TABLE OF CONTENTS

TheJOURNAL
of PEDIATRICS
February 2018 Volume 193 Copyright © 2018 by Elsevier Inc.

The Editors’ Perspectives

This Month in The Journal of Pediatrics ................................................................................................................. 1

Notes from the Association of Medical School Pediatric Department Chairs, Inc.
Hospitalist Medicine—Chairs’ Perspective of Specialty Status and Training Requirements e
D. Wade Clapp, MD, Teresa Quattrin, MD, Richard F. Jacobs, MD, and Valerie Castle Opipari, MD ............................................. 4

Editorials
Improvement of Outcomes for Children with Down Syndrome
Marilyn J. Bull, MD . ......................................................................................................................................................... 9

Is It Necessary to Heat and Humidify Respiratory Gases for Resuscitation in Preterm Infants?
Waldemar A. Carlo, MD, and Robert L. Chatburn, RRT-NPS .................................................................................................. 10

How to Make Pediatric Trials Better: Transparency and Accountability of Trial Registration and
Reporting
Ana Marušić, MD, PhD, and Shelly M. Pranić, PhD ............................................................................................................... 11

Medical Progress
Children as Biomarker Orphans: Progress in the Field of Pediatric Biomarkers e
Darla R. Shores, MD, PhD, and Allen D. Everett, MD ............................................................................................................. 14

Original Articles
Infants Born with Down Syndrome: Burden of Disease in the Early Neonatal Period
Therese Martin, MB, Aisling Smith, MB, Colm R. Breatnach, MB, Etaoin Kent, MD, Ita Shanahan, MB, Michael Boyle, PhD,
Phillip T. Levy, MD, Orla Franklin, FRCPCH, and Afif El-Khuffash, FRCPI, DCE ...................................................................... 21

Characteristics and Progression of Hearing Loss in Children with Down Syndrome e

Kathryn L. Kreicher, MD, Forest W. Weir, MD, Shaun A. Nguyen, MD, MA, and Ted A. Meyer, MD, PhD ..................................... 27

e – contains additional content that can be found at www.jpeds.com

The Journal of Pediatrics (ISSN 0022-3476) is published monthly by Elsevier Inc., 230 Park Avenue, Suite 800, New York, NY 10169-0901, USA. Periodicals
postage paid at New York, NY and additional mailing offices. POSTMASTER: Send address changes to Elsevier, Journal Returns, 1799 Highway 50 East,
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50 Years Ago in The Journal of Pediatrics – The Rationale for Genetic Counseling
Barbara Bowles Biesecker, PhD, MS, Leslie Glenn Biesecker, MD ......................................................................................... 33

Race Effects of Inhaled Nitric Oxide in Preterm Infants: An Individual Participant Data
Meta-Analysis e
Lisa M. Askie, PhD, MPH, BN, Lucy C. Davies, MSc, BSc, Michael D. Schreiber, MD, Anna Maria Hibbs, MD, MSCE,
Philip L. Ballard, MD, PhD, and Roberta A. Ballard, MD ........................................................................................................ 34

Laryngeal Mask Airway for Surfactant Administration in Neonates: A Randomized,

Controlled Trial e
Kari D. Roberts, MD, Roland Brown, Andrea L. Lampland, MD, Tina A. Leone, MD, Kyle D. Rudser, PhD, Neil N. Finer, MD,
Wade D. Rich, RRT, CCRC, T. Allen Merritt, MD, Adam J. Czynski, DO, Julie M. Kessel, MD, Sajani M. Tipnis, MD,
Erin C. Stepka, MD, and Mark C. Mammel, MD ................................................................................................................... 40

A Randomized Trial of Conditioned or Unconditioned Gases for Stabilizing Preterm Infants at Birth
Lorraine McGrory, MBChB, Louise S. Owen, MD, Marta Thio, PhD, Jennifer A. Dawson, PhD, Anthony R. Rafferty, PhD,
Atul Malhotra, MD, Peter G. Davis, MD, and C. Omar F. Kamlin, DMedSci . ............................................................................ 47

Altered Cerebral Perfusion in Infants Born Preterm Compared with Infants Born Full Term e
Marine Bouyssi-Kobar, MS, Jonathan Murnick, MD, PhD, Marie Brossard-Racine, PhD, Taeun Chang, MD, Eman Mahdi, MD,
Marni Jacobs, PhD, and Catherine Limperopoulos, PhD . ....................................................................................................... 54

Exposure to Gastric Acid Inhibitors Increases the Risk of Infection in Preterm Very Low Birth Weight
Infants but Concomitant Administration of Lactoferrin Counteracts This Effect e
Paolo Manzoni, MD, Ruben García Sánchez, MD, Michael Meyer, MD, Ilaria Stolfi, MD, Lorenza Pugni, MD,
Hubert Messner, MD, Silvia Cattani, MD, Pasqua Maria Betta, MD, Luigi Memo, MD, Lidia Decembrino, MD, Lina Bollani, MD,
Matteo Rinaldi, MD, Maria Fioretti, MD, Michele Quercia, MD, Milena Maule, PhD, Elena Tavella, MD, Alessandro Mussa, MD,
Chryssoula Tzialla, MD, Nicola Laforgia, MD, Fabio Mosca, MD, PhD, Rosario Magaldi, MD, Michael Mostert, MD, PhD, and
Daniele Farina, MD, on behalf of the Italian Task Force for the Study, and Prevention of Neonatal Fungal Infections and the Italian
Society of Neonatology ...................................................................................................................................................... 62

Long-Term Outcomes of Hyperglycemic Preterm Infants Randomized to Tight Glycemic Control e

Anna Catherine Tottman, MBBS, Jane Marie Alsweiler, PhD, Frank Harry Bloomfield, PhD, Greg Gamble, MSc,
Yannan Jiang, PhD, Myra Leung, BOptom, Tanya Poppe, MSc, Benjamin Thompson, PhD, Trecia Ann Wouldes, PhD, and
Jane Elizabeth Harding, DPhil, on behalf of the PIANO Study Group ....................................................................................... 68

Periconception Exposure to Air Pollution and Risk of Congenital Malformations e

Sheng Ren, PhD, Erin Haynes, DrPH, Eric Hall, PhD, Monir Hossain, PhD, Aimin Chen, MD, PhD, Louis Muglia, MD, PhD,
Long Lu, PhD, and Emily DeFranco, DO MS . ....................................................................................................................... 76

50 Years Ago in The Journal of Pediatrics – Atypical Exanthem after Exposure to Natural Measles:
Eleven Cases in Children Previously Inoculated with Killed Vaccine
Philip Toltzis, MD ........................................................................................................................................................ 84

Differentiating the Preterm Phenotype: Distinct Profiles of Cognitive and Behavioral Development
Following Late and Moderately Preterm Birth e
Samantha Johnson, PhD, CPsychol, AFBPsS, Ghazala Waheed, MSc, Bradley N. Manktelow, PhD, David J. Field, DM,
Neil Marlow, DM, FMedSci, Elizabeth S. Draper, PhD, and Elaine M. Boyle, MD, PhD ............................................................... 85

50 Years Ago in The Journal of Pediatrics – Phenylketonuria: Evaluation of Therapy and

Verification of Diagnosis
Ola Didrik Saugstad, MD . ............................................................................................................................................. 92

Origins and Predictors of Friendships in 6- to 8-Year-Old Children Born at Neonatal Risk e

Katharina M. Heuser, MSc, Julia Jaekel, PhD, and Dieter Wolke, PhD, Dr rer nat hc ................................................................... 93

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Obese Children Require Lower Doses of Pantoprazole Than Nonobese Peers to Achieve Equal
Systemic Drug Exposures e
Valentina Shakhnovich, MD, P. Brian Smith, MD, MHS, MPH, Jeffrey T. Guptill, MD, MA, MHS, Laura P. James, MD,
David N. Collier, MD, PhD, FAAP, Huali Wu, PhD, Chad E. Livingston, MBA, Jian Zhao, PhD, and
Gregory L. Kearns, PharmD, PhD, on behalf of the Best Pharmaceuticals for Children Act – Pediatric Trials Network . ................... 102

Usefulness of Routine Transtelephonic Monitoring for Supraventricular Tachycardia in Infants

Jonathan Yaari, MD, Dorota Gruber, MSc, and Andrew D. Blaufox, MD ................................................................................... 109

Characteristics and Outcomes of Pediatric Heart Failure-Related Emergency Department Visits in the
United States: A Population-Based Study e
Erika J. Mejia, MD, Matthew J. O’Connor, MD, Kimberly Y. Lin, MD, Lihai Song, MS, Heather Griffis, PhD,
Christopher E. Mascio, MD, Pirouz Shamszad, MD, Aaron Donoghue, MD, MSCE, Chitra Ravishankar, MD,
Robert E. Shaddy, MD, and Joseph W. Rossano, MD, MS ........................................................................................................ 114

Subclinical Atherosclerosis in Pediatric Liver Transplant Recipients: Carotid and Aorta Intima-Media
Thickness and Their Predictors e
Emily R. Perito, MD, MAS, Andrew Phelps, MD, Tabitha Vase, BA, Vickie A. Feldstein, MD, Robert H. Lustig, MD, and
Philip Rosenthal, MD ........................................................................................................................................................ 119

Self-Management Measurement and Prediction of Clinical Outcomes in Pediatric Transplant e

Rachel A. Annunziato, PhD, John C. Bucuvalas, MD, Wanrong Yin, MS, Ravinder Arnand, PhD, Estella M. Alonso, MD,
George V. Mazariegos, MD, Robert S. Venick, MD, Margaret L. Stuber, MD, Benjamin L. Shneider, MD, and Eyal Shemesh, MD ..... 128

Ultrasonographic Quantitative Analysis of Fatty Pancreas in Obese Children: Its Correlation with
Metabolic Syndrome and Homeostasis Model Assessment of Insulin Resistance e
Doo Ri Kim, MD, Mu Sook Lee, MD, Jeong Sub Lee, MD, Guk Myung Choi, MD, and Ki Soo Kang, MD ...................................... 134

Resource Use and Morbidities in Pediatric Cardiac Surgery Patients with Genetic Conditions e
Jamie Furlong-Dillard, DO, David Bailly, DO, Venugopal Amula, MD, MBBS, Jacob Wilkes, BS, and Susan Bratton, MD, MPH . ..... 139

Intrauterine and Early Postnatal Exposure to Particulate Air Pollution and Kawasaki Disease:
A Nationwide Longitudinal Survey in Japan e
Takashi Yorifuji, MD, PhD, Hirokazu Tsukahara, MD, PhD, Saori Kashima, PhD, and Hiroyuki Doi, MD, PhD . ........................... 147

Kawasaki Disease Substantially Impacts Health-Related Quality of Life e

Soultana Kourtidou, MD, MSc, April E. Slee, MS, Margaret E. Bruce, BS, Haaland Wren, MPH,
Rita M. Mangione-Smith, MD, MPH, and Michael A. Portman, MD ........................................................................................ 155

50 Years Ago in The Journal of Pediatrics – The Antepartum Diagnosis of Genetic Diseases
Bimal P. Chaudhari, MD, MPH ...................................................................................................................................... 163

Frequency and Cost of Vaccinations Administered Outside Minimum and Maximum Recommended
Ages—2014 Data From 6 Sentinel Sites of Immunization Information Systems
Loren Rodgers, PhD, Lauren Shaw, MS, Raymond Strikas, MD, MPH, Beth Hibbs, RN, MPH, JoEllen Wolicki, RN,
Cristina V. Cardemil, MD, MPH, and Cindy Weinbaum, MD, MPH . ....................................................................................... 164

Efficacy of Human Botulism Immune Globulin for the Treatment of Infant Botulism: The First 12
Years Post Licensure
Jessica R. Payne, MPH, Jessica M. Khouri, MD, Nicholas P. Jewell, PhD, and Stephen S. Arnon, MD, MPH .................................... 172

More Clinical Mimics of Infant Botulism

Jessica M. Khouri, MD, Jessica R. Payne, MPH, and Stephen S. Arnon, MD, MPH . .................................................................... 178

Severity and Features of Epistaxis in Children with a Mucocutaneous Bleeding Disorder e

Eva Stokhuijzen, MD, Catherine I. Segbefia, MB, ChB, Tina T. Biss, MD, Dewi S. Clark, BSc, Paula D. James, MD,
Jim Riddel, RN, MS, Victor S. Blanchette, MB BChir(Cantab), FRCP(C), and Margaret L. Rand, PhD ........................................... 183

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Clinical Indices Can Standardize and Monitor Pediatric Care: A Novel Mechanism to Improve
Quality and Safety e
Wallace Crandall, MD, MMM, J. Terrance Davis, MD, Jennifer Dotson, MD, MPH, Charles Elmaraghy, MD, Mike Fetzer, BSISE,
Don Hayes Jr, MD, MS, Edwin Horwitz, MD, PhD, Amy Kogon, MD, MPH, Randal Olshefski, MD, Hiren Patel, MD, and
Richard J. Brilli, MD, FAAP, MCCM .................................................................................................................................... 190

Patterns of Care at the End of Life for Children and Young Adults with Life-Threatening Complex
Chronic Conditions e
Danielle D. DeCourcey, MD, MPH, Melanie Silverman, MPH, Adeolu Oladunjoye, MPH, Emily M. Balkin, MD, and
Joanne Wolfe, MD, MPH . .................................................................................................................................................. 196

One-Year Outcome for Congenital Diaphragmatic Hernia: Results From the French National Register
François Barrière, MD, Fabrice Michel, MD, PhD, Anderson D. Loundou, PhD, Virginie Fouquet, MD, Elsa Kermorvant, MD,
Sébastien Blanc, MD, Elisabeth Carricaburu, MD, Amélie Desrumaux, MD, Odile Pidoux, MD, Alexis Arnaud, MD,
Nicolas Berte, MD, Thierry Blanc, MD, Frederic Lavrand, MD, Guillaume Levard, MD, Isabelle Rayet, MD, Sylvain Samperiz, MD,
Anne Schneider, MD, Marie-Odile Marcoux, MD, Norbert Winer, MD, Yann Chaussy, MD, Valérie Datin-Dorriere, MD,
Quentin Ballouhey, MD, Aurélien Binet, MD, Charles Muszynski, MD, Jean Breaud, MD, Armelle Garenne, MD,
Laurent Storme, MD, PhD, and Julia Boubnova, MD, on behalf of the Center for Rare Diseases for Congenital
Diaphragmatic Hernia ....................................................................................................................................................... 204

50 Years Ago in The Journal of Pediatrics – Differential Cyanosis in Congenital Heart Disease
Sadagopan Srinivasan, MD, and Kothandam Sivakumar, MD, DM ...................................................................................... 210

Enuresis Management in Children: Retrospective Clinical Audit of 2861 Cases Treated with
Practitioner-Assisted Bell-and-Pad Alarm
Esther Apos, PhD, Sharynn Schuster, PhD, MAPS, John Reece, PhD, MAPS, Shirley Whitaker, RN, Kerry Murphy, RN,
John Golder, MBBS, FRACGP, Beverley Leiper, RN, Linda Sullivan, RN, and Susie Gibb, MBBS, FRACP . ..................................... 211

Prolonged Time Lag to Final Diagnosis of Fragile X Syndrome e

Lidia V. Gabis, MD, Oded Hochberg, MD, Odelia Leon Attia, MA, Yonit Banet-Levi, RN, MHA, Dana Topf, BA, and
Shahar Shefer, PhD . .......................................................................................................................................................... 217

Postacute Care after Pediatric Hospitalizations for a Primary Mental Health Condition e
James C. Gay, MD, MMHC, Bonnie T. Zima, MD, MPH, Tumaini R. Coker, MD, MBA, Stephanie K. Doupnik, MD, MS,
Matthew Hall, PhD, Jonathan Rodean, MS, Margaret O’Neill, BS, Rustin Morse, MD, MMM, Kris P. Rehm, MD,
Jay G. Berry, MD, MPH, and Naomi S. Bardach, MD, MAS ..................................................................................................... 222

The Adolescent Champion Model: Primary Care Becomes Adolescent-Centered via Targeted Quality
Improvement e
Margaret Riley, MD, Vani Patterson, MPH, Jennifer C. Lane, MA, Katharine McDonald Won, MD, MPH, and
Lauren Ranalli, MPH . ....................................................................................................................................................... 229

The Conduct and Reporting of Child Health Research: An Analysis of Randomized Controlled Trials
Published in 2012 and Evaluation of Change over 5 Years e
Allison Gates, PhD, Lisa Hartling, PhD, Ben Vandermeer, MSc, Patrina Caldwell, PhD, Despina G. Contopoulos-Ioannidis, MD,
Sarah Curtis, MD, Ricardo M. Fernandes, MD, PhD, Terry P. Klassen, MD, Katrina Williams, PhD, and
Michele P. Dyson, PhD . ..................................................................................................................................................... 237

Grand Rounds
Hepatic Vascular Tumors in the Neonate: Angiosarcoma e
Courtney Kiser McLean, MD, Judy H. Squires, MD, Miguel Reyes-Múgica, MD, Andrew McCormick, MD, and
Burhan Mahmood, MD ..................................................................................................................................................... 245

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Clinical and Laboratory Observations

Increased Pituitary Volumes in Children after Fontan Operation: Congestion in
the Other Portal Circulation
Jun Muneuchi, MD, Yusaku Nagatomo, MD, Seigo Okada, MD, Chiaki Iida, MD, Hiromitsu Shirozu, MD, Yuichiro Sugitani, MD,
and Mamie Watanabe, MD . ............................................................................................................................................... 249

Nosocomial Transmission and Genetic Diversity of Rhinovirus in a Neonatal Intensive Care Unit e
Débora Natalia Marcone, PhD, Guadalupe Carballal, MD, PhD, Mariela Irañeta, MD, Yamile Rubies, MD,
Santiago M. Vidaurreta, MD, and Marcela Echavarría, PhD .................................................................................................... 252

Barth Syndrome: Different Approaches to Diagnosis

Atsuko Imai-Okazaki, MD, PhD, MPH, Yoshihito Kishita, PhD, Masakazu Kohda, PhD, Yukiko Yatsuka, MS,
Tomoko Hirata, MS, Yosuke Mizuno, PhD, Hiroko Harashima, Keiichi Hirono, MD, PhD, Fukiko Ichida, MD, PhD,
Atsuko Noguchi, MD, PhD, Masayuki Yoshida, MD, PhD, Chiho Tokorodani, MD, Ritsuo Nishiuchi, MD, PhD,
Atsuhito Takeda, MD, PhD, Akihiro Nakaya, PhD, Yasushi Sakata, MD, PhD, Kei Murayama, MD, PhD,
Akira Ohtake, MD, PhD, and Yasushi Okazaki, MD, PhD ....................................................................................................... 256

A Nonlethal Case of Junctional Epidermolysis Bullosa and Congenital Pyloric Atresia: Compound
Heterozygosity in a Patient with a Novel Integrin Beta 4 Gene Mutation e
Lauren Ko, MEd, Cornelia L. Griggs, MD, Konstantinos S. Mylonas, MD, Peter T. Masiakos, MD, MS, FACS, FAAP, and
Daniela Kroshinsky, MD, MPH . ......................................................................................................................................... 261

Workshop/Symposium Summary
The First International Conference on Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis
Syndrome e
Liora Harel, MD, Philip J. Hashkes, MD, MSc, Sivia Lapidus, MD, Kathryn M. Edwards, MD, Shai Padeh, MD,
Marco Gattorno, MD, and Gary S. Marshall, MD .................................................................................................................. 265

Rediscovering the Physical Exam

PHACE Syndrome—before and after Propranolol Therapy
Sigrid C. Disse, MD, Michael Zemlin, MD, MHBA, Cornelia Mueller, MD, and Sascha Meyer, MD .............................................. 275

Insights and Images

Occipital Dermal Sinus Tract
Laura M. Prolo, MD, PhD, and Gerald A. Grant, MD ............................................................................................................. 276

Letters to the Editor

Resistance of the Interface Device Used to Deliver Nasal Continuous Positive Airway Pressure or
Nasal Intermittent Ventilation
Harold Perl, MD ............................................................................................................................................................... 277

Reply
Asavari Kamerkar, DO . ..................................................................................................................................................... 277

European Paediatric Association Pages

Effective School Health Service: A Response to Adolescent Health Needs in Europe e
Pierre-André Michaud, MD, Leyla Namazova-Baranova, MD, Martin Weber, MD, and
Anne-Emmanuelle Ambresin, MD, MEpi.............................................................................................................................. 278

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Reader Services
Information for Readers ............................................................................................................................................ A10

Announcement and Upcoming Events . ........................................................................ Available at www.jpeds.com

Guide for Authors ............................................................................................................ Available at www.jpeds.com

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