:GLYCOHEMOGLOBIN (HbAlc)

• Reliable index of long-term glycemic control (lower HbAlc means better metabolic control)
• Reflects the average blood glucose concentration of the preceding 2-3 months
• For known diabetics:
06-7.5%: good diabetic control
7.6-9.9%: fair diabetic control
0;,10%: poor diabetic control

V. DAWN PHENOMENON& SOMOGYIPHENOMENON

• Explains elevation of blood glucose levels early morning before breakfast
• Dawn phenomenon: due to overnight growth hormone secretion, increased insulin clearance
• Somogyi phenomenon: theoretical rebound from late-night or early-morning
hypoglycemia ( exaggerated counterregulatory response)
Source:ADAStandardsof MedicalCarein Diabetes.DiabetesCare;2016
CraigME,et al. PediatricDiabetes;2009

DIABETIC KETOACIDOSIS (DKA)

I. ETIOPATHOGENESIS
• DKA is the end result of metabolic abnormalities due to severe deficiency of insulin or
severe insulin ineffectiveness
• Effects of insulin deficiency:
0Glucose utilization by muscle and fat decreases
Excess glucose production via glycogenolysis & gluconeogenesis, leading to hyperglycemia
0Resultant osmotic diuresis produces polyuria, urina,y losses of electrolytes & dehydration
Combination of insulin deficiency & elevated counterregulatory hormones leads to
accelerated lipolysis & impaired lipid synthesis
Increased plasma cholesterol & free fatty acids leads to ketone body formation
(principally acetoacetate & beta-hydroxybutyrate) resulting in metabolic acidosis

II. MANIFESTATIONS
• Abdominal discomfort or pain, nausea, emesis
• Tachypnea, tachycardia
• Fruity breath odor (from acetone)
• Kussmaul respiration (deep, heavy, nonlabored rapid breathing)
• Polyuria, dehydration
• Diminished neurocognitive function, confusion, drowsiness, possible coma
• Progression of symptoms may be accelerated during illness or trauma

428
JII. DIAGNOSIS
A. Biochemical Criteria for Diagnosis of OKA
0 Hyperglycemia (blood glucose> 11 mmol/L or >200 mg/dL)
Venous pH <7.3 or serum bicarbonate <15 mmol/L
Ketonemia (blood beta-hydroxybuyrate 2:3 mmol/L) or moderate/large ketonuria (2:2+)

8. Classification of OKA Based on Severity

MILD MODERATE SEVERE*
• Kussmaul
• Kussmaul or depressed
respiration, fruity
• Oriented, alert, respiration, sleepy to
Clinical breath odo1;
or fatigued depressed sensorium,
oriented but sleepy,
comatose
arousable
Venous pH** • 7.25-7.35 • 7.15-7.25 • <7.15

Serum HC03 ** • 16-20 mEq/L • 10-15 mEq/L • <10 mEq/L

'Severe hypernatremia(corrected Na• >150 meq/L)wouldalso be classifiedas severe OKA
"ISPAD2018 Guidelinesuse differentcut-offsfor classificationof OKA(note that 1 mEq/LHC03 = 1 mmol/L):
• Mild:pH <7.3 or serum HC03 < 15 mmol/L
• Moderate:pH <7.2, serum HC03 < 10 mmol/L
• Severe: pH <7.1, serum HC03 < 5 mmol/L

IV. MANAGEMENT
ASPECT MANAGEMENT
• Resuscitation fluids:
If volume depleted but not in shock: 10 mL/kg IVof0.9% saline over 30-
0

60 minutes (may be given more rapidly if with poor tissue perfusion)

If in shock: 20 mL/kg isotonic saline boluses
0

Fluids • Subsequent fluid management

May use 0.45% NaCl, 0.9% NaCl, or Ringer's lactate (choice offluid
0

depends on hydration status, serum Na, & serum osmolality)

Give maintenance fluid requirement+ replace estimated fluid deficit
0

over 24-48 hours (assume 5-7% deficit in moderate OKA,7-10%

deficit in severe OKA)
• Start insulin infusion (0.05-0.1 units/kg/hr) at least 1 hour after fluid
replacement therapy
Management of
• Addition of glucose to avoid hypoglycemia:
Hyperglycemia
5% solution when blood glucose <300 mg/dL
0

10% solution when blood glucose <200 mg/dL

0

• If patient is hypokalemic, start K' replacement before insulin therapy

Potassium (K•J • lfhyperkalemic, defer K+ replacement until urine output is documented
Replacement • If normal, start K' replacement after initial volume expansion,

Bicarbonate
(HC03 )
Therapy
concurrent with insulin therapy
• Rarely neeeded (may even increase risk ofhypokalemia & cerebral edema)
• Administer (1-2 mmol/kg over 60 minutes) only if with:
Life-threatening hyperkalemia
0
I
Severe acidosis (venous pH <6.9)
0

• Mannitol for cerebral edema

• Secure airway & decompress stomach with continuous nasogastric suction
Supportive
• Oxygen support for patients with circulatory impairment or shock
therapy
• Hook to cardiac monitor to look for evidence of hyper or hypokalemia
• Urinary catheterization, as needed

429
 • Vital signs, neurologic status, fluid input and output, capillary blood
Monitoring glucose hourly or more frequently as needed
• Electrolytes & blood gas every 2-4 hours or more frequently as needed
• Transition to oral intake & subcutaneous insulin upon resolution of OKA:
• HCO3>15 mEq/L
Transition
• pH >7.30
care
• Sodium 135-145 mEq/L
0 No emesis
Source:KliegmanRM.et al. NelsonTextbookof Pediatrics(21sted) Elsevier;2020
WolfsdortJI, et al. ISPAD,PediatrDiabetes;2018

REFERENCES
1. Abraham P, Avenell A, Park CM, Watson WA and Bevan JS. (2005). A systematic review of drug therapy for
Graves' hyperthyroidism. Eur J Endocrinol, 153, 489-498.
2. Ali 0. Hyperpituitarism, Tall Stature, and Overgrowth Syndromes. In Kliegman RM, Stanton BF, St Geme JW,
Schor NF. (Eds.) (2016) Nelson Textbook of Pediatrics (20th ed.). Philadelphia, PA: Elsevier.
3. American Diabetes Association Standards of Medical Care in Diabetes. (2016). Classification and diagnosis
of diabetes. Diabetes Care, 39(Suppl.1), S13-S22.
4. Breault OT and Majzoub JA. Diabetes lnsipidus. In Kliegman RM, Stanton BF, St Geme JW, Schor NF. (Eds.)
(2016) Nelson Textbook of Pediatrics (20th ed.). Philadelphia, PA: Elsevier.
5. Brook CGD,Brown RS.(2008) Handbook of Clinical Pediatric Endocrinology. Massachusetts: Blackwell Publishing.
6. Charmandari E, Nicolaides NC, and Chrousos GP. (2014). Adrenal insufficiency. Lancet, 383, 2152-2164.
7. Craig ME, Hattersley A and Donaghue KC.(2009). Definition, epidemiology and classification of diabetes in
children and adolescents. Pediatric Diabetes, lO(Suppl.12), 3-12.
8. John CA, Day MW. (2012). Central Neurogenic Diabetes lnsipidus, Syndrome of Inappropriate Secretion of
Antidiuretic Hormone, and Cerebral Salt-Wasting Syndrome in Traumatic Brain Injury. Crit Care Nurse, 32(2), el-e7.
9. Grim berg A and Lifshitz F. Worrisome Growth. In Lifshitz F. (Ed.) (2007) Pediatric Endocrinology. New York:
lnforma Healthcare.
10.Parks JS and Felnor El. Hypopituitarism. In Kliegman RM, Stanton BF, St Geme JW, Schor NF. (Eds.) (2019)
Nelson Textbook of Pediatrics (21st ed.). Philadelphia, PA: Elsevier:
11.Kliegman RM, Stanton BF, St Geme JW, Schor NF. (2020) Nelson Textbook of Pediatrics (21st ed.).
Philadelphia, PA: Elsevier.
12.Koeppen, B. M., Stanton, B. A. (2010). Berne & Levy Physiology. Philadelphia, PA: Mosby/Elsevier.
13. Leger J. Graves Disease in Children. In Szinnai G (Ed.) (2014) Paediatric Thyroidology. Endocr Dev. Basel: Karger.
14.Lietman SA, Germain-Lee EL, and Levine MA. (2010). Hypercalcemia in children and adolescents. Curr Opin
Pediatr, 22, 508-515.
15.Lee PA and Houk CP.Puberty and Its Disorders. In Lifshitz F. (Ed.) (2007) Pediatric Endocrinology. New York:
lnforma Healthcare.
16. Lifshitz F. (Ed.) (2007) Pediatric Endocrinology. New York: lnfonna Healthcare.
17.Lodish MB, Keil MF, Stratakis CA. (2018). Cushing's Syndrome in Pediatrics: An Update. Endocrinol Metab
Clin N Am, 47, 451-462.
18.Nowicka P, Santoro N, Liu H, et al. (2011). Utility of hemoglobin ale for diagnosing prediabetes and diabetes
in obese children and adolescents. Diabetes Care, 34(6), 1306-1311.
19.Rogol AD and Hayden GF. (2014). Etiologies and early diagnosis of short stature and growth failure in
children and adolescents.) Pediatr, 164(5), S l-S14.
20.Root AW, Diamond Jr FB. Disorders of mineral homeostasis in children and adolescents. In: Sperling MA.
(Ed.) (2014) Pediatric endocrinology. Philadelphia: Elsevier.
21.Svoren BM and Jospe N. Diabetes Mellitus. In Kliegman RM, Stanton BF,St Geme JW, Schor NF. (Eds.) (2016)
Nelson Textbook of Pediatrics (20th ed.). Philadelphia, PA: Elsevier.
22.Thanabalasingham G, Owen KR. (2011). Diagnosis and management of maturity onset diabetes of the young
(MODY]. BM), 343,838.
23.Tschudy MM, Arcara KM. (Eds.) (2012) The Harriet Lane handbook: a manual for pediatric house officers
(19th ed.). Philadelphia, PA: Mosby Elsevier:
24.Tsirlin A, Oo Y, Sharma R, Kansara A, Gliwa A, Banerji A. (2014). Pheochromocytoma: A review. Maturitas,
77, 229-238.
25.Vijayakumar P, Nelson RG, Hanson RL, et al. (2017). HbAlc and the prediction of type 2 diabetes in children
and adults. Diabetes Care, 40, 16-21.
26. White PC. Congenital Adrenal Hyperplasia and Related Disorders. In Kliegman RM, Stanton BF, St Geme )W,
Schor NF. (Eds.) (2016) Nelson Textbook of Pediatrics (20th ed). Philadelphia, PA: Elsevier:
27.Wolfsdorf JI, Glaser N, Agus M, Fritsch M, Hanas R, Rewers A, et al. (2018). Diabetic Ketoacidosis and
Hyperglycemic Hyperosmolar State: A Consensus Statement from the International Society for Pediatric and
Adolescent Diabetes. Pediatr Diabetes, doi: 10.1111/pedi.12701.

430
NEUROLOGY
 SECTION ONE
APPROACH "FOPATIENTS WITH NEUROLOGIC COMPLAINTS
NEUROLOGIC EXAMINATION
I.HEAD
• Head circumference
0Serial monitoring to detect hydrocephalus or microcephaly
See Chapter 2 for measurement and interpretation of head circumference
0

• Fontanelles
Anterior fontanelle -- diamond-shaped, around 2x2 cm, and closes at 9-18 months
0Posterior fontanelle -- admits tip of finger and closes at 6-8 weeks (may be closed
at birth)

II. GLASGOWCOMASCALE

FUNCTION INFANTS & YOUNG CHILDREN OLDER SCORE

CHILDREN
Spontaneous Spontaneous 4
To voice To voice 3
Eye Opening
To pain To pain 2
None None 1
Appropriate words; smiles, fixes, follows Oriented 5
Consolable crying Confused 4
Verbalization Persistently irritable Inappropriate 3
Restless, agitated Incomprehensible 2
None None 1
Obeys Obeys 6
Localizes pain Localizes pain 5
Withdraws Withdraws 4
Motor
Reflex flexion Reflex flex ion 3
Reflex extension Reflex extension 2
None None 1
Total Score 15

Sample Case
A 13 year-old boy was brought to the Emergency Room after sustaining injuries from a
bicycle accident. On assessment, he has eye opening when his name is called, responds
confusedly to questions, and localizes to pain. What is his GCS score?
• To compute for his GCSscore, identify the score for each of the 3 components
Eye opening (to voice)= 3 points
0

Verbalization (confused)= 4 points

0

0 Motor (localizes to pain)= 5 points

• Add the score for each of the 3 components to get the GCSscore
• Total GCSscore= 3+4+5 = 12 points
• The GCSof this boy is E3V4M5, for a total GCSscore of 12 points

III. CRANIAL NERVEEXAMINATION

Olfactory • Smell • Can be tested as early as 32 weeks AOG

I
• Visual acuity:
• Vision
0 Term 20/150
• Pupillary light
II Optic 0Adult level 20/20 by 5-6 yrs
reaction
0 E-game to test VA
• Fundoscopy 02.5-3 years: may use Pediatric Allen Chart
433
 • EOMs except superior
Ill Oculomotor oblique and lateral rectus • Check for nystagmus
muscles
• Superior oblique:
IV Trochlear • Check for nystagmus
depresses and intorts eyes
• Paralysis: medial deviation of eye
with inability to abduct beyond
VI Abducens • Lateral rectus
midline
• Check for nystagmus
• Sensation: check
• Difficult to assess in young children
ophthalmic, maxillary, &
• Nasal tickle test:
mandibular branches
cotton swab in nose
V Trigeminal • Motor: masseter, pterygoid,
• Observe symmetry of grimace
temporalis
• Observe tone while patient
• Sensory component of the
is sucking/ during mastication
corneal reflex
• Lower motor neuron or facial
nerve lesions: half of face is
affected
• Facial symmetry • Upper face receives bilateral
• Motor component of the cortical input
VII Facial corneal reflex • Lesions of motor cortex or
• Taste anterior 2/3 of corticobulbar tract have little effect
tongue on upper face strength (thus lesions
in this area usually present with
flattening of nasolabial fold and
drooping of the corner of mouth)
• Vestibular dysfunction presents
with nystagmus: fast component
directed away from affected nerve
• Vestibular: equilibrium,
• Romberg and tandem gait: fall
coordination, orientation
VIII Vestibulo-
cochlear toward abnormal ear
in space
• Caloric testing: 30-50 mL ice water
• Cochlear: hearing
with head elevated 30°
• If brainstem intact: eyes deviate
towards irrigated side
• Taste posterior third of
Glosso- • Isolated deficit is rare because it is
IX pharyngeal tongue
in close proximity to CN X
• Gag reflex
• Unilateral injury: weakness of
• Muscle of larynx
ipsilateral soft palate and hoarse
and pharynx except
X Vagus voice
stylopharyngeus (IX) and
• Bilateral: respiratory distress due
tensor veli palatine (V)
to vocal cord paralysis
• Shoulder shrug
• Sternocleidomastoid and • In younger children may observe
XI Accessory
trapezius turning of head from side to side
against resistance
• Tongue deviates toward side of injury
• Tongue: atrophy, weakness,
XII Hypoglossal • Bilateral: unable to protrude
fasciculations
tongue, dysphagia

Source:KliegmanR.el al. NelsonTextbook of Pediatncs(21sted.).Canada:Elsevier;2010

MenkesJ, et al. ChildNeurology(7thed.).Lippincott
Williams& Wilkins:2006

434
IV. MOTOREXAMINATION
A. General
Check for atrophy (decreased muscle bulk) or hypertrophy (increased muscle bulk)
° Check for abnormal movements such as fasciculations, tics, dystonia, chorea, athetosis

B. Muscle Tone
28 weeks: all 4 extremities are extended
0 32 weeks: flexor tone in lower extremities
36 weeks: flexor tone in upper extremities
Normal term: flexion of all 4 extremities
Neonates: postural tone
MUSCLE TONE REMARKS
• Examiner grasps infant's hands & pulls infant to sitting position
Traction
• Normal: head lags slightly behind body and then falls
Response
forward upon reaching sitting position
Postural • Examinerholds inrant by axillawithout grtppingthe thorax
Vertical
Tone in • Normal: infant remains suspended, lower extremity held in flexion
Suspension
Neonates • Hypotonic infant slips through the examiner's hands
• Examiner holds infant prone by placing hand under abdomen
Horizontal
• Normal: head rises, limbs flex
Suspension
• Hypotonic infant drapes over the hands and forms a U shape
• Initial resistance to passive motion followed by sudden release (clasp-knife
Spasticity phenomenon)
• Upper extremity flexors & lower extremity extensors usually affected
• Basal ganglia lesions
Rigidity
• Resistance to passive movement equal in flexor & extensors (lead pipe)

C. Muscle Strength
SCORE DESCRIPTION

0 • No contraction

1 • Flicker or trace of contraction

2 • Active movement with gravity eliminated

3 • Active movement against gravity

4 • Active movement against resistance

5 • Normal power

V. SENSORYEXAMINATION
• Often difficult to perform in young children
• Isolated disorders of sensory system are less common in children

VI. REFLEXES
A. Deep Tendon Reflexes
Infants younger than 3 months can have 5-10 clonus
Older children can have 1-2 beats of clonus
1 + to 3+ reflex may be normal as long as symmetrical
Babinski: extension of great toe, fanning out of remaining toes

B. Primitive Reflexes (See Neonatology Chapter for more details)

I
Reflex actions originating in the CNS exhibited by normal infants but not
neurologically intact adults
Disappearance indicates maturation of the cerebral hemispheres
Persistence beyond expected date suggests maturational lag or impaired CNSwhich
may be seen in cerebral palsy and other neurodegenerative disorders
435
VII. GAIT ABNORMALITIES
ABNORMALITY DESCRIPTION

• Stiff-legged like a soldier

Spastic gait
• May tiptoe due to contractures of Achilles tendon
• Spasticity and circumduction of leg
Hemiparetic gait
• Decreased arm swing on affected side

Cerebellar ataxia • Wide-based, reeling gait like a drunk person

• Wide-based, steppage gait (lift up legs higher than usual in swing

Sensory ataxia
phase and then slaps foot down)
• Waddling gait
Myopathic gait
• Hip girdle weakness

VIII. UPPER VERSUS LOWER MOTOR NEURON LESIONS (usual findings)

UPPER MOTOR LOWER MOTOR
ABNORMALITY
NEURON LESIONS NEURON LESIONS

Weakness + +
Atrophy - +
Fasciculation - +
Reflex Increased Decreased

Tone Increased Decreased

Source:KltegmanR, et al. NelsonTextbookof Ped,atncs
(21sted.).Elsevier;2019
Mohammed et al. Neurosciences;
2001
SPECIAL DIAGNOSTIC TESTS
I. OVERVIEW OF DIAGNOSTIC TESTS IN NEUROLOGY
TEST DESCRIPTION

Skull roentgenograms • Limited utility

• Method of choice for hemorrhage, periventricular

Cranial
leukomalacia, and hydrocephalus
ultrasonography
• Infant should have patent anterior fontanels

• Used for a variety ofneurologic emergencies (e.g.

hemorrhage, infarcts, signs of meningitis)
CT scan
• Younger children are more sensitive to radiation (weigh risk
and benefit carefully)
• For visualizing vascular structures
CT angiography • Preferred in older children because the procedure is faster, it
eliminates the need for sedation

• Detects variety of abnormalities including posterior fossa and

MRI spinal cord pathologies
• Highly susceptible to motion a1tifact (most will require sedation)

MR angiography and • Choice for stroke, vasrularmalformations, cerebral venous thrombosis

venography • Preferred in infants and young children to avoid radiation exposure

• Maps neuronal activity

Functional MRI
• Useful for pre-surgical localization of critical brain functions

• Provides recording of electrical activity between electrodes

Electroencephalography
placed on the scalp
(EEG)
• Used to detect epileptiform discharges and slowing
436
II. LUMBAR PUNCTURE (refer to "Proced11res Chapter" 011 how to petform LP)
A. Opening Pressure Normal Values
AGE NORMAL OPENING PRESSURE

Newborn 90-120 mmH 2 0

~

Young children 60-180 mmH,O

Older children and adults 12-120 mmH 2 0

BC . CSF A
PRESSURE PROTEIN GLUCOSE LEUKOCYTES
(mmH2O) (mg/dL) (mg/dL) (mm')
1} Normal CSF(beyond the neonatal period)
• >50 mg/dL, or
• <5 mm3
• 50-80 mmH 2 0 • 20-45 mg/dL >75% of serum
• 75% lymphocytes
glucose
2) Bacterial Meningitis
• <40 mg/dL, or • Elevated (100 to
• Elevated (100-
• ;,100 mg/dL • <50% of >10,000 mm 3)
300 mmH 2 0)
serum glucose • PMN predominance

3) Partially Treated Bacterial Meningitis

• 5-10,000 mm 3
• PMN predominance
• Normal or • Normal or • Mononuclear cells
• 100-500 mg/dL
elevated decreased may predominate
(if treated for
extended period)

4) Viral Meningitis
• Rarely > 1,000 mm 3
• Normal to slightly • PMN predominance
• Commonly
elevated • 50-200 mg/dL early on, then
normal
(80-150 mmH 2 0) mononuclear through
most of the course

SJ Tuberculous Meningitis
• 10-500 mm 3
• Commonly • PMN predominance
• Commonly • Very high
decreased early on, then
elevated • 100-3,000 mg/dL
(<50 mg/dL) lymphocytes through
most of the course
Source:KliegmanR.et al. NelsonTextbookof Pediatrics(21sted). Elsevier:
2020
MenkesJ. et at ChildNeurology(7thed.).LippincottWilliams& Wilkins;2006

I
437
 SECTION TWO
CONGENITAL ANOMALIES OF THE CNS

SPINAL CORD MALFORMATIONS

I. ETIOPATHOGENESIS & MANIFESTATIONS
• Due to failure of the neural tube to close spontaneously between the 3'' & 4'h week of in
utero development
• Hyperthermia, drugs, malnutrition, chemicals, maternal obesity or diabetes, and genetic
determinants adversely affect CNS development from the time of conception
• 75% are lumbosacral (but may be located anywhere along the neuroaxis)

TYPE FIGURE PATHOGENESIS & MANIFESTATIONS

• Midline defect of the vertebral bodies without protru-

sion of the spinal cord or meninges
• Most are asymptomatic
Spina Bifida • Rule out occult spinal dysraphism which usually presents
Occulta with cutaneous skin manifestation (e.g., hemangiomas,
discoloration, pit, lump, hairy patch, dermal sinus)
• Associated with more significant anomalies (e.g., syrin-
gomyelia, diastematomyelia, lipoma, tethered cord)

• Meninges herniate through a defect in the posterior

vertebral arches or anterior sacrum
• Spinal cord is usually normal
• Fluctuant midline mass that might transilluminate
Meningoceie
usually in the lower back
• Most are well covered with skin
• Look out for urologic manifestations such as bladder/
bowel incontinence or dysfunction

• Most severe form of dysraphism

• Meninges, along with spinal cord elements, herniate
through the defect
• Affects many structures (e.g., skeleton, skin, GIT,GUT)
• In newborns: sac-like cystic structure covered by a thin
Myelomeningocele
layer of skin or partially epithelized tissue
• Mayhave paralysis of the lower extremities, areflexia, sen-
so,y abnormalities, & bladder & bowel abnormalities
• Chiari II malformation presents with hydrocephalus and
myelomeningocele (80% of the time)

Source:KliegrnanR, et al. NelsonTextbookof Pediatrics(21sted).Elsevier:2020

II. DIAGNOSIS
A. Diagnostics Used:

DIAGNOSTIC REMARKS

Radiographs • Assess the vertebral bodies and bony structures

Ultrasonography • To assess soft tissue structures such as the spinal cord
• Diagnostic test of choice for all ages
MRI
• Expensive and requires sedation

• Cystometrogram to identify neurogenic bladder

Others
• Cranial CT scan to rule out hydrocephalus
438
 B I d' t' . .
Imagingis indicatedif any of the Indicationis uncertainif Imaging is not
followingare present: the followingare present: required if:
• Subcutaneous mass or lipoma
• Hairy patch
• Dermal sinus • Simple dimples
• Atypical dimples (deep> 5 mm, ( <5 mm, <25 mm
• Hyperpigmented patches
> 25 mm from anal verge)
• Vascular lesions like hemangioma • Deviation of gluteal fold from anal verge)
• Skin appendages or polypoid • Coccygeal pits
lesions like skin tags
• Scar-like lesions

Ill. MANAGEMENT
• Prenatal screening of maternal serum for AFP in the 16th-18th week of gestation:
identifies pregnancies at risk for fetuses with neural tube defects (NTD) in utero
Prevention
• Maternal periconceptual use of folic acid (0.4 mg OD) reduces its incidence
{stmtedbeforeconceptionuntilat leastdie 12" weekofgestationwhenneu111lation
iscomplete)
• Immediate surgery is indicated for CSFleaks to prevent complications:
Surgery • Repair of myelomeningocele
• Shunting procedure for hydrocephalus
• With aggressive treatment, there is a 10-15% mortality rate
Prognosis
• 70% of survivors have normal intelligence
Sources:KliegmanR.et al. NelsonTextbook of Pediatrics
(21sted).Elsevier;2020
MenkesJ, et al. ChildNeurology(7thed.).LippincottWilliams& Wilkins;2006
FenichelG. et al. ClinicalPediatricNeurologyA signsandsymptoms approach(6thed).Elsevier;2009
WilliamsH, et al.Archivesof Diseasein Childhood• Education andPractice;2009

HYDROCEPHALUS
I. ETIOPATHOGENESIS
A. Physiology
CSF is produced in the choroid plexus epithelium within the cerebral ventricles
CSF is re-absorbed in the arachnoid villus cells (located in the superior sagittal
sinus) and returned to the bloodstream within vacuoles (via pinocytosis)

Left lateral ventricle

Right lateral ventricle

3rd ventricle

Foramen of Magendie
I
Central canal of the spinal cord

Flow ofCSF {Mnemonic:"ComeLet Me 71-ecrt Sisa For Lunch Maybe Somewhere in Ayala']: produced in the
Choroidplexus-> Lateral Ventricle•>fora men of Monroe ->Third Ventricle·>aqueduct of Sylvius -> Fourth
ventricle->Luschkaand Magendie->Subarachnoidspace where it is absorbed in the arachnoidgranulations
439
 B. Types of Hydrocephalus and Pathophysiology
COMMUNICATING OR NONCOMMUNICATING
NONOBSTRUCTIVE OR OBSTRUCTIVE
HYDROCEPHALUS HYDROCEPHALUS
• Obliteration of subarachnoid
• Obstruction within the ventricular
Pathophysiology cisterns and/or malfunction
system
of arachnoid villi
• Achondroplasia • Aqueductal stenosis
• Basilar impression • Mitochondrial
• Benign enlargement of sub- • Autosomal recessive or dominant
arachnoid space • LlCAMmutations
Etiologies
• Choroid plexus papilloma • Chiari malformation
• Meningeal malignancy • Dandy-Walkermalformation'
• Meningitis • Klippel-Feilsyndrome"
• Posthemorrhagic • Mass lesionsc
a. Dandy-Walker malformation: triadof 1)agenesisof cerebellarvermis;2) cysticdilatationof the 4thventricle;
and 3) enlargementof the posteriorIossa
b. Klippel-Feil
syndrome:triadof 1) shortwebbedneck;2) decreasedrangeof motionofthe cervicalvertebrae;
and 3) lowposteriorhairlineaccompaniedby hydrocephalus
c. Masslesionsincludeabscess,hematoma,tumorsand neurocutaneous disorders,Veinof Galenmalformation,
Walker-Warburg syndrome(abnormalglycosylation of a-dystroglycan
causingcerebra-ocular dysgenesis)
Sources:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted).Elsevier;2020
FenichelG. et al. ClinicalPediatricNeurology
A signsand symptomsapproach(6thed).Elsevier:2009

II. MANIFESTATIONS
• Infants: accelerated rate of head enlargement
Symptoms • Wide and bulging anterior fontanel
• Older children: headache is a prominent symptom
• Eyes may deviate downward due to impingement of the dilated suprapineal
recess on the tectum (setting-sun sign)
• Long-tract signs (brisk DTRs, spasticity, clonus, Babinski sign)
Signs • Percussion of skull produce a "cracked pot" sensation or Macewen sign
(separation of sutures)
• Foreshortened occiput may be seen in Chiari malformation
• Prominent occiput may be seen in Dandy-Walker malformation

III. DIAGNOSIS
DIAGNOSTIC REMARKS

Plain skull films • May show separation of sutures

CT scan, MRI, and • Most important studies to identify the abnormality that causes
ultrasonogfaphy hydrocephalus

IV. MANAGEMENT
• Depends on the etiology
• Acetazolamide & furosemide: provide temporary relief by reducing rate of CSFproduction
• Most cases will require extra-cranial shunts
• Most common complication of shunts: Staphylococcus epidermidis infection

Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted).Elsevier:2020

440
 SECTION THREE
MIGRAINE AND HEADACHE

APPROACH TO HEADACHE IN CHILDREN

I. COMMON CAUSES OF HEADACHE
TYPE CAUSES
• Tension type headache
Primary • Migraine headache
Headache • Trigeminal autonomic cephalagias
• Other primary headache disorders
• Trauma or Injury
• Cranial or cervical vascular disorder
• Nonvascular intracranial disorder
Secondary
Headache • Substance abuse or withdrawal
• Infection
[with an • Disorders of homeostasis
underlying cause)
• Facial or cervical structures (e.g., cranium, neck, eyes, ears, nose, mouth)
• Psychiatric disorders
• Neuropathies and other facial pains

II. MIGRAINE HEADACHE VS TENSION TYPE HEADACHE

TENSION TYPE HEADACHE
MIGRAINE HEADACHE
(TTH)
Severity • Mild to moderate • Moderate to severe
• Focal
• Usually unilateral but in children
Location • Diffuse
may be bilateral ( commonly
bifrontal)
Character • Non-throbbing • Throbbing or pounding

• Not frequentlyassociated with aura • May or may not be present

• Symptoms like nausea, • Typical auras: visual/sensory/
Aura photophobia, phonophobia or dysphasic
vomiting never occur together • Atypical auras: hemiplegic/ Alice
at the same time in wonderland syndrome

Nausea/vomiting • Uncommon • Frequent

Effect of activity • Not affected • Usually exacerbated

III. INDICATIONS FOR NEUROIMAGING (imaging of choice is a cranial MRI)

Abnormal neurologic examination
Abnormal or focal neurologic signs of symptoms
Seizures or very brief auras ( <S mins)
Unusual headaches in children:
Atypical auras: basilar-type, hemiplegic
l·-
0 Trigeminal autonomic cephalagia including cluster headaches
0 Acute secondary headache
Brief cough headache
Headache is worse upon awakening or awakens child from sleep
Migrainous headache in a child with no family history of migraine or its equivalent
Sources:KliegmanR,et al. NelsonTextbookof Pediatrics(21sted).Elsevier:2020
MenkesJ, et al. ChildNeurology
(7thed).LippincottWilliams& Wilkins:2006
DooleyJ, et al. Paediatrics
& ChildHealth;2009
441
IV. RED FLAGS OF HEADACHE IN CHILDREN (SNOOPY acronvm)

s • Systemic signs or symptoms: fever; weight loss, rash, meningeal signs,

neurocutaneous lesions, malignancy
• Neurologic signs or symptoms: hemiparesis, papilledema, hemi-sensory loss, diplopia,
N
visual changes, dysarthria, seizures, ataxia, cognitive change, history of head injury
• Onset: sudden, "worst headache of life" (thunderclap), explosive onset; early
0
morning pattern or awakening with headache
0 • Occipital: location of the headache

p • PS: progressions of existing headache, previous headache history, precipitated by Valsalva,

postural, pregnancy (change in quality, frequency, or location, steadily worsening pattern)
y • Younger: age group of patients

MIGRAINE

I. ETIOPATHOGENESIS
• More common among females during adolescence
• More common in males among those younger than 10 years old
• >50% undergo spontaneous prolonged remission after the 10 11' birthday
• No one theory that can explain the exact pathogenesis of migraine
II. MANIFESTATIONS
• Episodic attacks moderate to severe in intensity, focal location, throbbing quality
• May be associated with nausea, vomiting, light sensitivity, sound sensitivity

TYPE DESCRIPTION

• Most prevalent type

• Throbbing or pounding
• Bifrontal or temporal areas
Migraine without aura
• Intense nausea and vomiting
• Usually with a family history in 90% (maternal side)
• Light-headedness, photophobia, osmophobia, phonophobia
Typical versus • Typical: visual/sensory /dysphasic
Atypical • Atypical: hemiplegic/Alice in wonderland
• Recurrent, severe vomiting (Sx/hour) in infants that may
persist for 1-5 days
Cyclic vomiting
• After deep sleep, child resumes normal play
• Complete resolution of symptoms between attacks
• Mid-abdominal pain with pain-free pe1iods between attacks
• At the time of abdominal pain, at least 2 of the following:
Migraine Abdominal Anorexia
0

with migraine Nausea

0

aura Vomiting
0

Pallor
0

Pain may persist from 1-72 hours

0

Hemiplegic aura • Unilateral senso1y or motor signs that may persist for days
-·- -·
(migraine aura) • Good prognosis in an older child or adolescent
• Vasoconstriction of basilar and posterior cerebral arteries
• Vertigo, tinnitus, diplopia, scotoma, ataxia
Basilar-type
migraine • Altered consciousness with seizures
• Complete resolution after the attack
• Girls <4 years old at risk
Source:Headache
Classification
Committee
on lhe International
Headache
Society(HIS);2013

442
IV. MANAGEMENT
A. Three Components in the Management of Migraine
Acute treatment should be developed for stopping a headache attack on a consistent
basis with return to function within a maximum of 2 hours
Preventive treatment when headaches are frequent (;,,1 per week) and disabling
• Biobehavioral therapy
B. Acute Treatment
• NSAID for mild to moderate, restrict attack not more than 2-3 per week
• Add triptans if moderate-to-severe or NSAID fails
Restrict to not more than 4-6 attacks/month
• NSAIDS can be repeated q3-4h1; triptans q2hr
• Fluid hydration: important because vascular dilation is common feature of migraine
• Status migrainosus: persistent headache >3 days (refer to a specialist)
V. PHARMACOLOGIC OPTIONS FOR MIGRAINE
CLASS REMARKS REPRESENTATIVE DRUGS
• Paracetamol 15 mg/kg/dose q4-q6
• NSAIDSnot more than 2-3 times (max dose 90mg/kg/24h)
NSAIDs per week to avoid medication over- • Ibuprofen 7.5 mg/kg/dose q6-q8 (max
use headache dose 40mg/kg/24h)
• Aspirin as alternative for >15 years old
• For migraines uncontrolled by NSAIDs
• Side effects: tightness of jaw, chest,
• Almotriptan
Triptans fingers due to vascular constriction,
feeling of grogginess, and fatigue • Rizatriptan
due to central serotonin effect
• Due to antagonism of dopaminer-
gic neurotransmitter • Procholorperazine
Antiemetics
• Used if severe and unresponsive to • Metoclopramide
NSAIDand Triptans

VI. PREVENTIVE THERAPY

A. Indications and Goals for Preventive Therapy

INDICATIONS FOR PREVENTIVE THERAPY GOALS OF PREVENTIVE THERAPY

• Frequent more than lx / week

• > 1 disabling headache a month
• To reduce frequency of migraine (1-2
• Missing school, home or social activities
or fewer per month)
• Contraindications to or overuse of acute therapy
• To reduce disability PedMIDAS<l0
• Uncommon conditions: hemiplegic migraine,
(see Ped MIDASscoring below)
basilar migraine, prolonged aura
• PedMIDAS score >20
Pediatric Migraine Disability Assessment (PedMIDAS) is a self-administered questionnaire
to assess migraine disability in pediatric & adolescent patients

B O t" p t" Tl
' '
DRUGS REMARKS

• Only flunarizine demonstrates a level of effectiveness

Flunarizine

Amitriptyline
• Dose: 5 mg OD, increased after 10 months to 10 mg OD, with a month
off the drug every 4-6 months

• Dose: lmg/kg daily

• Side effect: sleepiness, anticholinergic activity
I
• Dose in adults and adolescents: 50 mg BID
Topiramate
• Dose needs to be reached slowly
Others • Valproic acid, beta blockers
Source:KliegmanR,el al. NelsonTextbook
of Pediatrics(21sted).Elsevier,2020
443
 SECTION FOUR
SEIZURES & EPILEPSY
APPROACH TO SEIZURES
I. APPROACH TO A CHILD WITH SUSPECTED SEIZURE
Did the child have seizure?

Yes

t
Initial Seizure Recurrent Seizure

···---····················--·-·----
RBS,Ca1·, Metabolic : Consider and check the
Studies depending on ] following:
history and OE Drug compliance and
dosing
EEG? Metabolic disorder
Imaging? Structural lesion
CSF Examination 7 Drug Interaction
CNS degenerative

Studies and disease

Examination lntracta ble seizures

Follow up

.......... ,.....
Abnormal
Symptomatic seizures
Treat underlying cause
Anti-epileptic drug as
needed

I
---------
........................

...
111
No continuous

I
!'. ..
Normal
Isolated l5' seizure with normal EEG,

• drug treatment
nofamdyh1story
• Close observation

Follow up
i
=·_]
\
.
Normal except EEG
Consider drug therapy

I
Poor control
Regular follow up Consider hospitalization
Monitor drug levels and toxicity Re-adjustment of medication
.. E.E:(3.a~_!ri<;lic~jecJ Re-consider pathology
...Freguentf~llo.,v."P. ..
Source:KliegmanR.et al. NelsonTextbookof Pediatrics(21sted). Elsevier:2020
MenkesJ. et al. Child Neurology(7th ed). LippincottWilliams& Wilkins;2006

II FOCUSED HISTORY & PHYSICAL EXAMINATION

Onset of • First seizure episode? Was the patient febrile?
seizure • What was the patient doing prior to the seizure? (e.g., asleep, playing)

• Generalized seizures: commonly present with upward gaze, generalized

Description
tonic-clonic movement of extremities
of seizure:
• Focal seizures: present with leftward or rightward gaze, focal seizure
generalized or
activity in one or more extremities, turning of head to one side, etc.
focal? • Focal with secondary generalization

• Some strategies to determine the duration are as follows:

0 In relation to duration of songs/commercials/tv episode:
Duration of , 1 TV commercial approximately 1 minute
seizure , "Lupang Hinirang" approximately 3 minutes
, 1 TV episode approximately 30 minutes
, Travel time going to the hospital

Post ictal state • Drowsy? Awake? With incontinence?

444
III. INITIAL MANAGEMENT
• Secure Airway, Breathing, and Circulation
ABCs • Position patient to avoid aspiration
• Provide 100% oxygen while ongoing seizure episode
• Diazepam IV 0.3 mg/kg/dose (max 5 mg for <S years, 10 mg >S years)
Initial • Rectal Diazepam 0.5 mg/kg/dose
anticonvulsant • Buccal Midazolam 0.3 mg/kg/dose
• Midazolam IV 0.2 mg/kg/dose
• Hypoglycemia
Correct • Electrolyte imbalance
underlying • Pyridoxine for lsoniazid toxicity
causes if any • May give trial 100 mg of Vitamin B6 (pyridoxine) for pyridoxine
responsive seizures

IV. INVESTIGATE FOR UNDERLYING CAUSE OF SEIZURE

• Refer to a pediatric neurologist
• Laboratory work up depending on history, PE, and considered etiology:
CBC
0 Urinalysis (to rule out infection)
° Chest radiograph (to rule out infection)
0 Blood glucose
Electrolytes: Na, K, Ca, Mg
Metabolic screening, ABG, CSF analysis, imaging (e.g., ultrasound, MRT,CT), and
toxicology screening if warranted
• Differentials: metabolic causes, febrile convulsions, CNS infection, epilepsy syndromes,
increased ICP,toxins, and encephalopathies
of Pediatrics(21sted).Elsevier:2020
Sources:KliegmanR. el al. NelsonTextbook
MenkesJ. el al. ChildNeurology(7thed).LippincottWilliams& Wilkins:2006

SEIZURES AND EPILEPSY

I. DEFINITION OF TERMS
TERM DEFINITION

• Paroxysmal, time-limited changes in motor activity and/or behavior

Seizures/ that result from abnormal electrical activity in the brain
convulsions • Transient occurrence of signs/symptoms resulting from abnormal
excessive or synchronous neuronal activity in the brain
• A general term to include any one of the following:
Seizure 0 Epilepsy

disorder ° Febrile seizures

Seizures secondary to metabolic, infectious, or other etiologies
0

• Brain disorder characterized by an enduring predisposition to generate

seizures and by the neurobiologic, cognitive, psychological, and social
Epilepsy
consequences
• ~2 unprovoked seizures occurring in a time frame of>24 hours
Epileptic • Disorder that manifests one or more specific seizure types and has a
syndrome specific age of onset and prognosis
Status
epilepticus

Breakthrough
• A single seizure lasting> 15 mins or a series of seizures without return
to baseline mental status between each episode
• Occurs in patients already being treated with anti epileptic drug but has
I
seizure achieved low drug levels

II. SEIZURE TYPE AND EPILEPSY SYNDROME

• Seizure type: primary determinant of type of medications patient is likely to respond to
• Epilepsy syndrome: determines the type of prognosis one could expect

445
III. PATHOPHYSIOLOGY OF EPILEPSY
FOUR DISTINCT
MECHANISMS DESCRIPTION

• Any pathology or pathologic process that disrupts neural function

Underlying
etiology
and connectivity
• This often leads to epileptogenesis
• Mechanism: identify which part of the brain turns epileptic
Epileptogenesis • Kindling: repeated electrical stimulation of selected areas initially causes
no apparent changes but with repeated stimulation causes epilepsy
• Seen in all patients regardless of etiology
Epileptic state • In a seizure focus, neurons develop a stereotypic synchronized
of increased response called paroxysmal depolarization shift
excitability • When after-hyperpolarization is disrupted, the inhibit01y surrounding
is lost & neurons fire at the same rate & time resulting in a seizure focus
• Neuronal injury demonstrated in MRI
Seizure related
• Injuries may be transient or permanent
neuronal injury
• Injuries may include apoptosis and necrosis of neurons

IV. SEIZURE NOMENCLATURE

A. Epileptic Seizures is Divided Into Two Broad Categories:
NOMENCLATURE DEFINITION

• EEG changes suggest initial activation is limited to part of 1

Focal Seizures cerebral hemisphere
• Previously labeled as partial seizures
• EEG changes indicate synchronous involvement of all of both
Generalized Seizures
hemispheres

B. Other Nomenclatures for Seizure Disorders

NOMENCLATURE DEFINITION

Acute symptomatic • Occurs secondary to an acute problem affecting brain

seizure excitability such as electrolyte imbalance, trauma, tumors, etc.
• One that is not an acute symptomatic seizure (e.g., no
Unprovoked seizure
identified etiology)
Remote symptomatic
seizure
• Secondary to a distant brain injury such as an old stroke

Reflex seizure • Precipitated by sensory stimulus such as flashing lights

C. Other Nomenclatures for Epilepsy Disorders

PREVIOUS
NOMENCLATURE DEFINITION
TERMINOLOGY

• Genetic or presumed genetic

Genetic Epilepsy • No underlying disorder affecting Idiopathic Epilepsy
development or other neurologicfunction
• Caused by an underlying brain disorder
Structural/ Symptomatic
that may or may not be genetic
Metabolic Epilepsy Epilepsy
(e.g.,stroke, tuberous sclerosis)
• There is a presumed underlying Cryptogenic
Unknown Epilepsy
disorder but not determined Epilepsy
Sources:BergA.et al. Revisedterminology
andconceptsforseizuresandepilepsies.Epilepsia;2010
Source:Kliegman.et al. NelsonTextbook
of Pediatrics.
21sted. Elsevier.2020
446
V. SELECTED COMMONEPILEPSY SYNDROMES
SYNDROME DESCRIPTION

Benign Rolandic • Usually begins between 3-13 years old; remits by 16 years old
Epilepsy • Single nocturnal seizure with clonic movement of the mouth and gurgling
(benignchildhood • Seizures resolve by 16 years
epilepsywith • Altered consciousness, postictal confusion, and aura are rare
centrotemporalspikes) • Oxcarbazepine, carbamazepine, levetiracetam, valproic acid
• Accounts for 70% of partial seizures
• Many have a prior history of febrile seizures or head trauma
Temporal Lobe
• Prodrome: lethargy
Epilepsy
• Oral or motor automatisms, altered consciousness, head & eye deviation,
contralateral twitching or tonic-clonic movements, posturing
• Sudden cessation of motor activity or speech with a blank facial
expression & flickering of the eyelids
• Uncommon before 5 years old
Absence seizures
• Never associated with an aura and rarely persist >30 seconds
• Not associated with a postictal state
• Patients do not lose body tone & usually resume activity after seizure
• Most common generalized motor seizures
• Can be primarily or secondarily generalized
• Usually starts with loss of consciousness or at times sudden cry
Generalized
Tonic-Clonic • Tonic phase: jaw snaps followed by 10-15 seconds or longer of tonic
spasms, apnea & cyanosis
• Clonic phase: 1-2 minutes of rhythmic generalized contractions
• Postictal state: vomiting, confusion, somnolence & intense headache
• Repetitive seizures consisting of brief, often symmetric muscular
Myoclonic
contractions with loss of body tone & falling or slumping forward
Infantile • Begin at 4-8 months
spasms (West • Brief symmetric contractions of neck, trunk, extremities
syndrome) • EEG shows hypsarrhythmia

VI. MANAGEMENT
• Different societies and institutions have different recommendations for AED therapy
• The table below are commonly used drug therapies for specific seizure types:
SEIZURE TYPE INITIAL DRUG THERAPY OF CHOICE

Focal seizure • Oxcarbazepine, carbamazepine

Absence seizure
Juvenile myoclonic
epilepsy (12-18 years old)
Infantile spasms
Epilepsy with generalized
tonic-clonic seizures only
• Ethosuximide: as effective as valproic acid but less toxic
(most common initial drug for absence seizure)
• Valproic acid, lamotrigine (less effective)
• Levetiracetam, topiramate, valproic acid
• Adrenocorticotropic hormone (ACTH),steroids, vigabatrin
• Levetiracetam, lamotrigine, topiramate, valproic acid
Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted).Elsevier:2020

VII. RISKFOR EPILEPSY(IN%) AFTERA FEBRILESEIZURE

• Neurodevelopmental abnormalities (33%)
I
• Complex febrile seizure (29%)
• Family history of epilepsy (18%)
• Fever <l hour before febrile seizure (11 %)
• Complex febrile seizures (6%)
• Recurrent febrile seizures (4%)
• Simple febrile seizure (1%)
447
FEBRILE SEIZURE
I. ETIOPATHOGENESIS
• Most common seizure disorder in childhood
• Age: 6-60 months old
• Axillary temperature >38°C
II. MANIFESTATIONS
SIMPLE FEBRILE SEIZURE COMPLEX FEBRILE SEIZURE*
Duration • Lasts a few seconds to 515 minutes • >15 minutes
• Initiallygeneralized & tonic-clonic,then
Presentation • Focal seizure activity
a brief period of post-ictal drowsiness
Deficits • No focal neurologic deficit • May have focal neurologic deficit

• None • Repeated convulsions occur

Recurrence within 24 hours
• Occurs only once in 24 hours
of any one of the featuresof complexfebrileseizure classifiesit as such
• Fulfillment

III. DIAGNOSIS

DIAGNOSTICS FINDINGS/REMARKS

• Indications:
• For all infants younger than <6 months, who present with fever & seizure
• If the child is ill-appearing
• At any age with clinical signs or symptoms of concern
Lumbar
• For children 6-12 months of age, LP is an option if:
puncture (LP)
• Simple febrile seizure and is deficient in Hib and streptococcal
immunization (or if with unknown immunization status]
• Pretreated with antibiotics
• PPS recommends LP for all <18 months of age with first febrile seizures
• Not done if first episode and is otherwise neurologically healthy
EEG
• Not predictive of future recurrence of febrile seizures or epilepsy
• Electrolytes & CBCare not routinely recommended for a first simple seizure
Serum studies • Blood glucose if with prolonged post-ictal obtundation of if with poor
oral intake (prolonged fasting)
• Not recommended after a first simple febrile seizure (no evidence)
Neuroimaging
• Workup of children with complex febrile seizure need to be individualized

IV. MANAGEMENT
General • Counseling and education on acute management and first aid
Aspects • Reassurance, emotional support, and allaying fears of the parents
• Given to address the fever and decrease the comfort
Antipyretics
• Does not reduce the risk of having a recurrent febrile seizure
• Can reduce the recurrence of febrile seizures
Anticonvulsants
• However, its adverse side effects do not warrant their use

V. RISK FACTORS FOR RECURRENCE OF FEBRILE SEIZURES

Major Risks • Age <1 year, duration of fever <24 hours, and fever 38-39 °C

• Family history of febrile seizures, family history of epilepsy, complex

Minor Risks
febrile seizure, daycare, male gender, hyonatremia at presentation
Recurrence risk estimated based on number of risk factors:
• No risk factors: 12%
• 1 risk factor: 25-50%
• 2 risk factors: 50-59%
• 3 or more risk factors: 73-100%
448
STATUS EPILEPTICUS (SE)

I. STATUS EPILEPTICUS DEFINED BY INTERNATIONAL LEAGUE AGAINST EPILEPSY (ILAE)

• Condition resulting either from the failure of the mechanism responsible for seizure
termination or from initiation of mechanisms which lead to abnormally prolonged seizures
Can have long-term consequences which includes neuronal death, neuronal injury, and
alteration of neuronal networks, depending on the type and duration of seizures

OPERATIONAL DIMENSION OPERATIONAL DIMENSION

TYPE OF SE WHEN A SEIZURE IS LIKELY WHEN A SEIZURE MAY CAUSE
TO BE PROLONGED LONG-TERM CONSEQUENCES
Tonic-clonic SE 5 minutes 30 minutes
Focal SE with
impaired 10 minutes >60 minutes
consciousness

Absence SE 10-15 minutes unknown

Source:Trinka.E, et al. A definitionand classificationof statusepilepticus.Epilepsia:2015

II. APPROACH AND MANAGEMENT

TIME LINE INTERVENTIONS

• Assess airway, breathing, circulation, disability, neuro exam. Manage
as needed
• Note the time of onset of seizure and monitor vital signs
• Assess oxygenation: provide oxygen via nasal cannula/face mask,
0-5 minutes consider intubation if needed
(stabilization • ECGmonitoring
phase) • Do random blood sugar test. If sugar is <60 mg/dL,
0 Give 2 mL/kg of025W IV if>2 years old
0 Give 4 mL/kg 012.5W IV if <2 years old
• Establish IV access and collect blood for electrolytes, CBC,toxicology
screen (if appropriate), and anticonvulsant levels
• If seizure stops, provide symptomatic care
• If seizure continues, choose one of the following equivalent first-line options:
0 Diazepam IV0.2 mg/kg/dose, max dose 10 mg/dose; may repeat dose once

5-20 minutes 0 Midazolam IV 10 mg for >40 kg, 5 mg for 13-40 kg; single dose

(initial therapy 0 Lorazepam IV 0.1 mg/kg/dose, max 4 mg/dose; may repeat dose once
phase) • If none of the 3 options above are available, choose ONE of the ff:
0 Rectal diazepam 0.2-0.5 mg/kg/dose, max 20 mg/dose; single close
0 Intranasal/buccal midazolam
0 Phenobarbital IV 15 mg/kg/dose; single dose
• If seizure stops, provide symptomatic care
• If seizure continues: there is no evidence-based preferred second
therapy of choice
20-40 minutes
• Choose one of the following second line options and give as a single dose:
(secondary
therapy phase)
0 Valproic acid IV 40 mg/kg, max 3000 mg/dose
0 Levetiracetam IV 60 mg/kg, max 4500 mg/close

40-60 minutes
• If none of the above options are available, give phenobarbital IV 15
mg/kg/dose; single dose (if not given already)
• If seizure stops, provide symptomatic care
• If seizure continues, there is no clear evidence to guide therapy in this
I
(third therapy phase. Choices include repeat of second-line therapy or anesthetic
phase) doses of either thiopental, midazolam, pentobarbital, or propofol (all
with continuous EEG monitoring)
Source:GlauserT,et al. Epilepsy
Curr.2016

449
 SECTION FIVE

-------~-
TUBEROUS SCLEROSIS
NEUROCUTANEOUS SYNDROMES

I. ETIOPATHOGENESIS
Mode of inheritance: autosomal dominant
• Mutations: Tuberous sclerosis complex gene 1 and gene 2 (TSCl and TSC2) which
encodes for hamartin and tuberin respectively (both act as tumor suppressor genes)

II. FOUR MAIN MANIFESTATIONS

• Mental retardation
• Seizures
• Cutaneous lesions
• Tumors in various organs

III. DIAGNOSTICCRITERIA
• Careful examination of patient and parents is nearly as sensitive and much more cost
effective than imaging studies of another organ system
• Diagnosis should fulfill 2 Major OR 1 Major+ 2 Minor criteria

MAJOR FEATURES MINOR FEATURES

Skin lesions
• Facial angiofibromas
• Ungual or periungual fibromas
• Hypomelanotic maCLtles(>3)
• Cerebral white matter migration lines
• Shagreen patch
• Gingival fibromas
Brain lesions • Bone cysts
• Cortical tuber • Retinal achromatic patch
• Subependymal nodule • Confetti skin lesions
• Subependymal giant cell astrocytoma • Nonrenal hamartomas
Eye lesions • Multiple renal cysts
• Multiple retinal hamartomas • Hamartomatous rectal polyps
Tumors of other organs
• Cardiac rhabdomyoma
• Renal angiomyolipoma
• Pulmonary lymphangioleimyomatosis

IV. DIAGNOSTICS
• MRI of the brain reveals findings in most cases
• Echocardiography
• Imaging studies to locate hamartomatous lesions (e.g., ultrasound of kidneys)
• Genetic diagnosis has limited applications

V. MANAGEMENT
• No specific treatment
• Manage seizures
• Surgical management of tumors if warranted (e.g., ventricular obstruction)

450
NEUROFIBROMATOSIS
NEUROFIBROMATOSIS TYPE 1
DISEASE (VON RECKLINGHAUSEN NEUROFIBROMATOSIS TYPE 2
DISEASE)
Mode of • Both are autosomal dominant
Inheritance
• NF-1 gene mutation on • NF-2 gene mutation on
chromosome 17 q 11.2 chromosome 22ql.ll
Mutations
• Encodes for neurofibromin which • Encodes for merlin or
inhibits Ras oncogene schwannomin
• Progressive disease that can • Less common
affect almost every organ • Characterized by development
Clinical of CNS tumors, notably bilateral
• Most common skin lesion are
Manifestation
cafe-au-lait spots (light brown, vestibular schwannomas
well demarcated pigmentation)
NF-1 (most prevalent type) is NF-2 is diagnosed when 1 of the
diagnosed when any 2 of the following are present:
following are present: • Bilateral B'hnerve masses
• :e6 cafe au lait macules >5 mm in (acoustic neuroma)
diameter in prepubertals & >15 • Parent, sibling, or child with
mm in postpubertal individuals NF-2 & either unilateral B'" nerve
(predilection for the trunk & masses or any 2 of the following:
extremities with sparing of face) neurofibroma, meningioma,
• Axillary or inguinal freckling with glioma, schwannoma
multiple hyperpigmented areas
Diagnostic 2-3 mm in diameter
Criteria• • :e2iris Lischnodules (hamartomas
located within the iris)
• :e2 neurofibromas or one
plexiform neurofibroma (along
the skin, PNS, blood vessels &
within viscera)
• Distinctive osseous lesion
• Optic glioma
• 1" degree relative with NF-1
whose diagnosis was based on
the aforementioned criteria
• Symptomatic treatment
• Multidisciplinary approach
• Ophthalmologic examination
• Neurologic assessment
Management
• Blood pressure monitoring
• Scoliosis evaluation
• Developmental screening
• Genetic counseling
of criteriaimprovesas childgrowsolder.Diagnosiscannotbe madewithcertaintybefore1 yearofage in
·Reliability
almosthalfof childrenwitha negativefamilyhistory.DNAtestingis unnecessaryifdiagnosticcriteriaare met.

of Pediatrics(21sted).Elsevier;2020
Source:KliegmanR.et al. NelsonTextbook
(7thed).LippincottWilliams& Wilkins;2006
MenkesJ. et al. ChildNeurology
I
451
 SECTION SIX
CNS INFECTIONS
TUBERCULOUS (TB) MENINGITIS
I. ETIOPATHOGENESIS
• From metastatic caseous lesion in the cerebral cortex or meninges that develop during
the lymphohematogenous dissemination of primary infection
Gelatinous exudates infiltrate the corticomeningeal blood vessels, which produces
inflammation, obstruction, and subsequent infarction of the cerebral cortex
• Brainstem is the site of greatest involvement, commonly affecting cranial nerves Ill, VI
and VII
• Exudates also interfere with the flow of CSF resulting in a communicating hydrocephalus
• Most common in children 6 months to 4 years of age
• Accompanies miliary tuberculosis in 50% of cases

II. MANIFESTATIONS
• More rapid progression in infants and young children
• Signs and symptoms: acute onset of hydrocephalus, seizures, and cerebral edema
• More commonly progresses over weeks in 3 stages

STAGE MANIFESTATION
• Lasts 1-2 weeks
• Non-specific signs and symptoms (e.g., feve1; headache, irritability,
1" stage
drowsiness and malaise)
• Infants may experience loss of developmental milestones
• Focal neurologic signs ( e.g., nuchal rigidity, seizures, positive Kernig and
Brudzinski, hypertonia, cranial nerve palsies)
2 nd stage
• Development of hydrocephalus, increased ICP and vasculitis
• Signs of encephalitis (disorientation, movement disorder or speech impairment)
3,d stage • Coma, hemiparaplegia, hypertension, deteriorating vital signs and death

Ill. DIAGNOSIS

DIAGNOSTICS EXPECTED FINDINGS

• Refer to "Special diagnostic tests" discussed above
• Acid-fast organism almost never seen on smear
CSF analysis
• Ground glass appearance of fluid which forms pellicle when spun
• Ancillary tests to aid in diagnosis: PCR, ELISA, latex agglutination
• Basilar enhancement
Cranial Imaging • Communicating hydrocephalus
(CT or MRI) • Cerebral edema
• Focal ischemia

Other test • Refer to Pulmonology section for diagnosis of tuberculosis in children

IV. MANAGEMENT

DIAGNOSTICS EXPECTED FINDINGS

• Newly diagnosed: ZHRZE + lOHR
Anti-Koch's
• Previously treated drug-susceptible: 2HRZES + lHRZE + 9HRE

Corticosteroids • Improves survival but does not prevent severe disability

Sources:KliegmanR. et al. NelsonTextbook of Pediatrics
(21sted.).Elsevier:2020
MenkesJ. et al. ChildNeurology
(7thed.).LippincottWilliams& Wilkins:2006
Vande BosF.el al. TropicalMedicineandInternational Health:2004
WorldHealthOrganization.
Treatment of Tuberculosis
in children.Geneva.Switzerland:2010
452
VIRAL MENINGITIS
I. ETIOPATHOGENESIS
• Etiologic organisms:
0 Echovirus (Enterovirus subgroup of Picornavirus family)
° Coxsackie virus, adenovirus, cytomegalovirus, herpes simplex virus

II. MANIFESTATIONS
• Depends on etiologic agent
• May vary from mild illness to severe manifestations such as seizures, coma and death
• In general, viral meningitis have a more benign clinical course than bacterial meningitis
AGENTS PRESENTATION REMARKS

• Ranges from mild, self-limited illness

• Most common cause of
Enterovirus with primarily meningeal involvement to
viral meningoencephalitis
severe encephalitis
• Important cause of aseptic
• Similar to enterovirus but presents with
Parechovirus meningitis/encephalitis
more severe MRI fesions of cerebral cortex
in infants
• Usually presents with focal brain involvement
Herpes • Temporal lobe encephalitis is common • HSV 1 causes severe
Simplex in HSV 1 sporadic encephalitis in
Virus (HSV) • Mild & transient meningoencephalits may children and adults
accompany genital herpes infection with HSV2
• Most common manifestation of CNS • May cause CNS infection in
Varicella-
involvement is cerebellar ataxia temporal relationship with
Zoster Virus
• May present with severe encephalitis chicken pox
• Usually presents with mild • May cause deafness from
Mumps
meningoencephalitis damage of B'hcranial nerve

III. DIAGNOSIS
DIAGNOSTIC FINDINGS/ REMARKS

• Refer to "Special diagnostic tests" discussed above

CSF Studies
• Ancillary tests depending on suspected etiologic agent

EEG • Usually shows diffuse slow-wave activity without focal changes

Imaging • May show swelling of the brain parenchyma

IV. MANAGEMENT
• Supportive
• Only HSV has a specific treatment: Acyclovir

BACTERIAL MENINGITIS
I. ETIOPATHOGENESIS
AGE GROUP COMMON ETIOLOGIC AGENT*

First 2 months of life
2 months to 12 years old
Overall
•Modeof transmissionis usuallyby hematogenousdisseminationof microorganism
I
• Group B streptococcus
• Gram negative enteric bacilli
• L. monocytogenes
• S. pneumoniae
• H. influenzae
• N. meningitidis
• Viral infections are more common
froma distantsite of infection

453
II. MANIFESTATIONS
• Headache, nausea, vomiting, anorexia, restlessness, irritability, fever, neck pain & rigidity,
obtundation, coma, focal neurologic deficits (due to vascular occlusion)
• Nuchal rigidity secondary to inflammation of spinal nerves & roots produce meningeal
signs ofin·itation (Brudzinski and Kernig sign)
• 12-18 months old: Kernig and Brudzinski not consistently present
• Seizures due to cerebritis, infarction, or electrolyte losses (20-30% of patients)

III. DIAGNOSIS
DIAGNOSTIC FINDINGS/ REMARKS
• May see organisms on Gram stain and culture
Lumbar • If antibiotics started prior to LP: presumptive diagnosis of bacterial meningitis
puncture can be made despite negative cultures because pleocytosis, high CSFprotein
{LP) and CSF level, and low CSF sugar persist for several days even after antibiotics
GS/CS • If the LP is traumatic, it is prudent to rely on bacteriologic results because the
gram stain, culture, and glucose level may not be influenced by a traumatic LP
• Should be performed in all patients with suspected meningitis
Blood culture
• Reveals the responsible bacteria in up to 80-90% of cases of meningitis
High CRP,
ESR, and • Differentiates bacterial from viral causes of meningitis
procalcitonin

IV. MANAGEMENT
A. Antibiotics
• Ceftriaxone or cefotaxime
• Vancomycin if penicillin-resistant
Empiric Antibiotics
• Chloramphenicol for >l month old & allergic to penicillin
• Alternative: vancomycin + rifampin
• Penicillin G IV for 5-7 days
N. meningitidis • Alternative for penicillin allergic patients: meropenem or
vancomycin
• Vancomycin + 3" 1 generation Cephalosporin or Penicillin IV for
10-14 days
S. pneumoniae
• Vancomycin added for presumed S. p11eumo11iae meningitis
due to risk of resistance to penicillin, cefotaxime & ceftriaxone
Penicillin-resistant
• Vancomycin
isolates
• Ampicillin
L. monocytogenes
• Alternative: IV trimethoprim-sulfamethoxazole
Hib • Ampicillin for 7-10 days

• Cefotaxime or ceftriaxone for 3 weeks OR at least 2 weeks

E.coli
after CSF sterilization

P, aeruginosa • Ceftazidime for 3 weeks OR at least 2 weeks after CSFsterilization

Partially treated • Ceftriaxone or Cefotaxime for 7-10 days

B. Dexamethasone (Intravenous) 0.15 mg/kg every 6 hours for 2 days

' Useful for H. influenza meningitis especially if given 1-2 hours before start of antibiotics
' Less fever, lower CSF protein, reduced auditory nerve damage

454
V. COMPLICATIONS
• Hydrocephalus (communicating type): acute complication of meningitis
• Subdural effusions due to continued transudation (10-30% of patients)
• SIADH: may exacerbate cerebral edema and lead to hyponatremic seizures
Sources:KliegmanR.et al. NelsonTextbook
of Pediatrics
(21sted.).Canada:Elsevier;2020
MenkesJ, et al. ChildNeurology(7thed.).LippincottWilliams& Wilkins;2006
Kimberlin
OW,et al. 2018 Reportof theCommittee on Infectious
Diseases.Academyof Pediatrics: 2018

BRAIN ABSCESS
I. ETIOPATHOGENESIS
• Most common between 4-8 years old
• Etiology: embolization due to congenital heart disease with right to left shunts,
meningitis, chronic otitis media & mastoiditis, face & scalp infections, orbital cellulitis,
dental infections, penetrating head injuries, and VP shunt infections
Areas affected: cerebrum 80%; occipital lobe, cerebellum & brain stem 20%
• Majority are single abscess; 30% multiple
• Etiologic agents: S. aureus, streptococci, anaerobes, gram negative aerobic bacilli
(Proteus, Pseudomonas, Haemophi/us, Citrobacter)

II. MANIFESTATIONS
• Early stage: nonspecific symptoms: low-grade fever; headache, lethargy
• Vomiting, severe headache, seizures, focal neurologic signs (hemiparesis), papilledema,
and coma

Ill. DIAGNOSIS
• Positive blood culture in 10%
• CSF not done to avoid herniation
• Cranial CT scan & MRI (most reliable methods)

IV. MANAGEMENT
A. Empiric antibiotics
0 Duration of antibiotics: 4-6 weeks
' Depend on the probable pathogenesis & most likely organism
Unknown cause • 3rd generation Cephalosporin + Metronidazole

Head trauma or • Nafcillin or Vancomycin with 3rd generation Cephalosporin

neurosurgery + Metronidazole

Due to CHO • Penicillin + Metronidazole

Infected VP shunt • Vancomycin + Ceftazidime

lmmunocompromised • Broad spectrum + Amphotericin B

8. Surgery
Aspiration for encapsulated abscesses
, Indications for surgery:
0 With gas in the abscess
0 Multiloculated abscesses
0 Posterior fossa location

V. PROGNOSIS
, Fungal cause
0 Associated infections like mastoiditis, periorbital abscess, sinusitis
I
• High mortality: multiple abscesses, coma
• Long-term sequelae: behavior & learning problems, hydrocephalus, seizures, hemiparesis

Sources:KliegmanR.et al. NelsonTextbook of Pediatrics(21sted.).Canada:Elsevier;2020

MenkesJ, et al. ChildNeurology(7thed.).LippincottWilliams& Wilkins;2006

455
 SECTION SEVEN
NEUROMUSCULAR DISORDERS

GUILLAIN-BARRE SYNDROME
I. ETIOPATHOGENESIS
• Autoimmune disorder thought to be a post-infectious polyneuropathy (symmetric
ascending muscle weakness or paralysis)
• Etiology: autoimmune reaction that develops in response to a previous infection
leading to aberrant demyelination of peripheral nerves & ventral motor nerve roots

II. MANIFESTATIONS
• Initial symptoms include numbness and paresthesia followed by
progressive weakness and areflexia
Symptoms
• Weakness begins in the lower extremities & progressively involves the
trunk, upper limbs & bulbar muscles (Landry ascending paralysis)
• Cranial nerve deficits leading to dysphagia, dysa~thria, facial weakness,
papilledema, autonomic dysfunction, respiratory muscle paralysis
Signs
• Dysphagia and facial weakness: signs of impending respiratory failure
• Miller-Fisher syndrome: acute ophthalmoplegia, ataxia, areflexia
• Onset of weakness usually follows a nonspecific viral infection
(gastrointestinal or respiratory tract) by 10 days (Campylobacter
jejuni, H.pylori, Mycoplasma pneumonia, and Zika virus)
Course • Maximal severity of weakness is reached by 4 weeks after onset
• Benign clinical course with spontaneous recovery within 2-3 weeks
• Tendon reflexes usually the last function to recover & lower extremity
weakness last to resolve
• Three clinical features are predictive of poor outcome with sequela:
° Cranial nerve involvement
Prognosis
• Need for intubation
• Maximum disability at the time of presentation

III. DIAGNOSIS

DIAGNOSTICS FINDINGS/ REMARKS

• Increased protein, normal glucose, no pleocytosis
CSF Analysis • Dissociation between high CSF protein and a lack of cellular
response is diagnostic (albuminocytologic dissociation)
Electrodiagnostic • Reduced motor and sensory nerve conduction velocity
Tests • "Electromyography may show acute denervation of muscles

IV. MANAGEMENT

• Admit for observation because ascending paralysis may occur

within 24 hours
• Monitor respiratory effort by spirometry to identify hypoventilation
Acute stage
and respiratory failure
• For rapidly progressive ascending paralysis: may give !VIG(0.4
grams/kg/day for 5 consecutive days)
• Prevention of uli::ers
Supportive care • Pain management
• Nutritional support

Sources:KliegmanR,et al. NelsonTextbook of Pediatrics(21sted.).Canada:Elsevier;2020

MenkesJ, el al. ChildNeurology(7thed.).Lippincott
Williams& Wilkins;2006

456
CEREBRAL PALSY (CP)
I. ETIOPATHOGENESIS
• Group of permanent disorders of movement and posture causing activity limitation that
are due to non-progressive disturbances in the developing fetal or infant brain
Motor disorders are often accompanied by disturbances of sensation, perception, cognition,
communication, & behavior as well as by epilepsy and secondary musculoskeletal problems

A. Epidemiology:
More common in males
Major lesions that contribute to CP in preterm babies are:
lntracerebral hemorrhage
0

Periventricular leukomalacia
0

B. Risk Factors for Developing CP:

Congenital anomalies external to the CNS
Intrauterine exposure to maternal infections
Low birth weight especially <1000 g at birth

II. MANIFESTATIONS
• Arms more than legs (shows hand preference at a very early age)
• Decreased spontaneous movement on the affected side
Spastic • Delayed walking or walks on tiptoes
hemiplegia
• Circumductive gait
(25%)
• Spasticity apparent especially in the ankles
• Seizures (1/3 of patients) & cognitive impairment (25%)
• Bilateral spasticity of the legs (legs> arms)
• Damage to the immature white matter (during the 20-34'h week AOG)
• Commando crawl
Spastic diplegia
• Increased DTRs, (+) Babinski sign, and ankle clonus
(35%)
• Scissoring posture of extremities is seen when child is suspended by the axilla
• Normal intellect
• Periventricular leukomalacia (most common neuropathologic finding)
• Most severe form of CP due to marked motor impairment of all extremities
& high association with intellectual disabilities & seizures
Spastic • Swallowing difficulties due to supranuclear bulbar palsy
quadriplegia • Severe periventricular leukomalacia & multicystic encephalomalacia
(20%) (most common neuropathologic lesions)
• Increased tone and spasticity in all extremities, brisk reflexes and plantar
extensor responses

• Hypotonic with poor head control and marked head lag

• UE more than LE affected with dystonia (rigid muscles throughout their
Athetoid / range of motion and involuntary contractions can occur)
dyskinetic/
• Feeding difficulties, tongue thrust, drooling
extrapyramidal
(15-20%) • Absent or slurred speech
• Intellect is preserved; seizures are uncommon
• Can also be caused by kernicterus

Ill. DIAGNOSTICS
• Baseline EEG & cranial MRI scan (to determine the extent and location of structural

I
lesions and associated congenital malformations)
• Hearing & visual function tests
Sources:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Canada:Elsevier;2020
MenkesJ, et al. ChildNeurology(7thed.).LippincottWilliams& Wilkins;2006

457
IV. MANAGEMENT
• Multidisciplinary approach in the assessment & treatment
• For tight heel cord: tenotomy of Achilles tendon
• Drugs for spasticity:
Diazepam (oral): 0.01-0.3 mg/kg/day BID or QID
Baclofen: 0.2-2 mg/kg/day BID or TIO
0 Dantrolene: 0.5-10 mg/kg/day BID
Levodopa (for dystonia): 0.5-2 mg/kg/day
Reserpine or Tetrabenazine (for hyperkinetic movements)
Botulinum toxin
Deep brain stimulation

REFERENCES
1. Berg, A., Berkovic, S., Brodie, M., Buchhalter, J., Cross, J., and van Emde Boas, W. et al. Revised
terminology and concepts for organization of seizures and epilepsies: Report of the ILAE
Commission on Classification and Terminology, 2005-2009. Epilepsia 2010 51(4), 676-685
2. Dooley, ). The evaluation and management of paediatric headaches. Paediatrics and Child
Health, 2009, 14(1), 24-30. doi: 10.1093/pch/14.l.24
3. Febrile Seizures: Guideline for the Neurodiagnostic Evaluation of the Child with a Simple
Febrile Seizure. PEDIATRICS,2011, 127(2), 389-394
4. Fenichel, G. Clinical Pediatric Neurology A signs and symptoms approach 6th ed. 2009
Philadelphia: Saunders Elsevie1c
5. Glauser T, Shinnar S, Gloss D et al. Treatment of Convulsive Status Epilepticus in Children.
Epilepsy Cunc 2016 Jan-Feb; 16(1): 48-61.
6. Headache Classification Committee on the International Headache Society (HIS): The
International Classification of Headache Disorders, 3rd Ed. 2013 Cephalagia; 33(9):629-808
7. Jan, Mohammed and R Al-Buhairi, A and Baeesa, Saleh. Concise outline of the nervous system
examination for the generalist. Neurosciences 2001 (Riyadh, Saudi Arabia). 6. 16-22.
8. Kliegman, R., Stanton, B., St. Geme, J., Schor, N., and Behrman, R. Nelson Textbook of Pediatrics
21st ed. 2020. Canada: Elsevier
9. Menkes, J., Sarnat, H., and Maria, B. Child Neurology 7th ed. 2006 Philadelphia: Lippincott
Williams and Wilkins.
10.PedMIDAS Tool I Pediatric Migraine I Headache Cente1c 2018. Available from https://www.
cincinnatichildrens.org/service/h/headache-center/pedmidas
11.Pellock, J., Bourgeois, B., Dodson, W., Nordli, Jr, D., and Sankar, R. Pediatric Epilepsy Diagnosis
and Therapy 3rd ed. 2008. New York: Demos Medical Publishing.
12.Teasdale G, et al. Assessment of coma and impaired consciousness. Lancet; 1974
13.Trinka, E., Cock, H., Hesdorffer, D., Rossetti, A., Scheffer, I., and Shinnar, S. et al. A definition
and classification of status epilepticus - Report of the ILAE Task Force on Classification of
Status Epilepticus. Epilepsia, 2015 56(10), 1515-1523.
14.Van de Bos, F., Terken, M., Ypma, L., Kimpen, )., Ne!, E., and Schaaf, H. et al. Tuberculous
meningitis and miliary tuberculosis in young children. Tropical Medicine And International
Health, 2004 9(2), 309-313.
15.Williams, H. Spinal sinuses, dimples, pits and patches: what lies beneath. Archives of Disease
in Childhood-Education and Practice, 2006 91(3), ep75-ep80. doi: 10.l 136/adc.2006.105643
16.World Health Organization. Rapid Advice: Treatment of Tuberculosis in children. 2010
Geneva, Switzerland: WHO
17.Kimberlin OW, Brady MT,Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee
on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018

458
ACUTE
CARE
~ PEDIATRIC BASIC LIFE SUPPORT

0 PEDIATRICADVANCED LIFE SUPPORT AND ARRHYTHMIAS

~ SHOCK

~ FOREIGN BODIES AND CAUSTIC INGESTION

0 BURNS AND ELECTRICAL INJURIES

~ HEAD TRAUMA

~ TOXICOLOGY
 SECTION ONE
PEDIATRIC BASIC LIFE SUPPORT

BASIC LIFE SUPPORT (BLS) ALGORITHMS

I. BASIC LIFE SUPPORT FOR A SINGLE RESCUER

------------------------------·
;
r············.
Establish scene safety
c •••••••••••••
:

Shout for help

Activate emergency
response system

i Rescue breathing -1 breath every

!, .3-S secs (12-20 breaths per min)
'
Pulse check again every 2 mins
Has pulse
Simultaneously check pulse Has pulse -~~~-i;~;-~~t-ilEMT_i
__
Activate emergency response & observe for breathing/ ~No•mal ,esponder arrival
system after 2 mins Not normal only gasping/ not breathing ,
Add compressions if pulse stays breathing DEFINITEpulse in 10 secs? breathing
<60/min with poor perfusion,
despite adequate ventilation
If still no pulse, go to Initiate
, CPR Cycles box i Sudden collapse witnessed?
·----------------------------------'
Get defibrillator (AED)
Yes
Activate emergency response
system {if not already No
executed)
• -- •••••• -- • -- .. r. ··- ••....... -- ..

i, . Initiate CPR Cycles :

l:}f!~~~~:i~:~~:~~~~~~~~~...
30 compressions/ 2 breaths

:
:

:·-··-·--···-·-··--·····-···•
Rhythm check:
Shockable rhythm?

Non•shockable Shockable
rhythm rhythm
·--------------
---·-·-··-
Immediately resume CPRfor 2 min : - Deliver single shock
Rhythm check every 2 min : . Immediately resume CPR for 2 min
Resume cycle until ALSprovider arrives, or victim i- Resume cycle until ALS provider arrives,
___begins to move -----------·--·-··- ···-·· ····-·-- ..• 1 i··-or victim begins to.move_·-·--·--------

Source:PediatricAdvancedLifeSupportbyAmericanHeartAssociation2016
RappA. The UllimaleBLSCheatsheet;2019

I
461
II. BASIC LIFESUPPORT FOR DOUBLERESCUER

Establish scene safety

------i----------------
Shout for help
First rescuer: remain with victim
Second rescuer: activate
emergency response system &
get defibrillator {AED)
Rescue breathing - 1 breath
every 3.5 secs (12-20
breaths per min)
Simultaneously check pulse
Pulse check again every
Has pulse
and observe for breathing / Has pulse Monitor until EMT/
2 mins
only gasping/ not breathing responder arrival
Activate emergency Not normal DEFINITE pulse in 10 seconds? Normal
response system after 2 breathing ~----~-----~ breathing
mins
Add compressions if
No pulse;
pulse stays <60/min with
Only gasping/ not breathing
poor periusion, despite
adequate ventilation r
lf still no pulse, go to
Initiate CPR Cycles
Initiate CPR Cycles box First rescuer: 30 compressions / 2 breaths
Second rescuer: 15:2 compression / 2 breaths
Use AED immediately upon arrival
If second rescuer arrives, use -to-breath ratio
'·--· -------------------i-------------------------·

Rhythm check:
Shockab!e rhythm?

Non-shockable Shockable
rhyth rhythm

Immediately resume CPR for 2 min Deliver single shock

Rhythm check every 2 min Immediately resume CPR for 2 min
Resume cycle until ALS provider arrives, or Resume cycle until ALS provider
victim begins to move ,____arrives, or victim begins to move _____

Source:PediatricAdvancedLifeSupportby AmericanHeartAssociation2016
RappA. The UltimateBLSCheatsheet; 2019

462
 SECTION TWO
PEDIATRIC ADVANCED LIFE SUPPORT AND
ARRHYTHMIAS

BRADYARRHYTHMIAS
• Defined as heart rate (HR) that is slow compared with the normal range for the child's
age, level of activity, and clinical condition
• Symptomatic bradycardia: heart rate slower than normal with associated hypotension,
altered sensorium, or signs of shock

I. CLASSIFICATIONOF BRADYCARDIA
ETIOLOGY EXAMPLES
• Congenital abnormality of the heart
• Congenital or acquired heart pacemaker or conduction system
conditions that result in slow • Surgical injury to the pacemaker or
Primary
spontaneous depolarization or slow conduction system
conduction through the system • Cardiomyopathy
• Myocarditis
• Hypoxia
• Acidosis
• Non-cardiac conditions that alter the
Secondary • Hypotension
normal function of the heart
• Hypothermia
• Effect of drugs

II. TYPES OF BRADYARRHYTHMIA

CAUSES CHARACTERISTICS
• Sinus node depolarization rate slower
than normal for child's age • Regular rhythm with ventricular
• Often present in healthy children at rate slower than normal HR for age
Sinus
rest and in well-conditioned athletes • P waves with constant morphology
Bradycardia
due to their high stroke volume preceding every QRS complex
• May develop in response to hypoxia, • P wave is positive in limb lead II
hypotension, acidosis, and drug effects
Atrioventricular Block (AV Block): disturbance of electrical conduction through the AV node
First degree
• Prolonged PR interval
AV block
Second-degree • Progressive prolongation of the
Mobitz type I • Enhanced vagal tone PR interval until a P wave is not
(Wenckebach • Hypoxemia followed by a QR$ complex
Phenomenon) • Myocarditis • The cycle often repeats
• Electrolyte disturbance • Some, but not all, P waves are
Second-degree • Cardiac surgery blocked before they reach the
Mobitz type II • Acute rheumatic fever ventricle
• Intrinsic AVnode disease • Constant PR interval
• Drugs (e.g., calcium channel blockers,
digoxin, beta adrenergic blockers) • No relationship between P waves
and QRS complexes
Third degree
• No atrial impulses reach the
ventricles
• Ventricular rhythm maintained by
a slower pacemaker
I
Source:Pediatric
Advanced
LifeSupportbyAmericanHeartAssociation
2016

463
III. MANAGEMENT (Bradycardia with a Pulse and Poor Perfusion)

Identify and treat underlying cause

Maintain patent airway; assist breathing as necessary
Oxygen
Cardiac monitor to identify rhythm; monitor blood pressure and oximetry
10 / IV access
12-lead ECGif available; don't delay therapy
---------------------------------------------..,--------------------------------------------

Is there cardiopulmonary compromise?

Hypotension
Acutely altered mental status
Signs of shock
I
No
't Yes
CPR if HR< 60/min
, with poor perfusion despite ventilation ,
L---------------------- -------•-•-------------'

Support ABCs
Give oxygen
Observe
Bradycardia persists?
No
Consider expert consultation

Doses/Details

Epinephrine IO/IV0.0l mg/kg Epinephrine

(0.1 ml/kg of 1:10000 concentration) Atropine for increased vagal tone or
Repeat every 3-5 mins
primary AVblock
If IO/IV access not available but Consider transthoracic pacing/
endotracheal (ET} tube in place, may
transvenous pacing
give ETdose: 0.1 mg/kg(0.l ml/kg of
1:1000) ~ __-~~~~-t-~n-~~'.l_y_i~~-:~-~~:~
___________________
:

Atropine IO/IV 0.02 mg/kg

May repeat once
Minimum dose 0.1 mg and If pulseless arrest develops,
Maximum single dose 0.5 mg
go to Cardiac Arrest Algorithm

Source:PediatricAdvancedLife SupportbyAmericanHeartAssociation2016
KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.). Elsevier;2020
Park,M. (2008).Pediatriccardiologyfor practitioners(5th ed.).Philadelphi:MosbyElsevier.

TACHYARRHYTHMIAS
• Rapid abnormal rhythms that originate from the atria or the ventricles
• Can be tolerated without symptoms for a variable period of time
• May manifest with palpitations, lightheadedness, syncope, or acute hemodynamic
compromise (shock or cardiac arrest)
• Infants may manifest with poor feeding, irritability, rapid breathing

I. TYPES OF TACHYARRHYTHMIAS BASED ON MORPHOLOGY OF QRS COMPLEXES

NARROW QRS COMPLEX WIDE QRS COMPLEX
(S 0.09 seconds) (>0.09 seconds)
• Sinus tachycardia • Supraventricular tachycardia with aberrant
• Atrial flutter intraventricular conduction
• Supraventricular tachycardia • Ventricular tachycardia

464
II. SINUS TACHYCARDIAVERSUS SUPRAVENTRICULARTACHYCARDIA
SUPRAVENTRICULAR
PARAMETER SINUS TACHYCARDIA (ST)
TACHYCARDIA (SVT)
• Acute onset or acute termination,
• Gradual onset
• Infant: symptoms of congestive
• Signs of underlying cause of
Manifestations heart failure
ST can be identified such as
• Child: sudden onset of
fever, hypovolemia, anemia
palpitations
Usual heart rate
• <220/min • 2'220/min
(in infants)
Usual heart rate
• <180/min • ;;,180/min
(in children)
Source:PediatricAdvancedLifeSupportbyAmericanHeartAssociation
2016

III. PEDIATRICTACHYCARDIAWITH A PULSEAND POOR PERFUSIONALGORITHM

Identify and treat underlying cause

Maintain patent air\'Jay; assist breathing as necessary
Oxygen
Cardiac monitory to identify rhythm; monitor blood pressure and oximetry
I0/IVaccess
12-L ECGif available; don't delay therapy

NARROW (~0.09 sec) I I WIOE(>0.09sec)

Evaluate QRS duration
I I
Evaluate rhythm with 12-L If---------~
ECGor monitor I !
+ ..
Probable Sinus Tachycardia Probable Supraventricular Tachycardia
Compatible history consistent Compatible history (vague, nonspecific);
with known cause history of abrupt rate changes
P \'1avespresent/normal P waves absent/abnormal
Variable R-R; constant PR HRnot variable Hypotension
Infants: rate usually <220/min Acutely altered mental status
Children: rate usually <180/min
. + Si ns of shock

YES I No
Search for and treat cause
.....L ..
Consider adenosine if
Doses/Details rhythm regular and
QRSmonomorphic
Synchronized Cardioversion
Begin with 0.5-1 J/kg, if not
effective, increase to 2 J/kg
Sedate if needed, but don't delay
cardioversion
Atropine 10/IV dose:
First dose: 0.01 mg/kg rapid bolus
( maximum 6mg)
Second dose: 0. 2mg/kg bolus
(Maximum seconddose: 12mg)
Amlodarone 10/IV dose:
Smg/kg over 30-60 minutes
or
Procainamide 10/IV dose:
lSmg/kg over 30-60 minutes
Do not routinely administer
amiodarone & procainamide
together

I
465
IV. PEDIATRIC TACHYCARDIAWITH A PULSEAND ADEQUATEPERFUSIONALGORITHM

Identify and treat underlying cause

Maintain patent ain·,ay; assist breathing as necessary
Oxygen
Cardiac monitory to identify rhythm; monitor blood pressure and
oximetry
12-LECG if available; don't delay therapy

2
Narrow (5:0.09 sec) Wide (2:_0.09sec)
Evaluate QRS duration
3 9
Evaluat~,hythm Evaluate rhythm

4
Probable Sinus Tachycardia Probable Supraventricular
Compatible histoz y consistent with known Tachycardia
cause Compatible history (vague,
P waves present/normal nonspecific); histo1y of dbrupt rate
Variable R-R; constant PR
lnfanIs: rateusually<2.20/min
changes
P \·1aves absent/abnormal
Children: rate usually <1S0/min HR not variable
6····················· 1 Infants: rate usually 2:220/min
~~~!? .l!J!-1.~~ly
...9~!1~r.~~.:. z:.1.~9/f!l.i!~
..
Search for and treat cause
7 o,
Consider Vagal
Maneuvers :-
:............................. :
8
:·:·Esia·bliSKV.iSCUia·r·acce·ss
: • ConsidN adenoslne 0.1 mg/kg IV (maximum
first dose) May give second dose of 0.2mg/kg
: IV (maximum second dose 12mg)
~:. ~~~~ .~~pj~I.~l.~I!~~
.1~.~1~!1.i9.1!~
10 ... C .....
.
, Possible Supraventricular
Probable Ventricular
: Tachycardia
Tachycardia
: . R·R interval regular
: . Uniform QRS morphology
l
l2 : . Expe,t consultation st,only :
: recommended
: • Search afor and treat reversible
, causes
: • Obtain 12·Iead ECG
: • Consider pharmacologic
cardioversion
► Amlodarone I0/IV dose: Smg/kg
over 30·60 minutes
► Procalnam!de IO/IV dose:
15mg/kg over 30·60 minutes
► Do not rou1inely administer
amiodarone and procainamide
together
► May attempt adenosine if not
already administered
: • Consider electrical cardioverskm
: ► Consult pediatric cardiologist
• Attempt cardioversion with 0.5
to 11/kg (may incre.ise rn 2J/kg if
initial dose ineffective)
• Seddtc bcfurc Cd1diove1 sion
·--------------------------·
 SECTION
SHOCK
OVERVIEW OF SHOCK
I. ETI0PATH0GENESIS
• Shock is defined as a state in which the delivery of oxygen is inadequate to meet the
metabolic demands of vital organs and tissues
• All types of shock can lead to impaired functioning of vital organs such as the brain and kidneys
A. Classification of Shock by Effect on Blood Pressure:
COMPENSATED SHOCK
• BP within normal range
Characteristics • Clinical signs of inadequate
tissue perfusion
• Increased heart rate
• Delayed capillary refill time
• Decreased urine output
Findings
• Narrowed pulse pressure
• Fair distal pulses
• Cool extremities
B. ldentification of Shock by Type
ETIOLOGY CLINICAL CLUES
1) Hypovolemic Shock
• Massive losses from • History of fluid losses
diarrhea, hemorrhage, ( e.g., gastrointestinal,
burns, osmotic diuresis blood loss, burns,
• Inadequate fluid intake insensible losses)
2) Cardiogenic Shock
• Congenital heart disease
• Signs of CHF (e.g.,
• Arrhythmia
crackles, jugular
• Cardiomyopathy
venous distention,
• Myocarditis
hepatomegaly)
• Myocardial injury or
• Cyanosis
trauma
3) Septic Shock
• Fever, tachycardia,
• Bacterial, viral, fungal tachypnea,
infections leukocytosis
• Focus of infection
467
THREE
HYPOTENSIVE
(DECOMPENSATED) SHOCK
• BP below normal for age
• Clinical signs of impaired
perfusion
• Rapid progression to cardiac
arrest if not corrected
• Deteriorating clinical appearance
• Absent distal pulses and weak
central pulses
• Cold extremities
• Mottled skin
• Altered sensorium
PHYSIOLOGY
• Decreased µreload will lead
to decreased stroke volume &
cardiac output
• Compensatory mechanisms
include increased contractility
& increased afterload
• Decreased cardiac output due
to abnormal cardiac function
• Compensatory mechanisms:
tachycardia & increased
afterload
• Pulmonary edema may occur
• High or low SVR that leads to
maldistribution of blood flow
• Increased capillary permeability
& decreased cardiac contractility
I
 ETIOLOGY
4) Obstructive Shock
• Tension pneumothorax
• Pericardia! tamponade
• Constriction of ductus
arteriosus in infants with
ductal-dependent lesions
• Pulmonary embolism
5) Distributive Shock
• Anaphylaxis
• Spinal cord injury
CLINICAL CLUES
• History of trauma, sudden
difficulty of breathing
• Risk factors for
pulmonary embolism
are prolonged
immobilization,
malignancy,
hypercoagulable states
• Acute onset of
hypotension, often
accompanied by wheals,
angioedema, pruritus,
dyspnea, wheezing,
abdominal pain, vomiting,
or loss of consciousness
• History of trauma
• Dysautonomia
PHYSIOLOGY
• Impaired blood flow
due to limited venous
return to the heart or
limited pumping of blood
from the heart leads to
decreased cardiac output
and a compensatory
increase in SVR
• Vasodilation, increased
capillary permeability,
and pulmonary
vasoconstriction lead to
reduced cardiac output
• Loss of vascular tone leads
to severe vasodilation and
hypotension

II. DIAGNOSIS
DIAGNOSTIC POSSIBLE FINDINGS
• May be normal
CBC
• Leukocytosis or leukopenia, thrombocytopenia, anemia
• Low K· due to failure of the energy-dependent transport mechanism
• Increased lactate due to metabolic acidosis associated with tissue
Blood Chemistry hypoxia & anaerobic metabolism (present in all forms of shock)
• Renal dysfunction ( elevated creatinine, BUN)
• Hepatic dysfunction (elevated ALT,AST)
• Hypoxemia, hypercarbia
ABG
• Acidosis
• Used to monitor adequacy of oxygen delivery
scvo 2 (Central • Normal value is 70-75% if arterial 0 2 saturation is 100%
Venous 0 2
Saturation) • About 25-30% below the arterial 0 2 saturation if the arterial 0 2
saturation is not normal
Hematologic • Prolonged prothrombin and partial thromboplastin time
abnormalities • Reduced serum fibrinogen level

Sources:Pediatric
Advanced life SupportbyAmericanHeartAssociation2016
Kliegrnan
R. et al. NelsonTextbook
of Pediatrics
(21sted.).Elsevier;2020

468
III. MANAGEMENT
A. General Approach to Shock
APPROACH REMARKS
• Responsive and stable child: put in the most comfortable position
Positioning
• Hypotensive child: place the child in a supine position
• Maintain a patent or open airway
Support airway, • Provide high concentration of o, to all with shock via high flow delivery
oxygenation, system (e.g. Non-rebreather mask at lOLPM)
and ventilation • Oxygenation & ventilatory support for patients with ineffective
respirations, altered sensorium, or increased work of breathing
• For compensated shock: peripheral venous cannulation
Establish
• For hypotensive shock: immediate vascular access through
vascular access
intraosseous route if peripheral IV access is NOT readily achieved
• For hypovolemic shock: isotonic crystalloid solution (NS/LR) should be
given as a 20 mL/kg bolus over 5-20 minutes
• For suspected cardiogenic shock: smaller boluses of isotonic crystalloid
fluid at 5-10 mL/kg given over 10-20 minutes
• For trauma and massive bleeding: give packed red blood cells
(10 mL/kg) if the child does not respond to isotonic crystalloid
Provide fluid • For neurogenic, anaphylactic, and obstructive shock
resuscitation (cardiac tamponade): 20 mL/kg isotonic crystalloid
• Reassess the following parameters and repeat fluid boluses as needed:
0 Heart rate
• Capillary refill time
• Urine output
• Level of consciousness
• For most, fluid boluses can be administered up to and over 60 mL/kg
• SpO2 and heart rate monitoring as soon as possible
• Mental status
• Temperature
• Ensure optimal urine output (may insert indwelling urina1y catheter):
Monitor the
• Infants and children: 1.5 to 2 mL/kg/hr
patient
• Older child and adolescent: 1 mL/kg/hr
• Ongoing fluid losses
• Manage hypoglycemia (<45 mg/dL for neonates & <60 mg/dL for the rest)
• Consider central venous or arterial catheterization

B. Pharmacologic Management
Vasoactive medications are indicated when shock persists despite adequate fluid
resuscitation (fluid refractory shock)

VASOACTIVE DRUG MEDICATION EFFECT

• Increases cardiac contractility

• Dopamine
• Increases heart rate
Inotropes • Dobutamine
• Variable effects on systemic vascular
• Epinephrine
resistance (SVR)
• Epinephrine
• Increases SVR
• Norepinephrine
Vasopressors • Increases contraction of the
• Dopamine

I
myocardium ( except vasopressin)
• Vasopressin
• Decreases SVR
Phosphodiesterase
• Milrinone • Improves contractility
inhibitors
• Improves coronary artery blood flow

469
C. Summary Table of Commonly Used lnotropic And Vasoactive Drugs
DRUG DOSE RECEPTOR INOTROPY HR SVR PVR
Norepinephrine 0.02-0.2
mcg/kg/
min al> Pl, P2 i i i i
Dopamine 2-5
mcg/kg/ DAl, DA2 +--t +--t +--t +--t
min

5-10
mcg/kg/
min Pl, P2 >al i i -,t +--t

>10
mcg/kg/
min
al> Pl, p2 i i i i
Dobutamine 2-20
mcg/kg/
min
Pl> p2, al i i t t
Milrinone Loading
dose 25-
100 mcg/ Phospho-
kg diesterase
Infusion
III
inhibitor/
i i t t
0.25-0.75 TcAMP
mcg/kg/
min
Nitroprusside 0.3-0.5
mcg/kg/
Vascular
min
(max of
myocyte,
TcGMP
+--t -,i t t
10 mcg/
kg/min)
Epinephrine 0.1-1
mcg/kg/
min
al> Pl, P2 i i i i
SVR:Systemic
Vascular
Resistance
PVR:Pulmonary
VascularResistance

Source:AllenH. et al. MossandAdams'HeartDiseasein Infants,ChildrenandAdolescents(9th ed.);2016

D.For Specific Types of Shock:

For obstructive shock from ductal-dependent lesions like left ventricular outflow
obstruction: Prostaglandin El
0 For anaphylactic shock: epinephrine, antihistamines, short-acting beta2 agonist, steroids

Sources:
Pediatnc
Advanced LifeSupportbyAmericanHeartAssociation
2016
Kliegman
R, et al. NelsonTextbook
of Pediatncs
(21sted.).Elsevier.2020

470
 SECTION FOUR
FOREIGN BODIES & CAUSTIC INGESTION

FOREIGN BODIES IN THE EXTERNAL AUDITORY CANAL (EAC)

I. ETIOPATHOGENESIS
• Most aural foreign bodies are found in children ,;6 years old
• Most common objects removed include beads, tissue paper, toys, and insects

II. MANIFESTATIONS
• Frequently asymptomatic
• Common presentations include:
Witnessed by a parent/ caregiver that a foreign body was placed into the ear
Incidental finding during routine otoscopy
Decreased hearing or ear pain
0 Purulent or bloody ear drainage (rare)

Ill. DIAGNOSIS
Visualization of a foreign body in the EACon otoscopy
• Examine the other ear and nostrils for additional foreign bodies

IV. MANAGEMENT
A.Timing of Removal
Timing depends on type of foreign body
0 The following warrant urgent removal for the following reasons:
Button batteries: cause destruction due to strong electrical currents & pressure necrosis
0 Insects: live insect may damage tympanic membrane & middle ear (e.g., cockroach)
0 Penetrating foreign bodies: may penetrate middle ear structures
B. Elective Referral to an Otolaryngologist is warranted for asymptomatic patients with:
0 Spherical or other foreign body that is tightly wedged in the medial EAC (e.g., round
beads, paper, or rubber foam)
Foreign body that is pushed up against the tympanic membrane
° Foreign body that is not easily removed upon the first attempt
Uncooperative patients requiring sedation

C. Complications
0 Most common complication of foreign body removal: EACabrasion or laceration
0 Treatment consists of topical antibiotic ear drops

FOREIGN BODIES IN THE NOSE

I. ETIOPATHOGENESIS
• Intranasal foreign bodies typically present in toddlers & preschoolers and less often in
older children, adolescents, & adults with intellectual or behavioral disabilities
• Nasal foreign bodies are most commonly located on the floor of nasal passage under
inferior turbinate or superiorly in the nasal cavity just in front of middle turbinate

II. MANIFESTATIONS
• History of nasal foreign body insertion without symptoms (71-88%)
• Mucopurulent nasal discharge (17-24%)
• Others: foul odor (9%), epistaxis, nasal obstruction, mouth breathing
• Complications: septa! perforation with saddle nose deformity, nasal meatal stenosis,
inferior turbinate necrosis, collapse of the alar cartilage

III. DIAGNOSIS
• Visualization of the foreign body using a headlight or otoscope
• Some located high in nasal vault or posterior nasal cavity may require fiberoptic endoscopy
• Most nasal foreign bodies are radiolucent (radiographs are not routinely helpful)
• Plain radiographs are suggested when:
I
'

Type of foreign body (button battery or magnet) is not obvious on examination

There is significant epistaxis, black nasal discharge, pain, or facial swelling
471
IV. MANAGEMENT
A. Timing of Removal
0 Button batteries & magnets attached across nasal septum warrant urgent removal
0 Nasal foreign body extraction is an elective procedure
B. lndications for Subspecialty Referral
Posterior foreign bodies (not readily visualized by anterior rhinoscopy)
Chronic or impacted foreign bodies associated with marked inflammation
Button batteries which warrant urgent removal
Penetrating or hooked foreign bodies
Any foreign body that cannot be removed at initial attempt

Positive • Soft nasal foreign • Have patient (generally >3 years old) blow
Pressure body that occludes his or her nose while occluding the nostril
Technique anterior nasal cavity that has no foreign body
• Apply topical anesthesia
• Nonocclusive foreign
Using • In young children & uncooperative patients,
bodies in the anterior
Instruments sedation may be needed (removal under
portion of the nose
general anesthesia is advised)
Magnet • When foreign body is • Grasp one magnet with a mosquito
removal a magnet hemostat & pull it away from other magnet

FOREIGN BODIES IN THE GASTROINTESTINAL TRACT

I. ETIOPATHOGENESIS
Majority of foreign body ingestions occur in children between ages of 6 months & 3 years
• Most children are asymptomatic or have transient symptoms at the time of the ingestion
• Most foreign bodies that reach the gastrointestinal tract pass spontaneously
• Only 10-20% require endoscopic removal ( <l % require surgical intervention)
Commonly Ingested Objects
Coins • Most common foreign body ingested by children
Button • Contact of the flat esophageal wall with both poles of the battery conducts
batteries electricity that result in liquefaction necrosis & perforation of the esophagus
Sharp • The most common sharp-pointed objects ingested by children are straight pins,
objects needles, straightened paper clips, and fish bones

II. MANIFESTATIONS
• Esophageal foreign bodies tend to lodge in areas of physiologic narrowing
(upper esophageal sphincter, level of the aortic arch, lower sphincter)
• Dysphagia, drooling, gagging, substernal pain or irritation
Esophagus • Seconda,y airway symptoms (e.g.,wheezing/stridor) suggest tracheal compression
• If a sharp object perforated the esophagus, neck swelling, crepitus, or
pneumomediastinum may occur
• Acute lead toxicity present with lethargy and vomiting
• Objects that can pass safely into the stomach generally traverse the
Stomach& remainder of the GI tract without complications
intestines • Usually asymptomatic if objects reached the stomach
• If with gastric outlet obstruction: vomiting or feeding refusal

Ill. DIAGNOSIS
• Determining the presence of a foreign body requires an accurate history and often a
direct look down the airway
• Imaging can be used to confirm the findings and to localize the site of the foreign body
472
 A. Radiographs
° Flat objects (e.g. coins or disc batteries) usually orient in the coronal plane and appear
as a circular object on an anteroposterior projection
0 Objects lodged in the trachea tend to orient in the sagittal plane and are best seen in
lateral projection
0 Not readily seen on plain films: plastic, wood, thin metal objects, and bones

• X ray of neck, chest, & abdomen ("wide" CXR) • Chest X ray ("wide" CXR that includes
• Soft tissue lateral neck esophagus and abdomen)

B. Imaging with
° CT/MRI suggested if: symptomatic, dangerous characteristics of foreign body (>2 cm
width, >S cm length, or sharp), or type of foreign body is not definitively known
0 Ultrasound: may identify location & nature of foreign bodies in esophagus or stomach

IV. MANAGEMENT
A. Timing of Intervention
Urgent intervention is indicated if any are present (warning signs):
• Ingested object is sharp, long, large, a superabsorbent polyme,; a corrosive foreign
body (disc or button battery), a high-powered magnet or lead-containing
• Disc battery is in the esophagus (and in some cases in the stomach)
• Signs of airway compromise
• Evidence of near-complete esophageal obstruction (e.g.,patient cannot swallow secretions)
• Symptoms suggesting inflammation or intestinal obstruction (e.g. feve,; pain, or vomiting)
• GI obstruction or perforation
• Objects lodged for >24 hours or for an unknown duration (after this period,
complications such as transmural erosion, perforation, & fistulae are more common)
Expectant management {observation for 12 -24 hours) for blunt foreign bodies without
the above characteristics that are lodged in the esophagus in an asymptomatic patient,
because spontaneous passage often occurs.

B. Procedure
Flexible endoscopy for most extractions in esophagus, stomach, or proximal duodenum
0 Rigid endoscopy or retrieval with Magill forceps for objects in the hypopharynx or
proximal esophagus
Source:GilgerM, et al. Foreignbodiesof the esophagusandgastrointestinal
tractin children.Uptodate;2018.
KtiegmanR.et al. NelsonTextbookof Pediatrics(21sted.).Elsevier;2020

CAUSTIC INGESTIONS
I. ETIOPATHOGENESIS
• Caustic substance: strong acids/alkali capable of corroding or burning organic tissue
• Caustic ingestions: most common in children 1-3 years old
• May cause acute severe injury and long-term complications such as esophageal strictures
• Most commonly ingested caustic substance: household bleach
ALKALI INGESTION ACID INGESTION
Type of Necrosis • Liquefaction necrosis • Coagulation necrosis
• Superficial penetration of tissues (coagulum
Layer Involved • Deep bowel mucosa that forms on the mucosa limit deeper
penetration of the caustic substance)

I
• Standard liquid household
, Low viscosity results in rapid transit to
Characteristics detergents and bleaches
the stomach leading to gastric injury
have pH of9-11

• Upper airway injuries are more common

• May cause thrombosis of
Injury • May result in gastric outlet obstruction or
blood vessels
perforation in the antral or pyloric area

473
II. MANIFESTATIONS
• Upper airway injury: stridor, hoarseness, nasal flaring, retractions
• GI tract injury: dysphagia (most common symptom), drooling, retrosternal or abdominal
pain, hematemesis

III. DIAGNOSIS
• Timing of exposure and if witnessed or not
• Estimation of the amount of the substance ingested
• Exact type and brand of substance ingested
• pH determined from material safety data sheet or by the poison control center
• Rapid evaluation of vital signs, mental status, and pupils
History and
• Evaluate for respiratory compromise
Examination
• Drooling, refusal to eat or drink, and dysphagia suggest oropharyngeal or
esophageal injury
• Inspection oflips and oropharynx for plaques, circumferential ulcers, and
mucosa! sloughing
• Absence of lesions does not exclude significant esophagogastric injury
• Chest X ray in any patient with respiratory symptoms
• Signs of perforation: pneumomediastinum, mediastinal widening, subcutaneous
Imaging emphysema in the neck, pleural effusion, or subdiaphragmatic air
• Radiologic contrast studies generally are not valuable in the initial stage because
they are unreliable in detecting acute injury or in predicting stricture formation

IV. MANAGEMENT
A. Stabilization and Supportive Care
° Close observation with emphasis on prevention of vomiting, choking, or aspiration
0 Vomiting should not be induced
Diluting or neutralizing the caustic agent, administration of activated charcoal, or
doing gastric lavage is not recommended
B. Endoscopic Evaluation
Performed within 24 hours of ingestion to evaluate and stage the injury
Indications for endoscopy:
0 Definite history of caustic ingestion
0 Symptomatic patients
• Patients with oral lesions
Severity of esophageal injury is graded by endoscopic appearance of esophageal mucosa:
SEVERITY DESCRIPTION
Grade 0 • Normal mucosa

Grade 1 • Erythema and mucosa] edema

• Erythema, sloughing, ulceration, erosions, and whitish membranes
Grade 2 • Grade 2A: no deep focal or circumferential ulcers
• Grade 28: with deep focal or circumferential ulcers
• Deep mucosa! sloughing and ulceration and areas of brown-black or grayish
discolorations
Grade3
• Grade 3A: small scattered areas of focal necrosis
• Grade 3B: extensive necrosis
Grade 4 • Eschar, full thickness injury and perforation

C. Follow-Up Care for Evaluation of Stricture Formation:

All with significant esophageal burns (grade ZA & higher) on endoscopy or those
with persistent dysphagia should be evaluated with barium contrast studies 2-3
weeks post ingestion to assess for stricture formation
If strictures develop, esophageal dilation is performed to maintain or re-establish
normal swallowing

References:
Fishman.D. Causticesophageal injuryin children.Uptodate.Feb23,2018.
KliegmanR.et al. NelsonTextbookof Pediatrics(21sted.).Elsevier;2020
474
 SECTION FIVE
BURNS & ELECTRICAL INJURY

BURNS

I. ETIOPATHOGENESIS
• The spectrum of burn injuries is immense, ranging from simple first-degree burns with
no sequela to third-degree burns with hypermetabolic response
Scalds, which account for the most common etiology of burn in patients <5 years of age,
are the second most common type of burn injury among all age groups

II. CLASSIFICATIONOF BURNS

LAYER TIME OF
TYPE OF BURN APPEARANCE
INVOLVED HEALING
• Pink or light red, glistening,
blanches with pressure,
Superficial (First • Within 6 days
dry, with minor pain • Only the epidermis
Degree) without scarring
• Brisk capillary refill
time at the site of injury
• Painful, moist, bright
Partial Thickness • Epidermis and
red, often with blisters • Within 10-20 days
(Second Degree): papilla1y layer of
Superficial and bullae if with proper care
dermis
• Brisk capilla1y refill
• Pale,yellow-white in colo1;
Partial Thickness non-blanching, drier or • >21 days with
• Reticular layer of
(Second Degree): mildly moist, with blisters, residual scar
the dermis
Deep sensation by application formation
of pressure only
• Dry, white or black, • Epidermis is lost
Full thickness without blisters, with damage to • Many weeks, often
(Third Degree) without capillary refill the subcutaneous requires skin grafting
or sensation tissue
• Subcutaneous laye1;
• Extensive amount of • Many weeks, often
Fourth degree fascia,tendon,muscle,
necrotic tissue requires skin grafting
and/or bone
Source:ClinicalManualof Emergency byCrainandGershel.5thedition
Pediatrics

III. DIAGNOSIS
Obtain complete history surrounding the injury especially etiology (e.g., flame, scald,
electrical, chemical), time of exposure, whether the injury occurred in a closed space, and
the possibility of additional trauma
• Perform complete PE and look for head injuries, upper airway obstruction, cardiac
arrhythmias, eye and ear injuries, and circumferential burns (which can lead to extremity
compartment syndrome)

I
475
ASSESSMENT OF EXTENT OF BURNS BY BODY SURFACE AREA (BSA)

Front Back

2%
pi i 13%

Area ByageIn ye11&

0 1 6 10
Head(A/D) 10% 9% 7% 6%
Thigh(B/E) 3%" 3% 4% 5%·
Leg(C/F) 2% 3% 3% 3%

IV. MANAGEMENT
A. Fluid Resuscitation
0 Burn injury may lead to a combination of hypovolemic and distributive shock
0 Monitor weight, serum electrolytes, urine output, and nasogastric losses
0 Maintain urine output >0.5 ml/kg/hr

1. Indications for IV Fluid Resuscitation*

• Infants with burns >10% of BSA
• Children with burns >15% BSA
• Children with evidence of smoke inhalation
Considera bolusof20ml/kgofPLRor normalsalinesolution.Withhold
potassiumgenerallyforthe first48
hoursbecauseof largereleaseofpotassiumfromdamagedtissues

2. Parkland Formula (guide to estimate fluid needed):

4 mL lactated Ringer solution/ kg body weight/% BSAburned
Half is given for the first 8 hours calculated from the time of onset of injury
The other half is given for the next 16 hours.
Adjust the rate according to the patient's response to therapy

476
 B. Indications for Admission:
2"' degree burns: >5% TBSA in infants, 10% TBSA in children, 15% TBSA in adolescents
3'' degree burns: >2% TBSA
Significant electrical or chemical injury
Burns in critical areas such as face, hands, feet, perineum or joints
0 Suspicions of abuse or unsafe home environment
0 Patient with underlying chronic illness, multiple trauma
Evidence of significant inhalation injury

C. Oth er Asoects in Manaeement

• Early identification of patients who require intubation is critical
• Consider intubation for patients with >20-25% BSAburn or
Airway/Breathing
in respiratory distress (may be indicative of inhalation injury
such as upper airway edema, parenchymal damage, etc.)
• Morphine 0.1-0.2 mg/kg/dose q2-4h as needed IM/IV /SC;
Analgesia
max dose: 15 mg/dose
Indwelling Foley • For monitoring of urine output, decompression ofbladde1;
catheter and prevention of soiling of wounds
Nasogastric Tube • For decompression, if needed
Prophylaxis for
• Histamine-2 receptor blockers, antacids, PP!s
Curling's stress ulcers
Ophthalmologic
• Topical ophthalmic antibiotics: for abrasions
evaluation
Tetanus prophylaxis • As deemed appropriate
• Cooling decreases the severity of the burn if administered
Temperature within 30 minutes of injury and relieves pain
management • If burns are >10% BSA,cool for no more than 30 minutes
and apply clean, dry towels to avoid hypothermia
Chemical Burns • First aid is lavage with copious volumes of water
Source:ClinicalManualof EmergencyPediallicsby CrainandGershel.5th ed1t1on
Joffe.M.Emergency
Careof ModerateandSevereThermalBurnsin Children.Uptodate. July12,2017.
KliegmanR,et al. NelsonTextbook
of Pediatrics(21sted.).Elsevier:2020

ELECTRICAL INJURY
I. ETIOPATHOGENESIS
• Injury can occur through a variety of mechanisms (e.g., lightning strikes, household outlets)
• Mechanisms of injury:
Direct effect of electrical current on body tissues
° Conversion of electrical energy to thermal energy resulting in superficial and deep burns
0 Blunt mechanical injury from lightning strike, muscle contraction, or fall after electrocution
• Tissues with higher resistance (skin, bone, fat) have a tendency to heat up and
coagulate, rather than transmit current

II. MANIFESTATIONS
(range from mild superficial burns to severe multi-organ dysfunction & death)
SYSTEM MANIFESTATIONS

Cardiac • Ventricular fibrillation (with AC)or asystole (usually with DCor lightning)
Renal • Acute kidney injury, rhabdomyolysis from massive tissue necrosis
Neurologic • Loss of consciousness, autonomic dysfunction, paralysis, sensory deficits

Skin

Musculoskeletal
• Partial thickness to full thickness thermal burns
• "Kissing burns" at flexor creases
• Osteonecrosis and periosteal burns
I
Vascular system • Acute compartment syndrome, delayed arterial thrombosis

477
III. CLASSIFICATIONOF ELECTRICALBURNS
DEGREE REMARKS
• Rarely cause significant tissue damage
Low voltage • Initial lab evaluations include creatine kinase (CK) levels, ECG
burns monitoring for 4-6 hours and urinalysis (to check for myoglobinuria)
(<1,000 volts) • If there is significant soft tissue damage or arrhythmia, they should
be admitted for telemetry monitoring for at least 24 hours
• Can cause massive amounts of tissue damage and fractures either
from fall or from contraction of major muscle groups
High-voltage • CK and CK-MBis often used to evaluate cardiac injury but there is a lack
burns
(>1,000 volts) of evidence supporting its use especially in the absence of ECGchanges
• Evaluate serum electrolytes, serum troponin, BUN and creatinine,
myoglobin in urine
• Lightning injury is a dramatic occurrence that many people believe
is uniformly fatal. However, because the electrical energy from a
lightning strike often flows over the surface of the body rather than
Very high- through it, the survival rate is 65% or higher
voltage burns • Cardiac standstill and paralysis of the respiratory center are the most
(10 million common reasons for sudden death
volts) • A seemingly lifeless victim should still be treated because of a rare
phenomenon known as keraunoparalysis, which is a transient
paralysis with extreme vasoconstriction and sensory disturbances of
one or more extremities

IV. MANAGEMENT
Prolonged CPR should be undertaken following electrical injury regardless of the initial
rhythm
• Patients can have spontaneous cardiac activity but have paralysis of the respiratory muscles
• Secure the airway to prevent secondary cardiac or neurologic dysfunction
• Trauma resuscitation, including cervical spine immobilization
• Fluid resuscitation:
• Aggressive IV fluid replacement often required with soft tissue injury from a severe
electrical burn
Less fluid volumes required in lightning injuries
Parkland formula is not used since surface burns may grossly underestimate the extent
of injury

V. DISPOSITION
• Severely injured patients are admitted to an ICU
• Those with significant electrical burns are transferred to a burn center when stable
• When exposure to high-voltage is suspected, cardiac monitoring for 12-24 hours is
prudent, especially when there is history of cardiac disease, chest pain, or arrhythmia
Patients with mild symptoms, minor skin burns, normal ECG and urinalysis can be
observed for a few hours and discharged with prompt follow up based on their wounds
• Asymptomatic patients after a low-voltage exposure with a normal PE do not require
ancillary tests and can be discharged

Source:ClinicalManualof Emergency Pediatricsby CrainandGershel.5thedition

Joffe,M. Emergency
Careof ModerateandSevereThermalBurnsin Children.Uptodate.July 12,2017.
KtiegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Elsevier:2020

478
 SECTION SIX
HEAD TRAUMA
APPROACH TO HEAD TRAUMA
I. ETIOPATHOGENESIS
• Most head trauma in childhood is minor; however, some who appear low risk may have a
clinically important traumatic brain injury
• The challenge for evaluating minor head trauma is to identify clinically important
traumatic brain injury (ciTBI) while limiting unnecessary imaging and radiation exposure
Neuroimaging (usually with a CT scan) is highly sensitive for identifying brain injury that
requires acute intervention

II. DEFINITION OF TERMS

A. Minor Head Trauma
• History or signs of blunt trauma to the scalp, skull, or brain
<2 years old
• Infant or child is alert or awakens to voice or light touch
• GCS 14 or 15 at the initial examination
~2 years old • No abnormal findings on neurologic examination
• No physical evidence of skull fracture

B. Concussion
From a direct blow to the head, face, neck or a blow elsewhere with an "impulsive"
force transmitted to the head
Results in rapid onset of short-lived impairment of neurologic function that resolves
spontaneously
Symptoms: headache, confusion/disorientation, difficulties with memory, blank stare
or "stunned" appearance, inattentiveness, slow or incoherent speech, dizziness, gait
abnormalities, vomiting, and emotional )ability ( e.g., inappropriate laughing or crying)

C. Traumatic Brain Injury (TBI)

Associated with symptoms ( e.g., brief loss of consciousness, disorientation, vomiting)
Severity:
Mild TBI: GCS score 13-15
Moderate TBI: GCS score 9·12
Severe TBI: GCS score s8

D.Clinically Important Traumatic Brain Injury (ciTBI)

Presence of intracranial injury on CT scan associated with 2:l of the following:
Neurosurgical intervention (either surgery or invasive ICP monitoring)
Endotracheal intubation for the management of head injury
Hospitalization directly related to the head injury for 2:48 hours
Death
Depressed skull fracture warrants operative elevation
Symptoms: loss of consciousness, vomiting, headache, seizures
Signs of basilar skull fracture include CSF rhinorrhea or otorrhea, posterior auricular
hematoma (Battle sign), hemotympanum, and periorbital hematomas ("raccoon eyes")

Ill. DIAGNOSIS
IMAGING REMARKS

• Most children with minor head trauma do not need head CT scans
Head CT
• Decision to obtain a head CT should be made using clinical predictors to

I
Scan
determine risk of ciTBI

Skull • Little or no added value if a head CT is performed

Radiography

479
IV. APPROACH TO CHILDREN <2 YEARS OLD

• GCSs14
• Palpable skull fracture
• Altered sensorium (agitation, • Normal mental status
somnolence, slow response) • No parietal, occipital or temporal scalp
• Loss of consciousness hematoma
• Severe mechanism of injury: • No loss of consciousness >S seconds
Motor vehicle collision with patient
0 • No evidence of skull fracture
ejection or death of another passenger • Normal behavior according to the
Rollover
0
routine caregiver
Pedestrian or cyclist without helmet
0
• No high-risk mechanism of injury
struck by motorized vehicle
Head struck by high-impact object
0

'Skullradiographs
mayoccasionally
beusefulto screenfor fracturein selectedasymptomatic
patients3-24monthsold
.. Donotperformneuroimaging
forchildrenat verylowriskforTBI.Thesechildrenshouldmeetall of theabovecriterialistedabove

Observation for 4-6 hours for those with GCS15 and no altered mental status, but
with anv of the followina:
• Occipital, parietal, or temporal scalp hematoma
• History of loss of consciousness ;,,S seconds
• Not acting normally according to parent
• Vomiting that is self-limited
CT scanis performed
forworsening
signsandsymptoms
during
observation
period;
consideration
ofparentpreference:
patientis
<3 monthsold;or unwitnessed
traumaofconcern

V. APPROACH TO CHILDREN >2 YEARS OLD

• Normal neurologic examination

• GCS s14 • No physical evidence suggesting a skull fracture
• Signs of basilar skull fracture • No preexisting condition that might increase risk of
• Altered mental status intracranial hemorrhage (e.g., bleeding disorder)
• Prolonged loss of consciousness • Normal mental status
• No loss of consciousness, vomiting, severe headache
• No high-risk mechanism of injury
•oo notperformneuroimaging
for childrenat verylowriskfor ciTBI.ThesechildrenshouldmeetALLof the
followingcriteria

Skull radio.aravhs mav be reauested if:

• History of trauma is uncertain (e.g., skeletal survey in the evaluation of suspected abuse)
• To rapidly evaluate the location of a radiopaque foreign body
• In rare instances, to screen for fractures in selected asymptomatic patients 3 - 24 months
old with concerning scalp hematomas
If a screening skullradiograph
showsa fracture,thena headCTshouldbe performed.
If a screeningskullradiograph
showsno
fracture,the riskof a ciTBImaybe lower.

VI. DISCHARGEINSTRUCTIONS
• Caretakers of children with minor head trauma should be given instructions for
monitoring, when to seek medical help, and when to return for follow-up
Immediate medical attention is required when the following conditions are noted:
0 Inability to awaken the child as instructed
0 Persistent or worsening headache
Vomiting that begins or continues 4-6 hours after injury
Change in mental status or behavior
0 Unsteady gait, clumsiness or incoordination
0 Seizure
Source:Schutzman
S. Minorheadtraumain infants& children.UpToDate: 2016
McCroryP,
et al. ClinicalJournalOf SportMedicine:2013
480
 SECTION
COMMONLY ENCOUNTERED CASES IN TOXICOLOGY
ETIOLOGY
1) Paracetamol Toxicity
• Acute overdosage:
ingestion occurring
within a single 4-hour
period
• 150 mg/kg in children:
lowest dose capable of
producing significant
toxicity
2) Iron Toxicity
• Due to the direct
corrosive effects on
mucosa) tissue and
cellular dysfunction
• An elemental iron dose of:
0 20 mg/kg: abdominal
pain, diarrhea, vomiting
0 60 mg/kg may be fatal
3) /soniazid Toxicity
• Clinical effects occur
30 minutes to 2 hours
following acute ingestion
• Seizure dose: 80-120
mg/kg
4) Kerosene Toxicity
• Leading cause of
accidental ingestion in
children 56 years old
• May be absorbed in the
GIT (optimal), through
inhalation or via the skin
(minimal)
• Can depress the CNS and
lead to hypoxia
TOXICOLOGY
TOXICOLOGY
MANIFESTATIONS
• Stage f (0.5-24 hours):
0 Anorexia,vomiting. malaise
0 Normal lab results (except for
acetaminophen levels)
• Stage II (24-48 hours):
0 Resolution of symptoms
0 Elevated AST >1.000 IU/L
0 Prolonged INR
• Stage fl/ (3-5 days):
0 Peak AST >10,000 IU/L
0 Onset of liver failure
• Stage IV ( 4 days - 2 weeks):
0 Manifestations
resolve
0 AST normalizes in a few weeks
• fnitial period (0-6 hours):
0 Severe gastritis, vomiting
0 Tachycardia, hypotension
• Quiescentperiod(upto 12 hrs):
0 Deceptive improvement,
patient may appear stabilized
0 Subtle signs ofhypoperfusion
• Recurrentperiod(12-48 hours):
0 Multiorgan failure, GI bleeding,
letl1argy,vasomotor collapse,
pulmonary edema, hepatorenal
failure, hypoglycemia, acidosis
• Late period (2-6 weeks):
0 Gastric scarring, pyloric
obstruction, strictures
• Triad of acute toxicity: seizure,
coma, metabolic acidosis
• Severe overdose: seizures and
loss of consciousness
• Signs: hyperpyrexia, tachycardia,
dilated pupils, dysarthria,
hyperreflexia, hypotension, coma
• Nausea, vomiting, diarrhea,
cough, mucous membrane
irritation, difficulty breathing
• CNS symptoms: initially may be
excited but seizures may follow
• Signs: respiratory distress,
cyanosis, tachycardia, abdominal
tenderness, perianal burns,
altered sensorium
481
INITIAL MANAGEMENT*
• N-acetylcysteine (NAC)
should be started no later
than 8 hours from the time
of ingestion
• Insert NGT & do gastric
lavage with activated
charcoal (1 g/kg to make a
slurry)**
• Manage specific
complications: acute renal
failure, bleeding tendencies,
hepatic insufficiency,
metabolic problems (e.g.,
hypoglycemia, acidosis,
hypokalemia, hypocalcemia)
• Ask for amount of elemental
iron ingested, time ingested,
other substances ingested
• Collect 10 mL blood for total
serum iron (TSI) 3-5 hours
post ingestion and send
sample of gastric aspirate
• May request for stool exam
with occult blood and plain
abdominal radiograph (may
reveal presence of iron
tablets)
• Manage specific problems:
cerebral edema, decreased
PT, hypovolemic shock,
metabolic derangements,
renal failure, seizures
• Insert NGT& do gastric lavage
with activated charcoal (1 g/
kg to make a sluny)**
• To enhance excretion, give
alkalinization therapy with
NaHCO3 at 1 mEq/kg IV eve1y
6 hours until urine pH ;e7.S
• Remove clothes and do
decontamination procedures
I
• Give 0 2 via nasal cannula
• Do not induce emesis
• No benefit with using
activated charcoal
• Specific problems: gastritis,
seizures, aspiration
pneumonia
 ETIOLOGY
5) Or9anophosphate Toxicity
• Organophosphates
inhibit the action of
cholinesterase, the
enzyme that breaks
MANIFESTATIONS INITIAL MANAGEMENT*
• Muscarinic (mild): • Ifpoison absorbed dennally,
Malaise, vomiting, diarrhea,
0
inhaled, or ingested then
sweating, pain, salivation, miosis
vomited, provide oxygen,
remove the clothing and do
• Muscarinic & nicotinic (moderate):
Mild symptoms, plus sponge bath using alkaline soap
0

Dyspnea, decreased muscle • If poison ingested: insert

down acetylcholine to 0

strength, bronchospasm, NGT & do gastric lavage with

acetic acid and choline
• Can be absorbed
through inhalation,
ingestion, & dermal
contact
• Its toxic effects may
appear as early as 10
minutes to as late as 2
hours post-exposure
6) Benzodiazepine Toxicity
• Effects are mediated
mainly by binding with
GABAreceptors
• Most significant finding
of overdose: CNS
depression
• Other findings: slurred
speech, incoordination,
and ataxia
'Initialmanagementis to put the patienton NPOand start fluids(e.g., 05 0.3 NaClon maintenancerates. Refer
patientto toxicologyservice, as specificmanagementis usuallydose-related
"After lavage,give sodiumsulfate250 mg/kgin water to make a 10%solution(may be repeated onlyonce if initial
dose does not result in bowelmovementafter 1 hour)
muscle fasciculations, motor
incoordination, impairment of
speech, bradycardia, involuntary
defecation/urination
• Muscarinic,nicotinic& CNS(severe):
Moderate symptoms, plus
0
Respiratory paralysis, extreme
0
hypersecretion, coma, cyanosis,
hypotension, paralysis,
behavioral changes, seizures
• Symptoms: slurred speech,
dizziness, increased sleepiness
• Signs: changes in sensorium (may
be intermittent, may occur with
hallucinations), hypothermia,
bronchial hypersecretion, ataxia,
nystagmus, dysarthria, weakness,
aggressive behavior
activated charcoal (1 g/kg to
make a slurry)**
• Phenytoin for cases of
malathion, parathion, or
dichlorvos poisoning
• Pralidoxime chloride
• Atropine
• Manage other problems:
acidosis, pulmonary
congestion and seizures
• If within 1 hour of ingestion,
insert a NGT& do gastric
lavage with activated charcoal
at 1 g/kg to make a slurry*"
• Flumazenil (0.01 mg/kg) if
with respiratory depression
(may be repeated at 1-2
minute intervals up to a total
dose of 1 mg)
• Specific problems: hypoxia,
hypotension, respiratory
acidosis

REFERENCES
1. Allen, H., Shaddy, R, Penny, D., Fettes, T. and Cetta, F. Moss and Adams' Heart Disease in infants,
childrenand adolescents9th ed. 2016. Philadelphia:LippincottWilliams& Wilkins
2. AmericanHeart Association.PediatricAdvancedLifeSupport 2015.Availablefrom <https://ahajoumals.org>
3. CrainEF,GersheIJCandCwminghan1 SJ(eds).Clinical
ManualofEmergencyPediatJics
5thed.2011.Cambridge
University
Press
4. Dart, RC. Medical Toxicology 3rd ed. Philadelphia: Lippincott Williams and Williams. 2004
5. Fishman,D.Causticesophagealinjury in children.Uptodate.AccessedFebruary23, 2018.
6. Gilger, M, Jain, A, McOmbe,~ M. Foreign bodies of the esophagus and gastrointestinal tract in
children.Uptodate.AccessedFebruary 12, 2018.
7. HazinskiMF.NursingCareof the CriticallyIllChild.3rd ed; 2013. Elsevier
8. Hoffman,RSet al. Goldfrank'sManualofToxicologicEmergencies.9th ed. 2010 NewYork:McGraw-Hill. 2010
9. Joffe,M.EmergencyCareof Moderateand SevereThermal Burns in Children.Uptodate.AccessedJuly12, 2017
10. Kliegman, R., St. Geme, J., Blum, N., Shah, S., Taskei; R., and Wilson. Nelson textbook of
pediatrics 21st ed. 2020. Philadelphia:Elsevier
11. McCrory, P., Meeuwisse, W, Aubry, M., Cantu, 8., Dvorak, )., & Echemendia, R et al. (2013).
Consensus Statement on Concussion in Sport-the 4th International Conference on
Concussion in Sport Held in Zurich, November 2012. Clinical Journal Of Sport Medicine,
23(2), 89-117.doi: 10.1097/jsm.Ob013e31828b67cf
12. Minorhead trauma in infantsand childrenby Sara Schutzman,MD.UptoDate.AccessedJune4, 2016
13.Olson,KR Poisoningand DrugOverdose.6th ed. 2011 NewYork:McGraw-Hill.
14. Park,M.Pediatriccardiologyfor practitioners Sth ed. 2008. Philadelphia:MosbyElsevier
15. RappA. The UltimateBLSCheatsheet:2019 Availablefrom <https://emedce,t.com/blog/ultimate-bls-cheatsheet>

482
ESSENTIALS
INPEDIATRICS
 SECTION ONE
EXAMINATION OF THE PEDIATRIC PATIENT
VITAL SIGNS
HEARTRATE RESPIRATORY
rsLOOD PRESSURE (BP)* I
AGE 1 I
(beats/min) (bre::min) s;;t~;i~-~~stolic BP r-~M;.~-

Premature 120-170 40-70 55-75 35-45

0-3 months 100-150 35-55 65-85 45-55

3-6 months 90-120 30-45 70-90 50-65 Rectal
temperature
6-12months 80-120 25-40 80-100 55-65

1-3 years 70-110 20-30 90-105 55-70 36.6-37.9°(

or
3-6 years 65-110 20-25 95-110 60-75 97.9-100.2°F

6-12 years 60-95 14-22 100-120 60-75

>12 years 55-85 12-18 110-135 60-85
·RoutineBP measurementfor ;,3 years old
.. Sites to measure temperature:rectum(preferredin infants& youngchildren),mouth,axilla
Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Elsevier;2019

GLASGOW COMA SCALE (GCS)

INFANTS& SCORE
FUNCTION OLDER CHILDREN
YOUNG CHILDREN (Total=15)
Spontaneous Spontaneous 4
To Sound/ Command To Voice 3
Eye Opening
To Pain To Pain 2
None None 1
Appropriate for Age Oriented 5
Inconsolable Cry Confused 4
Verbalization Persistently Irritable Inappropriate 3
Restless/ Agitated, Lethargic Incomprehensible 2
None None 1
Spontaneous Obeys 6
Localizes Pain Localizes Pain 5
Withdraws Withdraws 4
Motor
Reflex Flexion Reflex Flexion 3
Reflex Extension Reflex Extension 2
None None 1

GROWTH & DEVELOPMENT

I. AVERAGEBIRTH MEASUREMENTS
PARAMETER AT BIRTH EXPECTED VALUES BY THE FIRST BIRTHDAY

Weight 3000 g 3x the birth weight ~ 9-10 kg

Length 50cm 50% increase from birth length ~75cm

Head ~ 45cm

I
35 cm (32-37 cm) Increase by 10 cm
Circumference
NavarroX, et al. Fundamentals
of PediatricsCompetency-Based
(1sted.).C & E Publishing;
2014

485
II. WEIGHT & LENGTH
A. Formulas to Estimate Weight

EXPECTED/ESTIMATED WEIGHT
AGE
PARAMETER FORMULA
:56 months Weight in grams Age in months x 600 + Birth Weight in grams
6 to 11 months Weight in grams Age in months x 500 + Birth Weight in grams
1 to 6 years Weight in kg Age in years x 2 + 8
(Age in years x 7) - 5
7 to 12 years Weight in kg
2

B. Expected Changes in Length/Height

AVERAGE GAIN in 1st Year is 25 cm, Distributed as Follows

AGE
Expected gain in length Average gain per month
Birth to 3 months +9 cm 3 cm per month
3-6months +8cm 2.67 cm per month
6-9 months + 5 cm 1.67 cm per month
9-12 months +3 cm 1 cm per month
2- 12 Years old Height in cm= Age in years x 6 + 77

C. Expected Changes in Length/Height

0 Aside from the above formula for length/height, some institutions also ask when a
child is expected to reach a certain height in clinical rounds and exams
AVERAGE EXPECTED HEAD CIRCUMFERENCE*
AGE
Expected increase in HC Expected Average increase in HC
1 to 4 months + 2 Inches 1/2 inch per month
4 to 12 months + 2 Inches 1/4 inch per month
1 to 3 years + 2 inches linch/year
3 to 6 years + 1.5 Inches 1/2 inch per year
6 to 20 years + 1.5 inches 1/2 inch per 5 years

Source:Del MundoF et al. Textbookof Pediatricsand ChildHealth.4th ed. JMC Press;2000

III. DEVELOPMENTAL MILESTONES

AGE SKILLS
2 months • Social smile
• Hold head without head lag
3 months
• Coo
5 months • Roll over
6months • Babble
7 months • Sit (6 months with support, 8 months without support)
• Pull himself/herself to stand
• Pincer grasp
9 months
• Say "mama/papa" but non-specific
• Understand and follow the command "no"
486
 AGE SKILLS
• Walk independently
12 months
• Follows one-step commands
• Run well, jump with both feet
24 months • Two-word phrase
• Follows two-step commands
• Ride a tricycle
3 years
• Draw circle
• Hop
4 years
• Draw square
• Skip
5 years
• Draw triangle
• Dress up completely
6 years
• Tie shoelaces

IV. SEXUALMATURITYRATINGFOR MALES

SMR PUBIC HAIR PENIS TESTES
1 • None • Preadolescent • Preadolescent
• Scanty, long • Enlarged scrotum
2 • Slight enlargement
• Slightly pigmented • Pink texture altered
• Darker
3 • Starts to curl • Longer • Larger
• Small amount
• Resembles adult type but • Larger
• Larger
4 less in quantity • Glans and breadth
• Scrotum dark
• Coarse, curly increases in size
• Adult distribution
5 • Spread to the medial • Adult • Adult
surface of thighs

V. SEXUALMATURITYRATINGFOR FEMALES
SMR
PUBIC HAIR BREASTS
STAGE
1 • None • Preadolescent
• Sparse, lightly pigmented • Breast and papilla elevated as small
2 • Straight mound
• Medial border of labia • Areolar diameter increased
• Darker
• Breast and areola enlarged
3 • Beginning to curl
• No contour separation
• Increased amount
• Coarse,curly • Areola and papilla form secondary
4
• Abundant but amount less than in adult mound
• Adult feminine triangle spread to the • Mature; nipple projects
5
medial surface of thighs • Areola part of general breast contour
Source:KliegmanR, et al NelsonTextbook
of Pediatrics
(21sted).Elsevier;2020
Source:NavarroX et al. Fundamentals
of Pediatrics
Competency-based (1sted).C & E Publishing;
2014

487
I
 AGE IN BIRTH WEEKS MONTHS YEARS
0 2 4 6 8 10 12 14 16 18 20 22 6 8 10 12 14 16 18 20 2 4 6 8 10 12 14 16 18
Legend:
Rangeof Recommended
Age ...___, I
BCG ecc
I
Hep B He1 B
Catchup immunization HepB' Hep e• I I I I
(OTwP-Hib-
I [ I Hep e• .. '
Hep B) and
j I I I I I I I
I
DTwP• /DTap• [
other DTaP
NOTE:Forthe latest combo j DTwP• /DTap• DTaP
OT•P.
Tdap/Td
ChildhoodImmunizationSchedule
(usuallyupdatedeveryFebruary) IPV/OPV
OPV*/l~V-
I I
IIII
OPV•/IPV
IPV
HIB
,PV

visitwww.philvaccine.org.
PCV
PCV.- T-T 1·-, I I I [
.j>.
co I- I I 1 rev~ I
PCV
-·
co RV

Influenza
RV Serles•
1-T 11
Influenza Yearly
I
I I
Measles

JE Vaccine
Measles
I I I I I I 11 JEV
IIIII I
I I I I I I I I I I I I I
MMR MMR MMR
·f I I I I I ··I· ; I I
Vari eel la
I
a,lc:ell• Varlcella

I I I I I
..
Hep A

HPV
I reTTe•, 1
'
I l
HPV Serles
' >
I
I I

Source:ChildhoodlmmunizalionScheduleby PediatriclnfecliousDiseaseSocietyof !he Philippines,PPPS,andPhilippineFoundalionfor Vaccination;

2019
 SECTION THREE
NEONATOlOGY
I. CARING FOR THE WELL NEWBORN

1 Immediate and thorough drying

2 Initial assessment (APGAR score)
3 Skin-to-skin contact
4 Properly timed cord clamping
5 Non-separation of mother and baby, early breastfeeding
6 Physical examination of the newborn, including Ballard's score
7 Eye prophylaxis, vitamin K, vaccination (Hepatitis B, BCG)
8 Bathing after 6 hours
Daily monitoring (weight, urine output, bowel movement, suck,
9
jaundice, other problems)
10 Discharge physical examination
11 Schedule for follow-up

II. APGAR SCORE (Evaluation of Newborn Infants)

SIGN 0 POINT 1 POINT 2 POINTS

A ctivity
(muscle tone) • Limp • Some Hexionof extremities • Active motion

Pulse
• Absent • < 100 bpm • > 100 bpm
(heart rate)
Grimace
• No response • Grimace • Cough or sneeze
(reflex irritability)

Appearance • Body pink,

(skin color) • Blue, pale • Completely pink
extremities blue
R espiratory effort • Absent • Slow, irregular • Good, crying
Interpretationof the APGARScore:
7-10points:goodcardiopulmonary adaptation,newbornwilldo well
4-6 points:need forresuscitation,especiallyventilatorysupport,& medicalintervention
0-3 points:need forimmediateresuscitationand mayneed NICUcare
Source:ApgarV.A Proposalfor a NewMelhodof Evaluationof the NewbornInfant.Anesthesia& Analgesia,1953

III. BALLARD SCORING SYSTEM

Maturity Rating Score (physical and neurologic scores are added)
Score Weeks Score Weeks
-10 20 25 34
-5 22 30 36
0 24 35 38
5 26 40 40
10 28 45 42
15
20
30
32
489
50 44
I
A. Physical Maturity

-1 0 1 2 3 4 5
Smooth, Superficial Parchment
Sticky. Gelatinous, Cracking, Leathery,
Pink peeling deep
SKIN friable, red, pale areas, cracked
visible and/or rash cracking
transparent translucent rare veins Wrinkled
veins few veins No vessels

LANUGO None Sparse Abundant Thinning Bald Areas Mostly Bald

Heel-toe Anterior Creases

PLANTAR >50mm, Faint red Creases on
40-S0mm: -1 transverse over entire
SURFACE no crease marks ant. 2/3
<40mm: -2 crease only sole

Stripped Raised
Barely Flat areola, Full areola,
BREAST Imperceptible areola, areaola.
perceptible no bud 5-10mm bud
1-2mm bud 3-4mm bud
Slightly Well-curved
Lids fused: Lids open, Formed and Thick
curved pinna, soft
EYE/EAR loosely (-1). pinna flat. firm, instant cartilage. ear
pinna: soft. but ready
tightly (-2) stays folded recoil stiff
slow recoil recoil
Scrotum Testes in Testes Testes Testes
GENITALS Scrotum flat.
empty, faint upper canal, descending. down, good pendulous.
MALE smooth
rugae rare rugae few rugae rugae deep rugrae
Prominent Prominent Majera and
Clitoris Majera Majera
GENITALS clitoris, clitoris. minora
prominent. large. cover clitoris
FEMALE small labia enlarging equally
labia flat minora small and minora
minora minora prminent

B. Neurological Maturity
-1 0 1 2 3 4 5
I
I
I
Posture Q¢):='. c¢::: c¢c ~ ~
Square
Window
(Wrist) r r r
>90° goo 60°
~
45°
~
30°
I
oo
I

I
Arm Recoil
~180°
iJ'
140-180°
i}-
110-140°
¾
90-110°
i}
<90°
I

I Popliteal
Angle ~ ~ ~ ~ ~ ~ ~
180° 160° 140° 120° 100° goo <90°

Scarf Sign
t ~ & ~ ~ § I
I
I
Heel to Ear Ge:::::3 ci=s CX3 CC9 CC9 cd
I -- -------· -----

490
 SECTION FOUR
FLUID AND ELECTROLYTES

FLUID & ELECTROLYTES

I. MAINTENANCEFLUID BASED ON BODY WEIGHT
A. Holiday-Segar Method (Weight Based)

WEIGHT MAINTENANCE THERAPY (FOR 24 HOURS)

0-10 kg • 100 mL/kg
11-20 kg • 1000 mL + 50 mL/kg for each kg in excess of 10 kg
>20kg • 1500 mL + 20 mL/kg for each kg in excess of 20 kg

HollidayM & Segar.W. Pediatrics:1957

B. Ludan Method (Basal Caloric Expenditure-based)

WEIGHT MAINTENANCE THERAPY FOR 24 HOURS

0-lOkg • 100 mL/kg
10-20 kg • 75 mL/kg
20-30 kg • 50-60 mL/kg
30-60 kg • 40-50 mL/kg
DelMundo,et al. Textbookof PediatricsandChildHealth.JMCPressInc: 1947

C. Crawford Formula (Body Surface Area or BSA-based)

Maintenance Therapy Body surface area (m 2 ):

(weight in kg X length in cm)

= 1500 ml/ BSA/ 24 hours
3600
CrawfordJ, et al. Pedratncs:1950

II. COMPUTATIONOF FLUID DEFICIT

Fluid Deficit(%) = Pre-illness weight-Admission weight x 100

Pre-illness weight

III. PRIMARYACID-BASEDISORDERS

pH 7.4 7.35-7.45 Acidosis Alkalosis

pco, 40mmHg 35-45 mmHg Alkalosis Acidosis
HCO3 24 mEq/L 20-28 mEq/L Acidosis Alkalosis

491
I
IV. COMPOSITION OF INTRAVENOUS FLUIDS & COMPARISON WITH PLASMA

Fluids

Plain Normal pNSS = 308 Isotonic'

Saline Solution 154 0 154
(pNSS)or DSNSS DSNSS = 560 Hypertonic
Plain 0.45 Plain 1/2 NS= 154 Hypotonic
NaCl (1/2 NS)" 77 0 77
orDS 1/2 NS D5 1/2 NS= 410 Hypertonic

D5 0.3 NaCl 51 0 51 355 Hypertonic

3%NaCI 513 0 513 1026 Hypertonic

Lactated Plain LR= 274 Isotonic

130 4 109
Ringer's (LR) OS LR= 525 Hypertonic
051MB 25 20 22 350 Hypertonic
DSNM 40 13 40 368 Hypertonic
Isotonic
Electrolyte 145 4 127 276 Isotonic
Solution (IES)
considered
.1 isotonicin varioussources
~½NS is notreadilyavailableandis usuallycustom-made.

KliegmanR, et al. Nelsontextbookof pediatrics(21sted.).Philadelphia:

Elsevier:2020
PhilippinePediatricSociety,Inc.Consensusstatements:2017
V. GUIDE FOR IVF SELECTION

CONDITION RECOMMENDED FLUID EXAMPLE

• 1/2 NS, LR, NSS, JES with or • Patient on NPO for surgical
General use
without dextrose and K· procedures
For volume • Hypovolemic shock
• pNSS, pLR, p!ES
resuscitation • Severe dehydration
Significant
electrolyte • Custom-made or cocktail fluids
derangement

VI. OSMOLALITY

Estimating ECF Osmolality

• Na·: in mmol/L
ECFosmolality = 2(Na) + G/ucose/18 +BUN/2.8 • Glucose and BUN: in mg/dL
• Normal value= 285-295 mOsm/kg
Corrected Sodium (Na•)

• RBS: in mg/dL
• For every increase of glucose by 100 mg/
Corrected Na·= Actual Na·+ 0.016 (RBS - 100)
dL above lOOmg/dL, Na+ is expected to
increase by 1.6 mEq/L
492
VII. COMMONLYUSED DRIPS

Concentration _ Volumeof drug (mL)x Preparationof drug (mg/mL) x 1000 (if desired dose is in mcg)
(mcg/mL) - Totalvolumeof preparation (mL)

Rate weight (kg) x desired dose (mcg/kg/min) x 60 (min/hour)

(mL/hour) Concentration(mcg/mL)

EASY PREP
DRUG CONCENTRATION
(amount of drug + USUAL DOSE
(in mcg/ml)
diluent)
5 mL of dopamine
Dopamine
+ 45 m L of diluent= 4000
200mg/5mL
50 mL solution • 5-20 mcg/kg/min
Dopamine pre-mixed
No need for diluent 800
200mg/250ml
20 mL of
Dobutamine • 2.5-15 mcg/kg/min
dobutamine 5000
250mg/20mL • Max:40 mcg/kg/min
+ 30 mL of diluent

Epinephrine 5 mL of epinephrine
250 • 0.1- 1 mcg/kg/min
lmg/mL + 15 mL of diluent

4mLof • Start at 0.05 - 0.1

Norepinephrine
norepinephrine 160 mcg/kg/min
4mg/4mL
+ 21 mL of diluent • Max: 2 mcg/kg/min
3 mL ofmidazolam
+ 47 mL of diluent
300
Midazolam
• 1-2 mcg/kg/min
5mg/mL 6 mL ofmidazolam
600
+ 44 mL of diluent

VIII. WHO CLASSIFICATIONOF DEHYDRATION

PARAMETER
~;::~:i;oN--
General Lethargic,
Alert Irritable
condition Unconscious
Eyes Normal Sunken Sunken
Drinks poorly, not
Thirst None Drinks eagerly able to drink
Skin
Quick Slow Very slow
retraction
Weight loss% <5% 5-10% >10%
Does not fulfill
2:2 of the 4 moderate 2:2 of the 4 severe
Criteria criteria for some or
signs signs
severe dehydration
Fluid deficit
ml/kg
<SO mL/kg 50-100 mL/kg

Source:WorldHealthOrganization.
493
>100 mL/kg

Thetreatmentof diarrhea.Geneva;2005
I
IX. WHO-CDCPROTOCOLON REHYDRATIONFOR DIARRHEA
PLAN REMARKS
• Fluids: Give oral rehydrating solution (ORS) after every loose stool
0 <2 years: 50-100 mL per loose stool
Plan A
0 2-10 years: 100-200 mL per loose stool
0 >10 years: as much as tolerated
PlanB • Fluids: Give 75 cc/kg ORS per orem over 4 hours
• Give a total of 100 cc/kg (PLR or PNSS) IV as follows:
Plane ° First 30 cc/kg over 1 hour for infants ( <12 months) or 30 mins for children
0 Next 70 cc/kg over 5 hours for infants (<12months) or 2.5 hours for children

X. BLOODTRANSFUSION
BLOOD PRODUCT DOSE
Packed red blood cells • 10-15 mL/kg over 4 hours
Platelet • 1 unit/10 kg or 4 units/BSA as fast drip
Fresh frozen plasma • 10-15 mL/kg as rapidly as tolerated

XI. COMPUTATIONAND INTERPRETATIONOF ESTIMATEDGFR (eGFR)

A. Modified Schwartz Formula (for patients> 1 year old)

Height in cm x 36.5
eGFR = Crea ti nine in mmol/l
OR
Height in cm x 0.413
eGFR = Creatinine in mg/dl

8. Pediatric-Modified RIFLE (pRIFLE) Criteria for Acute Kidney Injury

CREATININE eGFR Urine Output

Risk Increase >l.5x <0.5 ml/kg/hr

Decreased by 2:25%
for 2:8 hours

Injury Increase;;, 2x <0.5 ml/kg/hr

Decreased by 2:50%
for 2:16 hours
Increase 2:3x or <0.3 ml/kg/hr
Failure Decreased by 2:75% or
>4 mg/dL with an for 2:24 hours or
<35 ml/min/1.73111 2
acute rise of>0.5mg/dL anuric for 2:12 hours
Loss Persistent failure >4 weeks
End-stage Persistent failure >3 months
Source:KDIGOClinicalPracticeGuidelineforAcuteKidneyInjury.Kidneyinter.. Suppl:2012

C.Stages of Chronic Kidney Disease

STAGE DESCRIPTION GFR (ml/min/1.73m 2 )
1 Kidney damage with normal or increased GFR >90
2 Kidney damage with mild decrease in GFR 60-89
3 Moderate decrease in GFR 30-59
4 Severe decrease in GFR 5-29
5 Kidney failure <15 or on dialysis
Source:KDOQIclinicalpracticeguidelinesfor CKD:Am J Kidney:2002

494
 SECTION FIVE
MEASUREMENT OF COMMONLY USED EQUIPMENT
NASOGASTRIC TUBE: ESTIMATION OF TUBE SIZE (FORMULA)
(Patient's age+ 16)
Appropriate tube size (Fr)= 2

CONDITIONS INFLUENCING THE SELECTION OF NASOGASTRIC TUBE SIZE

EITHER AGE OR WEIGHT
NASOGASTRIC TUBE SIZE (Fr)
AGE WEIGHT(kg)
8 0-5 months 3-6
10 6-12 months 4-9
10-12 1-3 years 10-15
12 4-7 years 16- 20

FOLEY CATHETER
CONDITIONS INFLUENCING THE SELECTION OF FOLEY CATHETER SIZE
AGE WEIGHT(kg) FOLEY (Fr)
< 1.2 3.5 umbilical catheter
Neonate 1.2 - 1.5 5 umbilical catheter
1.5 - 2.5 5 umbilical catheter or size 6 Nelaton
0-6 months 3.5-7 6
1 year old 10 6-8
2 years old 12 8
3 years old 14 8-10
5 years old 18 10
6 years old 21 12
8 years old 27 12
12 years old Varies 12-14

ENDOTRACHEAL TUBE SIZE

I. FOR NEONATES
SIZE (unfcuffed) WEIGHT GESTATION

2.5mm < 1000 g < 28 weeks

3.0mm 1000to2000g 28 to 34 weeks

3.5mm 2000 to 3000 g 34 to 38 weeks

4.0mm > 3000 g > 38 weeks

II. FOR CHILDREN MORE THAN 1 YEAR OLD

Age (Years)+ 4
Size (uncuffed) =
4
*Subtract 0.5 "if using cuffed ET tube

495
Length=
Age (Years) + 12
2
I
 SECTION SIX
LABORATORY REFERENCE VALUES
The following section provides a guide for the normal reference values per age of common
laboratory examinations requested in pediatric patients.

COMPLETE BLOOD COUNT

ANALYTE AGE REFERENCE VALUE
0-30 days 15-24
1-23 months 10.5-14.0
2-9 years 11.5-14.5
Hemoglobin (g/dL) 10-17 years male 12.5-16.1
10-17 years female 12.0-15.0
18-99 years male 13.5-18.0
18-99 years female 12.5-16.0
0-30 days 44-70
1-23 months 32-42
2-9 years 33-43
Hematocrit (%) 10-17 years male 36-47
10-17 years female 35-45
18-99 years male 42-52
18-99 years female 37-47
0-30 days 9.1-34
1-23 months 6-14
Leukocyte Count
[x 1,000cells/mm 3 (uL)] 2-9 years 4-12
10-17 years 4-10.5
18-99 years 4-10.5
Differential Counts %
Myelocyte 0
Neutrophils (bands) 3-5
Neutrophils (segmenters) 54-62
Lymphocytes 25-33
Monocytes 3-7
Eosinophils 1-3
Basophils 0-0.75
Differential Counts [cells/mm 3 (uL)]
Myelocyte 0
Neutrophils (bands) 150-400
Neutrophils (segmenters) 3,000-5,800
Lymphocytes 1,500-3,000
Monocytes 285-500
Eosinophils 50-250
Basophils 15-50

496
 ANALYTE AGE REFERENCE VALUE

Platelet Count Newborn (<l week) 84-478

(x10 3 /mm 3 (uL)) Infants to adult 150-400
0-30 days 99-115
1-23 months 72-88
Mean Corpuscular Volume
(MCV) (um 3 ) 2-9 years 76-90
10-17 years 78-95
18-99 years 78-100
0-30 days 33-39
1-23 months 24-30
Mean Corpuscular 2-9 years 25-31
Hemoglobin (MCH)
10-17 years male 26-32
(pg/cell)
10-17 years female 26-32
18-99 years male 27-31
18-99 years female 27-31
Mean Corpuscular Hemoglobin Concentration (MCHC) 32-36
(% Hb/cell or g Hb/dL)

BLOOD CHEMISTRY
I. CHEMISTRY GENERALLY USED IN NEPHR0L0GY

REFERENCE CONVENTIONAL
AGE SI UNITS
VALUES UNITS
Cord Blood 70-380 U/L 70-380 U/L
5-8 hours 214-1,175 U/L 214-1,175 U/L
Creatine kinase 24-33 hours 130-1,200 U/L 130-1,200 U/L
72-100 hours 87-725 U/L 87-725 U/L
Adult 5-130 U/L 5-130 U/L
0-4 years 0.03-0.50 mg/dL 2.65-44.2 umol/L
4-7 years 0.03-0.59 mg/dL 2.65-52.2 umol/L
Creatinine
7-10 years 0.22-0.59 mg/dL 19.4-52.2 umol/L
enzymatic
10-14 years 0.31-0.88 mg/dL 27.4-77.8 umol/L
>14 years 0.50-1.06 mg/dL 44.2-93.7 umol/L
Cord blood 21-40 mg/dL 7.5-14.3 mmol/L
Premature (1 week) 3-25 mg/dL 1.1-9.0 mmol/L
Urea Nitrogen Newborn 3-12 mg/dL 1.1-4.3 mmol/L
Infant or child 5-18 mg/dL 1.8-6.4 mmol/L
Thereafter

497
7-18 mg/dL 2.5-6.4 mmolfL

I
 REFERENCE CONVENTIONAL
AGE SI UNITS
VALUES UNITS
Cord Blood 5.0-6.0 mg/dL 1.25-1.50 mmol/L
Newborn 3-24 hours 4.3-5.1 mg/dL 1.07-1.27 mmol/L
Calcium, Ionized
24-48 hours 4.0-4.7 mg/dL 1.00-1.17 mmol/L
Thereafter 4.8-4.92 mg/dL 1.12-1.23 mmol/L
Cord Blood 9.0-11.5 mg/dL 2.25-2.88 mmol/L
Newborn 3-24 hours 9.0-10.6 mg/dL 2.3-2.65 mmol/L
24-48 hours 7.0-12.0 mg/dL 1.75-3.00 mmol/L
Calcium, Total
4-7 days 9.0-10.9 mg/dL 2.25-2.73 mmol/L
Child 8.8-10.8 mg/dL 2.2-2.7 mmol/L
Thereafter 8.4-10.2 mg/dL 2.1-2.55 mmol/L
0-6 days 1.2-2.6 mg/dL 0.48-1.05 mmol/L
Magnesium 7 days - 2 years 1.6-2.6 mg/dL 0.65-1.05 mmol/L
2-14 years 1.5-2.3 mg/dL 0.60-0.95 mmol/L
0-5 days 4.8-8.2 mg/dL 1.55-2.65 mmol/L
1-3 years 3.8-6.5 mg/dL 1.25-2.1 mmol/L
Phosphorus,
Inorganic 4-11 years 3.7-5.6 mg/dL 1.20-1.80 mmol/L
12-15 years 2.9-5.4 mg/dL 0.95-1.75 mmol/L
16-19 years 2.7-4.7 mg/dL 0.90-1.50 mmol/L
0-1 week 3.2-5.5 mmol/L 3.2-5.5 mmol/L
1 week - 1 month 3.4-6.0 mmol/L 3.4-6.0 mmol/L
Potassium 1-6 months 3.5-5.6 mmol/L 3.5-5.6 mmol/L
6 months - 1 year 3.5-6.1 mmol/L 3.5-6.1 mmol/L
>l year 3.3-4.6 mmol/L 3.3-4.6 mmol/L
Newborn 133-146 mmol/L 133-146 mmol/L
Infant 134-144 mmol/L 134-144 mmol/L
Sodium
Child 134-143 mmol/L 134-143 mmol/L
Therafter 135-145 mmol/L 135-145 mmol/L
1-3 years 1.8-5.0 mg/dL 100-300 umol/L
4-6 years 2.2-4.7 mg/dL 130-280 umol/L
7-9 years 2.0-5.0 mg/dL 120-295 umol/L

10-11 years M: 2.3-5.4 mg/dL M: 135-320 umol/L

F: 3.0-4.7 mg/dL F: 180-280 umol/L
Uric Acid
12-13 years male 2.7-6.7 mg/dL 160-400 umoljL
14-15 years male 2.4-7.8 mg/dL 140-465 umol/L
12-15 years female 3.0-5.8 mg/dL 180-345 umol/L

16-19 years M: 4.0-8.6 mg/dL M: 235-510 umol/L

F: 3.0-5.9 mg/dL F: 180-350 umol/L

498
II. CHEMISTRY GENERALLY USED IN GASTROENTEROLOGY
CONVENTIONAL SI
REFERENCE
AGE UNITS UNITS
VALUES
MALES FEMALES MALES FEMALES
0-7 days 6-40 U/L 6-40 U/L
Alanine
amino- 8-30 days 10-40 U/L 8-32 U/L 10-40 U/L 8-32 U/L
transferase 1-12 months 12-45 U/L 12-45 U/L
(ALT/SGPT)
1-19 years 5-45 U/L 5-45 U/L
Premature 1 day 1.8-3 g/dL 18-30 g/L
Fullterm <6 days 2.5-3.4 g/dL 25-34 g/L
Albumin 8 days to 1 year 1.9-4.9 g/dL 19-49 g/L
1-3 years 3.4-4.2 g/dL 34-42 g/L
4-19 years 3.5-5.6 g/dL 35-56 g/L
1-9 years 145- 420 U/L 145- 420 U/L
10-11 years 140-560 U/L 140-560 U/L
Alkaline 12-13 years 200-495 U/L 105-420 U/L 200-495 U/L 105-420 U/L
phospahatase
14-15 years 130-525 U/L 70-230 U/L 130-525 U/L 70-230 U/L
16-19 years 65-260 U/L 50-130 U/L 65-260 U/L 50-130 U/L
Ammonia 11-35 umol/L 11-35 umol/L
Amylase 1-19 years 30-100 U/L 30-100 U/L
0-7 days 30-100 U/L 24-95 U/L 30-100 U/L 24-95 U/L
8-30 days 22-71 U/L 22-71 U/L

Aspartate 1-12 months 22-63 U/L 22-63 U/L

amino-
1-3 years 20-60 U/L 20-60 U/L
transferase
(AST/SGOT) 3-9 years 15-50 U/L 15-50 U/L
10-15 years 10-40 U/L 10-40 U/L
16-19 years 15-45 U/L 5-30 U/L 15-45 U/L 5-30 U/L
Lipase 1-18 years 145-216 U/L 145-216 U/L

499
I
III. CHEMISTRY GENERALLY USED IN ENDOCRINOLOGY
REFERENCE CONVENTIONAL SI UNITS
AGE
VALUES UNITS
Cord blood 45-96 mg/dL 2.5-5.3 mmol/L
Premature 20-60 mg/dL 1.1-3.3 mmol/L
Neonate 30-60 mg/dL 1.7-3.3 mmol/L
Glucose (serum) Newborn 1 day 40-60 mg/dL 2.2-3.3 mmol/L
Newborn >l day 50-90 mg/dL 2.8-5.0 11111101/L
Child 60-100 mg/dL 3.3-5.5 mmol/L
Adult 70-105 mg/dL 3.9-5.8 mmol/L
0-3 days 1.00-20.00 uIU/L 1.00-20.00 ulU/L
Thyroid 3-30 days 0.5-6.5 ulU/L 0.5-6.5 ulU/L
Stimulating
Hormone (TSH) 1-5 months 0.5-6.0 u!U/L 0.5-6.0 uIU/L
6 months - 18 years 0.5-4.5 ulU/L 0.5-4.5 ulU/L
0-3 days 2.00-5.00 ng/dL 25.7-64.3 pmol/L
Thyroxine, free 3-30 days 0.90-2.20 ng/dL 11.6-28.3 pmol/L
31 days -18 years 0.7-2.00 ng/dL 9.0-25.7 pmol/L
Cord blood 20-240 pg/dL 0.3-3.7 pmol/L
1-3 days 200-610 pg/dL 3.1-9.4 pmol/L
Triodothyronine,
free 6 weeks 240-560 pg/dL 3.7-8.6 pmol/L

Adult (20-50 years) 230-660 pg/dL 3.5-10.0 pmol/L

IV. HEMATOLOGY& OTHER INFLAMMATORYMARKERS

Inflammatory markers
REFERENCE VALUES AGE UNIT
2-5 years < 120-160 Todd units
Antistreptolysin- 0 (ASO)
6-9 years < 240 Todd units
Titer
10-12 years < 320 Todd units

C-Reactive Protein 0-0.5 mg/di

Child 0-10 mm/hr
Erythrocyte
Adult male 0-15 mm/hr
Sedementation Rate
Adult female 0-20 mm/hr
<l year 170-580 U/L
Lactate Dehydrogenase 1-9 years 150-500 U/L
10-19 years 120-330 U/L

500
Evaluation of anemia
CONVENTIONAL SI
REFERENCE UNITS UNITS
AGE
VALUES
MALES FEMALES MALES FEMALES
0-6 weeks 0-400 ng/mL 0-400 ug/L
7 weeks to
10-95 ng/mL 10-95 ug/L
<1 year
Ferritin
1-9 years 10-60 ng/mL 10-60 ug/L

Iron
10-18 years
10-300
ng/mL I
22-184 ug/dL
10-70
ng/mL
10-300
ug/L I
4-33 umol/L
10-70
ug/L

Total Iron Infant 100-400 ug/dL 17.90-71.6 umoI/L

Binding
Capacity Thereafter 250-400 ug/dL 44.75-71.6 umol/L

Source:KliegmanR, et al. NelsonTextbookof Pediatrics(21sted.).Elsevier;2020

HughesHK,et al. TheHarrietLaneHandbook (21sted.).Philadelphia:2018

REFERENCES
l. Bloomfield, D., and Adam, H. Tachypnea. Pediatrics In Review, 2002, 23(8), 294-295. doi:
10.1542/pir.23-8-294
2. Crawford, J., Terry, M., and Rourke, G. Simplification of drug dosage calculation by application
of the surface area principle. Pediatrics, 1950, 5(783)
3. Del Mundo Fetal. Textbook of Pediatrics and Child Health. 4th ed. 2000. )MC Press,166.
4. Engorn, B., and Flerlage, J. The Harriet Lane Handbook 20th ed. 2015. Philadelphia: Elsevier.
5. Flynn JT, Kaelber DC, Baker-Smith CM, et al; Subcommittee on Screening and Management of
high blood pressure in children. Clinical Practice Guideline for Screening and Management of
High Blood Pressure in Children and Adolescents. Pediatrics. 2017; 140(3):e20171904.
6. Hazinski, MF. Manual of Pediatric Critical Care 1st ed. 2009. Elsevier.
7. Hazinski, MF. Nursing Care of the Critically Ill Child 3rd ed. 2013. Elsevier.
8. Mwangome, M., Fegan, G., Fulford, T.,Prentice, A., and Berkley, J. Mid-upper arm circumference
at age of routine infant vaccination to identify infants at elevated risk of death: a retrospective
cohort study in the Gambia. Bulletin Of The World Health Organization, 2012, 90(12), 887-
894. doi: 10.2471/blt.12.109009
9. Kliegman, R., Stanton, B., St. Geme, )., Schm; N., and Behrman, R.. Nelson Textbook of Pediatrics
21st ed. 2020. Canada: Elsevier.
10.Navarro, X., Bauzon, A., Aguilar, )., and Malanyaon, 0. (2014). Fundamentals of Pediatrics
Competency-based (1st ed.). Quezon City: C and E Publishing, Inc.
11.Parthasarathy A, Menon PSN, Agarwal RN, Choudhury P, Thacker NC, Ugra D (2009). IAP
textbook of pediatrics (4th ed.). New Delhi: Jaypee Brothers
12.The Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in
Children and Adolescents. PEDIATRICS, 2004, 114(2), 555-576. doi: 10.1542/
peds.114.2.sZ.555
13.The WHO Child Growth Standards. Retrieved from http://www.who.int/childgrowth/
standards/en/
14.World Health Organization. Management of severe acute malnutrition in infants and children.
Available from https://www.who.int/elena/titles/full_recommendations/sam_management/en/
15.World Health Organization. Supplementary foods for the management of moderate acute
malnutrition in children aged 6-59 months. Available from https://www.who.int/elena/
titles /food_child ren_mam/ en/
16.World Health Organization. Training Course on Child Growth Assessment. Geneva, WHO, 2008.

501
I
502
 CORRELATES
BOARD
FLUIDS, ECG, ABG, CHEST RADIOGRAPHS

BASIC BEDSIDE PROCEDURES IN PEDIATRICS

PREVENTIVE PEDIATRICS

NEONATOLOGY

INFECTIOUS DISEASES

GASTROENTEROLOGY

HEMATOLOGY AND ONCOLOGY

PULMONOLOGY

IMMUNOLOGY

RHEUMATOLOGY

CARDIOLOGY

NEPHROLOGY

ENDOCRINOLOGY

NEUROLOGY

ACUTE CARE
FLUIDS, ECG, ABG, AND CHEST RADIOGRAPHS: MOST COMMON
Cause of volume depletion
Cause ofrenal Mg'• loss
Cause of metabolic acidosis
Cause of metabolic alkalosis
Presenting complaint of
chronic metabolic acidosis
Evaluating a pediatric ECG
Right atrial enlargement
Left atrial enlargement
T waves in ECG
Basic steps in ABG evaluation
Cardiothoracic ratio (CTR) in
chest X ray
Signs of pulmonary
congestion in chest X ray
Causes of tracheal deviation
as seen in chest X ray
BASIC BEDSIDE PROCEDURES IN PEDIATRICS
Insertion of Foley catheter
Orogastric tube insertion
Selection of nasogastric tube
size
lntraosseous (IO) infusion
Landmark of IO insertion in
the proximal tibia
Umbilical vessel
catheterization
Lumbar puncture
Site of needle insertion
to relieve tension
pneumothorax and primary
spontaneous pneumothorax
Endotracheal tube size
• Gastroenteritis
• Gitelman and Bartter syndromes
• Diarrhea
• Emesis or diuretic use
• Failure to thrive
• Determine the rhythm, rate, axis, check P wave & PR
interval, QRScomplex, QT,and ST & T wave abnormalities
• Tall, peaked P waves ;,3 mm in lead II
• Wide and notched P waves with duration of>0.10
seconds in children and >0.08 seconds in infants
• Peaked T waves in hyperkalemia and LVH; flat or low T
waves in hypokalemia, hypothyroidism, & myocarditis
• Determine primary acid base disorde1; compensation,
secondary disorde1; anion gap, and oxygenation status
• Normal CTR in infants: 0.6
• Normal CTR in children and adolescents: 0.5
• Cephalization of blood vessels, cardiomegaly, alveolar
edema, and Kerley B lines
• If pushed contralaterally: pleural effusion or
pneumothorax
• If pushed ipsilaterally: atelectasis
• Insert the Foley catheter into the urethral opening until
the "Y" of the catheter
• OGT is used for neonates because they are preferential
nasal breathers
• Appropriate tube size (Fr)= patient's age+ 16 / 2
• If IO needle or device is not available, an alternative
would be a regular gauge 16 or 18 needle
• Flat surface of the tibia is about 1-2 cm below and
slightly medial to the tibial tuberosity
• For less than 3.5 kgs: Fr 5
• For more than 3.5 kgs: Fr 8
• Insert needle in the interspace between the dorsal
processes of vertebrae L4-LS
• Second intercostal space superior to the 3"' rib at the
midclavicular line
• Size (uncuffed) = (age in yeus / 4) + 4
505
I
PREVENTIVE PEDIATRICS

• Complete sexual maturity usually achieved

Sexual maturity rating in boys
at17-18yearsofage
• Full fertility usually reached at an average
Sexual maturity rating in girls
of14-15 years of age
Most common solid breast mass seen in
• Fibroadenoma
adolescent girls
Most common causes of breast pain in
• Exercise and benign breast changes
adolescents
Most common cause of secondary • Endometriosis (usually in the fallopian
dysmenorrhea in adolescents tubes and ovaries)
Most common cause of pelvic pain in
adolescents • Primary dysmenorrhea

Most common diagnosis when adolescent

presents with secondary amenorrhea • Pregnancy

Most common form of rape for victims • Acquaintance rape (by a person known to
16-24 years of age the victim)
• Anaphylactic reaction and encephalopathy
Permanent contraindications to
vaccination not due to another cause occurring within
7 days after pertussis vaccination
Vaccines given at birth • BCG& the 1" dose of hepatitis B vaccine
Prophylactic drug of choice for animal
bites category III • Co-amoxiclav for 7 days

• Done at the time of eruption of the first

First dental visit
tooth and not later than 12 months of age
Recommended age of deworming of • All children 1-12 years of age using
children in the Philippines Albendazole or Mebendazole eve1y 6 months
• Picture tests/ HOTV/ Tumbling E chart
Visual acuity tests in children for 3-5 years old
• Snellen chart for 6 years old and above
Visual acuity in children • 20/20 achieved by 5-6 years old
• Ideally done in newborns after 24 hours
Hearing screening test
from birth by ABR or OAE
• Reliable growth indicator even when the
Weight-for-length /height
age is not known
• Allows early detection and prevention of
Body mass index
overweight and obesity problems
• Encouraged to exclusively breastfeed up
to 6 months & continued up to 2 years &
Feeding of infants
beyond with complementary feeding to
begin at 6 months old
• Vaginal wet mount
Screening tests for sexually active
females • PAP smear
• Non-culture test for gonorrhea & Chlamydia
• Serologic test for syphilis
Screening tests for sexually active males
• Non-culture test for gonorrhea & Chlamydia

506
 • Recommended to be done at 9 months,
Developmental screening tests 18 months, 30 months old and every year
thereafter
Most common neurobehavioral disorder
of childhood • Attention-deficit/hyperactivity disorder

Best prognostic factors for autism • Language impairment and presence or

spectrum disorder outcome absence of intellectual disability
Most common learning disability • Dyslexia
Most common method of attempted
suicide • Ingestion of medication

Most common preexisting psychiatric

illness in those who complete suicide • Major depression

• RA 7610 - Special Protection of Children

Legal framework of child abuse in the
Against Abuse, Exploitation, and
Philippines
Discrimination Act
Most common manifestation of physical
• Bruise
abuse
Most common fracture in abused infants • Rib, metaphyseal, skull

NEONATOLOGY: MOST COMMON

Cause of aneuploidy • Meiotic nondisjunction

Form ofaneuploidy • Trisomy
• Trisomy 21 (Down syndrome)
• Trisomy 18 (Edwards syndrome)
• T,·isomy 13 (Patau syndrome)
Numerical abnormalities in liveborn • Sex chromosomal aneuploidies:
children
Turner syndrome (usually 45,X)
0

° Klinefelter syndrome (47,XXY;47,XXX;

and 47,XYY)
Type of trisomy in live born infants • Trisomy 21 (47,XX,+21 or 47,XY,+21)
Genetic cause of moderate intellectual
• Trisomy 21
disability
Autosomal aneuploidy in spontaneous
• Trisomy 16 (47,XX/XY,+16)
abortion
Most common sex chromosome
• Klinefelter syndrome
aneuploidy in humans
Cause of oligohydramnios • Rupture of the membranes
Life-threatening emergency of the
gastrointestinal tract in the newborn • Necrotizing Enterocolitis
period
Cause of hemolytic disease of the • ABO incompatibility
newborn
Eye disease of newborns • Ophthalmia neonatorum

507
I
.
INFECTIOUS DISEASES· MOST COMMON
Source of postnatal infections in
hospitalized newborns
Bacterial causes of neonatal meningitis
Cause of neonatal sepsis
Most common bacteria in intrapartum
and postpartum infections
Most common viruses in intrapartum
and postpartum infections
Cause of hospital acquired infection,
particularly in neonatal units
Vaccine-preventable disease occurring
among pediatric and adult travelers
Health complaint among international
travelers
Connective tissue diseases associated
most commonly with FUO
Causative organisms associated
defective splenic function
Organisms involved in catheter-
associated skin & soft tissue Infection
Clinical presentation of Catheter-
Related Bloodstream Infection (CRBSI)
Species of CoNSon skin & mucus membranes
Cause of nosocomial bacteremia,
usually in association with central
vascular catheters
Pathogen associated with CSF shunt
meningitis
Cause of bone and joint infections
Cause of pyogenic infection of the skin
and soft tissues
Site oflmpetigo
Lesions that precede nonbullous
impetigo
Etiologic agents in cellulitis
Cause of bacterial pharyngitis
Anatomic site of Corynebacterium
diphtheriae infection
Cause of bacterial otitis media
Cause of external otitis (also called
swimmer's ear although it can occur
without swimming)
Causative organism of necrotizing
externa
• Hand contamination of personnel
• GBS,£. coli, and L. monocytogenes
• Group B streptococcus
• GBS and£. coli
• CMV,HSV,enteroviruses, and HIV
• Coagulase-negative staphylococci (CoNS)
• Influenza
• Traveler's diarrhea (from ingestion of
contaminated food or water)
• Juvenile idiopathic arthritis (]IA) and
systemic lupus erythematosus (SLE)
with • S. pneumoniae, H. influenzae type b, and
Salmonella
• S. aureus, CoNS, P aeruginosa, Candida
spp., and mycobacteria
• Fever without an identifiable focus
• S. epidermidis
• S. epidermidis
• CoNS
• Staphylococcus aureus
• Staphylococcus aureus
• Face and extremities
• Insect bites, abrasions, lacerations,
chickenpox, scabies pediculosis, and burns
• Streptococcus pyogenes (group A
streptococcus) and S. aureus
• Group A streptococcus
• Tonsils or pharynx
• H. influenzae, S. pneumoniae, Moraxel/a
catarrhalis
• P aeruginosa
otitis
• P aeruginosa
508
Isolated organism in perichondritis
chondritis
Etiologic organisms in chronic
suppurative otitis media (CSOM)
Infectious cause of congenital
sensorineural hearing loss (SNHL)
Cause of bacterial meningitis that
results in SNHL
Cause of bacterial meningitis in
children older than 1 month of age
Mechanism of bacterial meningitis
Neurologic sequelae of bacterial
meningitis
Cause of viral meningoencephalitis
Most common cause of eosinophilic
meningitis in Southeast Asia
Age group associated with brain
abscesses
Exotoxin associated with scarlet fever
Population group associated with
retropharyngealabscess
Clinical manifestation ofmeningococcal
infection
Sexually transmitted infection found in
sexually-abused children
Initial symptom of Disseminated
Gonococcal Infection
Enteropathogens
Implicated food in Salmonella infections
Clinical presentation of salmonellosis
Mode of transmission of Typhoid Fever
Age group affected in Shigella infections
Cause of bacillary dysentery in
industrialized societies
Cause of bacillary dysentery in pre-
industrialized ~ocieties
Complication of shigellosis
Surgical complication of shigellosis
Extraintestinal manifestations of
bacillary dysentery
Cause of traveler's diarrhea
and
• P aeruginosa
• P aeruginosa and S. aureus
• CMV
• Streptococcus pneumoniae
• Streptococcus pneumoniae and Neisseria
meningitides
• Hematogenous dissemination
• Hearing loss, cognitive impairment,
recurrent seizures, delay in acquisition
of language, visual impairment, and
behavioral problems
• Enteroviruses
• Human infection with the rat lungworm
Angiostrongylus cantonensis
• Children between 4 and 8 year old and in
neonates
• Streptococcal pyrogenic exotoxin A
• Children younger than 3-4 years of age
• Asymptomatic carriage of the organism in
the nasopharynx
• Gonorrhea
• Acute onset of polyarthralgia with fever
• Rotavirus and G. lamb/ia
• Eggs
• Acute enteritis
• Ingestion of food or water contaminated
with S. typhi from human feces
• <S years old (especially 2-3 years old)
• Shigel/a sonnei
• Shige//a flexneri
• Dehydration
• Intestinal obstruction and appendicitis,
with or without perforation
• Neurologic findings
• Enterotoxigenic Escherichia coli (ETEC)
509
I
Most common serotypes of Shiga Toxin-
Producing Escherichia coli (STEC)
Mode of transmission ofCampylobacter
infections
Manifestation ofCampylobacter
infections
Late-onset complications of
Campylobacter infections
Yersinia species causing human disease
Mode of transmission ofY.
enterocolitica
Form of tetanus
Age group with TB among nonwhite
populations
Form of reactivation TB
Symptoms of primary TB
Form of cardiac tuberculosis
Age group associated with military TB
Signs and symptoms of middle ear TB
Form of extra pulmonary TB in children
Findings in TB meningitis
Age group in TB meningitis
Symptoms of genital tract TB
Sequelae of female genital tract TB
Organism associated with oral thrush
Opportunistic fungal infections in
patients with prolonged neutropenia
Cause of invasive candidiasis among
immunocompromised pediatric
patients
Cause of esophagi tis in HIV patients
Infection caused by Candida
Infection caused by Candida in HIV-
infected children
Pathogenic fungal infection among
persons infected with HIV
Form of cryptococcosis
Symptom of cryptococcosis
510
• E.coli 0157:H7, E.coli 0111:NM, and
E.coli 026:Hll
• Ingestion of contaminated poultry
(chicken, turkey) or raw milk
• Gastroenteritis
• Reactive arthritis (especially those HLA-B27
positive) & Guillain-Barre syndrome
• Yersinia enteroco/itica
• Consumption of contaminated water or
food, especially undercooked pork
• Neonatal (or umbilical) tetanus
• Young adults and children younger than 5
years of age
• Infiltrate or cavity in the apex of the upper
lobes
• Nonproductive cough & mild dyspnea
• Pericarditis
• Infants and young children
• Painless unilateral otorrhea, tinnitus,
decreased hearing, facial paralysis, and a
perforated tympanic membrane
• Scrofula (TB of superficial lymph nodes)
• Basilar enhancement and communicating
hydrocephalus with signs of cerebral
edema or early focal ischemia
• Children between 6 months and 4 years
of age
• Lower abdominal pain and dysmenorrhea
or amenorrhea
• Infertility
• C.albicans
• Candida spp. and Aspergillus spp.
• C.albicans
• C.albicans
• Diaper dermatitis
• Oral thrush and diaper dermatitis
• Cryptococcus neoformans var. neoformans
• Pneumonia
• Cough
Predisposing factor for disseminated
cryptococcosis • Advanced HIV infection

Clinical manifestation of disseminated

cryptococcal infection • Subacute or chronic meningitis

Organism causing fungemia • Malassezia fwfur

Aspergilli species that causes invasive
disease • Aspergillus Jumigatt1s

Form of aspergillosis • Invasive pulmonary aspergillosis

Cause of death in measles • Pneumonia
Causes of superimposed bacterial • Streptococws pneumoniae, Haemophilus
infection in measles influenzae, and Staphylococcus attreus
Complication of measles • Acute otitis media
Finding among infants with Congenital
Rubella Syndrome (CRS) • Nerve deafness

Ocular abnormality but have little early

effect on vision among infants with CRS • Salt-and-pepper retinopathy

Long-term sequela associated with

congenital CMVinfection • Hearing loss

Route of CMVinfection in early

childhood • Breastfeeding

• Meningitis, with or without encephalitis,

Complications of mumps
and gonadal involvement
Manifestation ofparvovirus 819 • Erythema infectiosum (fifth disease)
Manifestation of Parvovirus Bl 9
infection in patients with impaired • Chronic anemia
humoral immunity
Complication of roseola • Convulsions
Agent in hand-foot-mouth disease • Coxsackie A16
Mode of transmission ofHSV-1 • Contact with contaminated oral secretions
Mode of transmission of HSV-2 • Anogenital contact
Manifestation of recurrent HSV-1
• Fever blisters (cold sores)
infections
Cause of recurrent aseptic meningitis
• HSV
(Mollaret meningitis)
Organisms associated with genital ulcer
• HSV and Treponema pa/lidum (syphilis)
syndromes
Identified pathogens in urethritis
associated with sexually transmitted • Chlamydia trachomatis and N. gonorrhoeae
infections (STI)
• C. trachomatis and N. gonorrhoeae (no
Identified pathogens in cervicitis
pathogen identified in majority of cases)

Found in genital warts and are rarely

• Low-risk HPV types 6 and 11
found isolated in malignant lesions
HPV types associated with cervical
• HPV types 16 and 18
cancer
Cause of common warts (verruca vulgaris) • HPV Types 2 and 4

511
I

Hematologic abnormalities during
dengue hemorrhagic fever and dengue
shock syndrome
Viral causes of meningitis
Opportunistic infection among HIV-
infected children
CXRfindings in Pneumocystis
pneumonia
Bacterial pneumonia in HIV-infected
children
Finding in amebic liver abscess
Means of infection in amebiasis
Human helminthiasis in the world
Clinical problems with ascariasis
Cause of human hookworm infection
Cause of cutaneous larva migrans
(creeping eruption)
Complaint in pinworm infection
Dermatophyte pathogen
Form of lice to affect children
GASTROENTEROLOGY:MOSTCOMMON
Esophageal disorder in children of all ages
Congenital anomaly of the esophagus
Form ofEA
Encountered foregut duplications
Location for peptic strictures
Type of esophageal hiatal hernia
Oral lesion of GERO
Ingested foreign bodies
Ingested caustic substance
Clinical association of pyloric stenos is
Location of gastric duplications
512
• Hemoconcentration (increase of >20% in
hematocrit)
• Thrombocytopenia
• Prolonged bleeding time
• Moderately decreased prothrombin level
• Enteroviruses (coxsackieviruses,
echoviruses)
• Arboviruses (La Crosse encephalitis virus
and equine encephalitis virus)
• Pneumocystis jiroveci (formerly
Pneumocystis carinii) pneumonia
• Interstitial infiltrates or diffuse alveolar
disease which rapidly progresses
• S. pneumoniae
• Single abscess in the right hepatic lobe
• Food or drink contaminated with
Entamoeba cysts and oral-anogenital sex
• Ascariasis (most common in tropical areas)
• Pulmonary disease and obstruction of the
intestinal or biliary tract
• Necator americanus
• Ancylostoma braziliense
• Itching and restless sleep secondary to
nocturnal perianal or perinea! pruritus
• Trichophyton rubrum
• Pediculosis capitis
• Gast1·oesophageal Reflux Disease (GERO)
• Esophageal atresia (EA)
• Type C (upper esophagus ends in blind pouch
& TEF is connected to distal esophagus)
• Esophageal duplication cysts
• Lower esophagus
• Sliding hiatal hernia
• Dental erosions
• Coins and small toy items
• Drain decloggers (especially since they are
tasteless unlike acidic agents)
• Hyperbilirubinemia
• Greater curvature of stomach
Surgical approach in gastric volvulus • Laparoscopic gastropexy
Symptom ofmalrotation in 1" year of life • Vomiting
• Failure of the cecum to move into the right
Type of malrotation
lower quadrant
Congenital anomaly of the GI tract • Meckel diverticulum
Cause oflower intestinal obstruction in • Congenital Aganglionic Megacolon
neonates (Hirschsprung Disease)
Cause of intestinal obstruction between
5 months & 3 years and most common • lntussusception
abdominal emergency in children <2 years
Time of onset of inflammatory bowel • Preadolescence, adolescence, young
disease adulthood
Hepatobiliary disease associated with IBD • Sclerosing cholangitis
Cause of exocrine pancreatic
• Cystic fibrosis
insufficiency in children
Form of malignant lymphomas of the
• Burkitt lymphoma
small intestines
Manifestations of gastroenteritis • Diarrhea and vomiting
Identifiable viral cause of
• Rotavirus infection
gastroenteritis in all children
Acute surgical condition in children and
• Acute appendicitis
a major cause of childhood morbidity
Misdiagnosis in child with appendicitis • Gastroenteritis
Cause of small bowel obstruction
in children without history of prior • "Missed" appendicitis
abdominal surgery
• Wound infections and intraabdominal
Complications of appendicitis
abscesses
Intestinal tumors of childhood • Hamartomatous polyps
Genetic polyposis syndrome • Familial adenomatous polyposis (FAP)
Primary GI carcinoma in children • Colorectal carcinoma
GI malignancy in the pediatric • Lymphoma (usually in the distal small
population bowel and ileocecal region)
Pancreatic disorder in children • Acute pancreatitis
• Palpable mass in 50%
Signs of pancreatic pseudocyst
• jaundice in 10%
Pancreatic neoplasm in young children • Pancreatoblastoma

Causes of cholestasis in early infancy • Biliary atresia and neonatal hepatitis

Syndrome with intrahepatic bile duct • Alagille syndrome (arteriohepatic

paucity dysplasia)
• Obliteration of the entire extrahepatic
Form of biliary atresia
biliary tree at or above the porta hepatis

Indication for liver transplantation in

• Biliary atresia
children

I
Transplantation-related infections • CMVand EBV infections
513
Cause ofacute hepatitis and jaundice
in the world; increased morbidity and
mortality in pregnancy
Chronic liver disease in children
Presentation of portal hypertension
Causes of ascites
Cause of chylous ascites
Presentation of chylous ascites
Cause ofperiappendiceal and pelvic
abscesses
Cause of acute secondary peritonitis
Micronutrient deficiency
Reason for using infant formula
Complementary food between 4 and 11
months of age
Vegetable in toddlers
Cause ofrickets globally
Sites of bleeding in Vitamin K-deficiency
bleeding (VKDB)
Cause of DIC
HEMATOLOGY AND ONCOLOGY: MOST COMMON
Cause ofmicrocytic anemia
Population group associated with
thalassemias and hemoglobinopathies
Acquired red cell aplasia in children
Cause of folate deficiency in older children
Cause of a plastic crisis
Inherited abnormality of the RBCmembrane
Molecular defects in Hereditary
Spherocytosis
Population group associated with priapism
(unwanted painful erection of the penis)
Population group associated with
a-thalassemia syndromes
Disease involving enzymes of the hexose
monophosphate pathway
Manifestations of glucose-6-phosphate
dehydrogenase (G6PD) deficiency
Cause of drug-immune hemolytic anemia
Underlying cause of paroxysmal cold
hemoglobinuria
514
• Hepatitis E virus (HEV) infection
• Nonalcoholic fatty liver disease (NAFLD)
• Bleeding from esophageal varices
• Hepatic and renal diseases
• Lymphatic malformation
• Painless abdominal distention
• Perforated appendix
• Perforated appendix
• Iron deficiency
• Parental preference
• Infantcereal
• French fries
• Vitamin D deficiency
• GIT,mucosa! & cutaneous tissue,
umbilical stump, postcircumcision site
• Sepsis
• lron deficiency
• Patients of Mediterranean, Middle Eastern,
African, or Asian descent
• Transient erythroblastopenia of childhood
• Malnutrition
• Infection with parvovirus Bl 9
• Hereditary spherocytosis
• Abnormalities of spectrin or ankyrin
• Males with sickle cell anemia
• Patients from Southeast Asia
• Glucose-6-phosphate dehydrogenase
(G6PD) deficiency
• Neonatal jaundice & episodic acute
hemolytic anemia induced by infections,
certain drugs, and fava beans
• Cephalosporins
• Nonspecific viral infections

Hypoxic polycythemia
Congenital coagulation factor
deficiencies
Inherited bleeding disorder
Type ofvWD
Inherited thrombophilia
Inherited risk factor for thrombosis
Medical conditions associated
thromboembolic events
Spontaneous TE in neonates
Imaging study for diagnosis of upper,
or more often lower, extremity venous
thromboembolism
Adverse effect related to heparin
warfarin therapy
Cause of acute onset thrombocytopenia
in an otherwise well child
Viruses associated with ITP
Neoplasms in childhood
Cancer in adolescents & young adults
Primary brain tumors
Location of primary brain tumors
Type of astrocytoma
Site of PA
Extracranial solid tumor in children
and the most commonly diagnosed
malignancy in infants
Site ofneuroblastoma
Primary malignant renal tumor of
childhood
Initial clinical presentation ofWilms
tumor
Primary malignant intraocular tumor of
childhood
• Cardiovascular defects involving right-
to-left shunts and pulmonary diseases
interfering with proper oxygenation
• Hemophilia A (factor VIII deficiency) and
hemophilia 8 (factor IXdeficiency)
• von Willebrand disease (vWD)
• Type 1
• Factor V Leiden mutation
• Factor V Leiden mutation
with • Cancer; congenital heart disease, and
prematurity
• Renal vein thrombosis
• Ultrasound with Doppler flow
or
• Bleeding
• Idiopathic (autoimmune)
thrombocytopenic purpura (ITP)
• EBV,HIV
• Leukemias
• Hodgkin lymphoma
• In children 0-14 years old: pilocytic
astrocytomas and medulloblastoma/
primitive neuroectodermal tumors
• In adolescents 15-19 years old: pituitary
tumors and pilocytic astrocytomas
• <l year old: supratentorial
• 1-10 years old: infratentorial
• >10 years old: supratentorial
• Pilocytic Astrocytoma (PA)
• Cerebellum
• Neuroblastoma
• Adrenal glands
• Wilms tumor (also called nephroblastoma)
• Incidental discovery of an asymptomatic
abdominal mass by parents or by a
physician during a routine examination
• Retinoblastoma
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I
PULMONOLOGY: MOST COMMON

• Failure to initiate & maintain effective

Delivery room emergency for neonates
respirations
• Mixedapnea (50-75% of cases),with obstructive
Pattern of idiopathic apnea
apnea usually preceding central apnea
Cause of respiratory distress among
• Transient tachypnea of the newborn
term infants born by Cesarean delivery
Cause ofpneumothorax • Over-inflation resulting in alveolar rupture
• Lower respiratory infection in <7 years old
Cause ofpneumomediastinum
• Asthma in older children and teenagers
Cause of extubation failure in children • Postextubation upper airway obstruction
Complications of tracheotomy • Pneumomediastinum & pneumothorax
Cause ofrespiratory insufficiency • Head trauma
Mechanism of arterial hypoxemia in
• Ventilation-perfusion mismatch
lung disease
Cause of post-neonatal infant mortality • Sudden infant death syndrome
Cause of septa! deviation noted at birth • Trauma from delivery
Congenital anomaly of the nose • Choanal atresia
Site of epistaxis (nosebleed) • Kiesselbach plexus
Pathogens associated with common cold • >200 types of human rhinoviruses
Complication of a cold • Acute otitis media
Cause of bacterial infection in pharynx • Group A ~-hemolytic streptococcus (GABHS)
Cause of cough in children • Airway reactivity (asthma)
Form of acute upper respiratory obstruction • Croup
Isolated pathogen in bacterial tracheitis • S. aureus
Congenital laryngeal anomaly • Laryngomalacia
Cause of stridor in infants and children • Laryngomalacia
Cause of secondary tracheomalacia • Aberrant innominate artery
Objects that children choke on • Food like peanuts, coins, balloons, and toys
Bronchial foreign body • Nuts
Cause of airway obstruction requiring
tracheostomy in infants • Laryngotracheal stenosis

Factor contributing to laryngeal injury • Oversized endotracheal tube

Cause of chronic hoarseness in children • Vocal nodules
Respiratory tract neoplasm in children • Papillomas
HPV types associated with laryngeal
disease • HPV types 6 and 11

Underlying problem associated with

recurrent pneumonias in hospitalized • Oropharyngeal incoordination
children
Parasite causing transient pulmonary • A. lwnbricoides
infiltrates with eosinophilia syndrome (formerly Loffler syndrome)

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Causes ofparapneumonic effusions and
empyema • S. aureus, S. pneumoniae, and S. pyogenes

Presenting symptom of pulmonary

embolism in adolescents • Pleuritic chest pain

Presenting symptom of pulmonary

embolism in all pediatric patients • Unexplained and persistent tachypnea

Pulmonary malignancy in children • Metastatic lesions

Cause of pleural effusion in children • Bacterial pneumonia
• Streptococcuspneumoniae(S.aureusin
Cause of empyema
developing nations & posttraumatic empyema)

IMMUNOLOGY: MOST COMMON

Cause of eosinophilia in developing • Invasive infection with multicellular

countries helminthic parasites
• Patients recovering from
Cause ofmonocytosis
myelosuppressive chemotherapy
Cause oflymphocytosis • Acute viral illness
Skin abnormality of allergic children • Xerosis (dry skin)
Hematologic abnormality of allergic
patients • Eosinophilia

Chronic symptoms of asthma • Intermittent dry coughing & wheezing

Adverse effects of inhaled • Oral candidiasis (thrush) and dysphonia
corticosteroids (hoarse voice)
Chronic relapsing skin disease seen in
• Atopic dermatitis
infancy and childhood
Hypersensitivity response of the eye • Allergic conjunctivitis
Trigger of seasonal allergic conjunctivitis • Pollens
Site of a topic keratoconjunctivitis • Lower tarsal conjunctiva
Causes of acute urticaria • Drugs and foods
• Cutaneous non pitting and nonpruritic
Symptom of hereditary angioedema
edema not associated with urticaria
Cause of anaphylaxis occurring inside
• Allergic reactions to medications and latex
the hospital
Cause of anaphylaxis occurring outside
• Food allergy
the hospital
Cause of food-induced allergy • Peanut allergy
Food proteins implicated in Food Protein-
• Cow's milk, soy
Induced Gastrointestinal Syndromes
Symptoms of food allergic reactions • Acute urticaria and angioedema
Form of adverse drug reactions • Cutaneous reactions
Drugs involved in adverse drug • Ampicillin, amoxicillin, penicillin, and
reactions trimethoprim-sulfamethoxazole
Drug associated with Red Man Syndrome • Vancomycin

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I
RHEUMATOLOGY: MOST COMMON
Rheumatic disease in children • Juvenile idiopathic arthritis ()IA}
Subtype ofJIA • Oligoarthritis (40-50%)
Affected joints in Enthesitis-Related
• Knees, ankles, and hips
Arthritis
Presenting complaints of children with • Fever, fatigue, hematologic abnormalities,
SLE arthralgia, and arthritis
• Infection,complications of glomerulonephritis
Causes of death in individuals with SLE
and neuropsychiatric disease
Subtype of Juvenile Localized Scleroderma
• Linear scleroderma
(JLSor morphea)
Extracutaneous manifestation of JLS • Arthritis
Cause of death in Juvenile Systemic • Heart failure caused by myocardial and
Sclerosis pulmonary fibrosis
Manifestations of Sjogren syndrome in • Recurrent parotid gland enlargement and
children parotitis
• Sicca syndrome (dry mouth, painful
Manifestations of Sjogren Syndrome in
mucosa, sensitivity to spicy foods,
adults
halitosis, widespread dental caries)
• Periodic Fever, Aphthous Stoma ti tis,
Recurrent fever syndrome in children
Pharyngitis, and Adenitis (PFAPA)
Vessels affected in Kawasaki disease • Coronary arteries
Vasculitis of childhood • Henoch-Schiinlein purpura
Population group in Takayasu Arteritis (TA} • Asians
Aortic branches affected in TA • Subclavian, renal, and carotid arteries
• Patients with ulcerative colitis who
Population group associated with
ankylosing spondylitis have the human leukocyte antigen B27
phenotype
Presenting problem of children referred
to pediatric rheumatology clinics • Musculoskeletal pain

Cause of recurrent musculoskeletal • Benign nocturnal pains of childhood

pain in children (growing pains)

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CARDIOLOGY: MOST COMMON
Findings for innocent or functional
murmur
Congenital heart defect
Form of atrial septal defect
Cause of death in adolescent athletes
Congenital defect associated with
sudden death in children
• Medium-pitched, vibratmy or "musical;'
relatively short systolic ejection murmur. heard
best along the left lower & midsternal border
• Ventricular septa! defect (VSD)
• Ostium secundum defect
• Hypertrophic cardiomyopathy
• Valvar aortic stenos is

Form of acquired heart disease in all ages • Rheumatic Heart Disease (RHD)
Cause of serious morbidity in those with • Heart failure caused by vegetations
documented infective endocarditis involving the aortic or mitral valve
Form of cardiomyopathy in children • Dilated cardiomyopathy (DCM)
Etiology of DCM • Idiopathic
• Sharp, positional chest pain worse with
Symptom of acute pericarditis
inspiration & relieved by sitting upright
Pediatric cardiac tumors • Rhabdomyomas (myxomas in adults)
Diuretic in children with heart failure • Furosemide
Manifestation of target-organ damage
• Left ventricular hypertrophy (LVH)
in hypertensive children
NEPHROLOGY: MOST COMMON
Cause of gross hematuria • Bacterial urinary tract infection
Chronic glomerular disease in children • lgA nephropathy
Cause ofhypoalbuminemia in children • Renal disorder
Cause ofnephrotic syndrome worldwide • Membranous nephropathy from malaria
Cause of persistent proteinuria in
school-age children and adolescents • 01thostatic (postural) proteinuria

Glomerular lesions associated with • Minimal change disease

idiopathic nephrotic syndrome
Presenting symptom of children with
nephrotic syndrome • Edema

Form of glomerulonephritis (GN) • Postinfectious GN

Form of hemolytic-uremic syndrome (HUS) • Diarrhea-associated HUS from E.coli
Age group associated with HUS • Preschool and school-age children
Hereditary human kidney disease • Autosomal dominant polycystic kidney disease
Serious bacterial infection in infants <24
• Pyelonephritis
months with fever without an obvious focus
Characteristic of urinary tract obstruction • Hydronephrosis
Etiology or renal insufficiency in child with
• Acute tubular necrosis
volume depletion
Cause of severe obstructive uropathy • Posterior urethral valves

Cause of daytime incontinence • Overactive bladder (urge incontinence)

Metabolic abnormality in children with • Normocalcemic hypercalciuria
urinary calculi
519
I
Sign of urethral prolapse • Bloody spotting on the underwear or diaper

Disorder of sexual differentiation in boys • Undescended (cryptorchid) testis

Tumor developing in an undescended
• Seminoma
testis in an adolescent or adult
Cause of testicular pain in boys >12 years • Testicular (spern1atic cord) torsion
Cause of testicular pain in boys 2-10 • Torsion of the appendix testis
years old but is rare in adolescents
Surgically correctable cause of subfertility • Varicocele
in men (virtually the only one)
Gynecologic-based problem for
• Vulvovaginitis
prepubertal children
Foreign object causing vaginal bleeding
• Toilet paper
in prepubertal girls
Vulvar lesion • Condyloma acuminata
Miillerian anomaly • Uterine septum
Structural uterine anomaly • Uterine septum
Cause of vaginal agenesis • Mayer-Rokitansky-K(ister-Hauser syndrome
Obstructive anomaly of the vagina • lmperforate hymen

ENDOCRINOLOGY· MOST COMMON

Manifestations of hypoglycemia In
newborn
Cause ofhyperinsulinemic
hypoglycemia in neonates
Transient hyperinsulinemic
Cause of infants large for gestational age
Cause ofhypopituitarism
Pituitary adenoma in childhood
Pituitary tumors in adolescents
Presentation of prolactinoma
Condition characterized by
disproportionate short stature
Cause of tall stature
Cause of permanent congenital
hypothyroidism
Cause of congenital hypothyroidism
worldwide
• Pallor; tremulousness, refusal to feed,
somnolence, lethargy, seizures
• Transient hyperinsulinism
state • Infants born to diabetic mothers
• Maternal diabetes
• Pituita1y tumors
• Prolactinoma
• Prolactin-secreting pituitary adenomas
• Headache, primary or secondary
amenorrhea, and galactorrhea
• Achondroplasia
• Normal variant, familial, or constitutional
• Thyroid dysgenesis (aplasia, hypoplasia, or
an ectopic gland]
• Iodine deficiency or endemic goiter

Cause of acquired hypothyroidism • Chronic lymphocytic (Hashimoto) thyroiditis

Most common cause of thyroid disease • Chronic lymphocytic (Hashimoto]
in children and adolescents thyroiditis

Site of distant metastasis in thyroid cancer • Lungs

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Clinical manifestations of chronic
lymphocytic thyroiditis
Differentiated thyroid cancer
Etiology for adrenal insufficiency
Cause of ambiguous genitals
Form of congenital adrenal hyperplasia
Cause of Addison disease
Cause of secondary adrenal
insufficiency
Test to diagnose secondary adrenal
insufficiency
Cause of Cushing syndrome
Etiology of endogenous Cushing syndrome
in children older than 7 years of age
Presenting symptom in children with
adrenocortical tumors
Site of origin of pheochromocytomas
Cause of adrenal calcification
Manifestations of Noonan syndrome
Cause ofhypogonadism and infertility
in males
Form of Hypogonadotropic Hypogonadism
in males (secondary hypogonadism)
Cardiovascular malformations in
Turner Syndrome
Form of disorder of sex development (DSD)
Form of 46,XX DSD
Form of male DSD
Brain lesion causing central precocious puberty
Cause of abnormal uterine bleeding in
adolescents in early postmenarchal years
Endocrine-metabolic disorder of
childhood and adolescence
Oral agent for T2DM in children and
adolescents (only one FDA-approved)
Site of acanthosis nigricans
521
• Goiter and growth retardation
• Follicular cell origin (papillary carcinoma
as the most common subtype)
• Congenital adrenal hyperplasia & adrenal
destruction or dysfunction (autoimmune)
• Congenital adrenal hyperplasia
• 21-hydroxylase deficiency
• Autoimmune destruction of adrenal glands
• Suppression by prolonged administration
of high doses of a potent glucocorticoid
and that agent is suddenly withdrawn or
the dose is tapered too quickly
• Low-dose ACTH stimulation testing (1
ftg/1.73 m2 of cosyntropin given IV)
• Prolonged exogenous administration of
glucocorticoid hormones at high doses
• Cushing disease
• Virilization
• Adrenal medulla
• Hemorrhage into the adrenal gland at or
immediately after birth
• Short stature, webbing of the neck, pectus
carinatum or pectus excavatum, cubitus
valgus, right-sided congenital heart
disease, and characteristic facies
• Klinefelter syndrome
• Kallmann syndrome
• Aortic valve abnormalities and aortic
coarctation
• Virilization of a female
• Virilizingfonns of congenitaladrenal hyperplasia
• Androgen Insensitivity Syndromes
• Hypothalamic hamartomas
• Anovulation (because of immaturity of the
hypothalamic-pituitary-ovarian axis)
• OM Type 1
• Metformin
• Neck and other flexural areas
I
NEUROLOGY: MOST COMMON
Cause of anosmia (loss of smell)
Developmental abnormality of the brain
Malformation of posterior fossa & hindbrain
Cause of non-obstructive or
communicating hydrocephalus
Megalencephalic syndrome
Form of craniosynostoses
Type of focal seizures without
impairment of consciousness
Type of benign epilepsy syndromes with
focal seizures
Cause of surgically remediable partial
epilepsy in adolescents and adults
Generalized motor seizures
Generalized epilepsy in young adults
Epilepsy related mortality in patients
with chronic epilepsy
Type of status epilepticus
Age group in status epilepticus
Form of primary headache of childhood
Migraine triggers
Aura experienced by children
Type of visual aura in children and
adolescents
Preventive therapy for headache and
migraine
Acquired chorea of childhood
Presenting manifestations of neuromuscular
disease in infants & young children
Hereditary neuromuscular disease
affecting all races and ethnic groups
Genetically determined neuropathy
Cause of neuromuscular paralysis
worldwide
Cause of Bell palsy
522
• Transient abnormality in association with an
upper respiratory tract infection or allergies
• Dandy-Walker malformation
• Chiari malformation
• Subarachnoid hemorrhage which is
usually a result of intraventricular
hemorrhage in a premature infant
• Sotos syndrome (cerebral gigantism)
• Sagittal type (scaphocephaly)
• Brief motor seizures
• Benign childhood epilepsy with
centrotemporal spikes
• Mesia! (also termed medial) temporal
sclerosis
• Generalized tonic-clonic seizures that can
be either primarily generalized (bilateral)
or secondarily generalized
• juvenile myoclonic epilepsy (Janz syndrome)
• Sudden Unexpected Death in Epilepsy
(SUDEP)
• Convulsive status epilepticus (generalized
tonic, clonic, or tonic-clonic)
• Children <5 years old
• Migraine and tension-type headaches
• Skipping meals, inadequate or irregular
sleep, dehydration and weather changes
• Epigastric discomfort or pain & feeling of fear
• Photopsia (flashes of light or light bulbs
going off everywhere)
• Bloody spotting on underwear or diaper
• Amitriptyline
• Sydenham chorea (St. Vitus dance)
• Generalized hypotonia and motor
developmental delay
• Duchenne muscular dystrophy (DMD)
• Charcot-Marie-Tooth disease
• Guillain-Barre syndrome (GBS)
• Active or reactivation of herpes simplex or
varicella-zoster virus
ACUTECARE·MOSTCOMMON
• 0-4 years old: falls, nonaccidental trauma
Cause of traumatic brain injury
• > 14 years old: motor vehicle injury

Type of sports and recreational injury • Bicycle-related trauma

Cause of forearm fracture • Fall on the outstretched hand
Dislocated joint in childhood • Elbow
• junction of the middle and lateral 3rd
Site of clavicular fracture
clavicle
Fractured bone of lower limb in children • Tibia
Cause of upper airway obstruction • Adenotonsillar hypertrophy
Cause of stridor • Croup
Cause of choking in infants • Liquids
Type of shock in children worldwide • Hypovolemic shock (usually from diarrhea)
Causes of distributive shock • Sepsis and burn injuries
• Congenital heart disease, myocarditis, and
Causes of cardiogenic shock
cardiomyopathies
Pre-arrest rhythms in young children • Bradyarrhythmias
Cause of syncope in the normal • Neurocardiogenic syncope (vasovagal
pediatric population syncope)
Cause of mortality and long-term
• CNS injury
morbidity in drowning
Mostcommon cause of burn in young children • Scald burn
Cause of burn in children 5-14 years of • Flame injury (result of match play or
age gasoline ignition)
Injured site in burns • Hands
• Nonnutritive hand-to-mouth activity of
Pathway for lead to enter the body
young children
• Condition of old paint, occupational exposure
Sources oflead exposure via an adult living in the home, and/or
proximity to an industrial source of pollution
• jellyfish (Cnidaria), stingrays
Encountered venomous marine (Chondrichthyes), and members of
creatures the family Scorpaenidae-the lionfish,
scorpionfish, and stonefish

REFERENCES
1. Kliegman, R., ST GEME Ill, j., Blum, N., Shah, S., Tasker, R., Wilson, K., & Behrman, R. (2020).
Nelson Textbook of Pediatrics (21st ed.). Philadelphia: Elsevier.

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