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Oxford Specialist
Handbooks in Paediatrics
Paediatric
Respiratory
Medicine
Second Edition
Jeremy Hull
Consultant in Paediatric Respiratory Medicine
Oxford Children’s Hospital, Oxford, UK
Julian Forton
Consultant in Paediatric Respiratory Medicine
The Children’s Hospital for Wales, Cardiff, UK
Anne Thomson
Consultant in Paediatric Respiratory Medicine
Oxford Children’s Hospital, Oxford, UK
1
3
Great Clarendon Street, Oxford, OX2 6DP,
United Kingdom
Oxford University Press is a department of the University of Oxford.
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© Oxford University Press 205
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First edition published 2008
Second edition published 205
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Published in the United States of America by Oxford University Press
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v
Preface to the
Second Edition
In writing this new edition, we have gone through each chapter, bringing
sections up to date, where needed, and including key new references.
Following the publication of the Global Lung Initiative prediction equations
for spirometric lung volumes, the section on lung function normal values has
been removed. Otherwise, the structure of the book is unchanged. The aim
of the handbook is to assist clinicians in the day-to-day care of children with
respiratory disease. For rarer conditions, there is perhaps more detail than
the reader might expect—this reflects our view that, when faced with a child
with a rare condition, a bit of background reading is required! The topics
covered in the book include all those required for the European Respiratory
Society Hermes exam in paediatric respiratory medicine, and so this hand-
book is a useful text for those considering taking that qualification.
JH, JF, AHT 205
vi
Preface to the
First Edition
In writing this handbook, our aim has been to provide a practical guide
to paediatric respiratory medicine that will be a useful first point of refer-
ence for paediatricians faced with children with acute or chronic respiratory
problems.
The book has been written for use by general paediatricians and by spe-
cialists in paediatric respiratory medicine at both consultant and trainee
level. It deals with common problems seen by all paediatricians and rarer
conditions more often seen at specialist centres, but managed in collabora-
tion with general paediatricians. The information is in a readily accessible
format, with extensive use of bullet points. The focus is on clinical presen-
tation, diagnosis, and management of respiratory problems. There is less
emphasis on background information, such as epidemiology and pathogen-
esis, but this is sufficient where necessary to provide insight into clinical
presentation and management, or where this information would be helpful
for parents.
The book is divided into four parts. Part provides a practical approach
to acute and non-acute clinical problems. Part 2, the bulk of the book,
provides detailed information about common and not-so-common clinical
conditions. Part 3 provides useful information on supportive care, including,
for example, use of non-invasive ventilation and the care of a child with a
tracheostomy. Part 4 gives details on how to perform several practical pro-
cedures, such as ciliary brush biopsy, flexible bronchoscopy, and inserting
a chest drain. Finally, the appendices provide information on lung function
testing and tables of age-corrected normal values for several respiratory
parameters.
The book has been written by two consultants who work in a tertiary
respiratory unit and by a respiratory trainee. This combination of authors
has provided the experience necessary to deal with topics where there is an
absence of published evidence and to present the information in a format
that both consultants and trainees will find useful.
Foreword to the
First Edition
So why would anyone want to buy a book in the 2st century, when a
profusion of information is available at the touch of a computer key? Cough
in children yields more than 6500 references on a PubMed search, so why
would anyone want to read a 5 page section on the same subject in this
book? One of the most misquoted phrases in English poetry, ‘a little learning
is a dangerous thing’ could now be adapted as ‘a lot of PubMed is a danger-
ous thing’, and unless the searcher has a sound grounding in the subject
searched, it is likely to lead to confusion in mind, an unbalanced perspective,
and ultimately, inappropriate management of children. A view of the woods
is an essential prelude to a detailed study of an individual tree.
And that is where this book will be so valuable to all in the field of pae-
diatric respiratory medicine, from the raw young tyro to the elderly pro-
fessorial dodderer, and all stages in between. The reader will find a clear
account of the subject, from both a problem-based and a disease-based
approach. It gives a commonsense overview of all the important topics in
the field, with crisp tables and bullet points, written in clear English. There
are a few, up-to-date papers, reviews and websites as a basis for further
learning, and the authors have fully achieved their aim, of writing a practical
handbook in line with the long tradition of the Oxford series. The trainee can
rapidly acquire a good grasp of the subject, and can then safely dive into
PubMed for more advanced studies, particularly of pathophysiology. Few if
any of the allegedly trained will read this without finding something to learn,
or some new idea not previously thought of, to try when next a problem
arises. Few will agree with absolutely every statement, but that is inevitable
and part of the intrinsic beauty of the subject—and in any event, medicine
is learned by doing, and cannot be learned solely from books, whatever the
views of those currently changing medical training with all the natural talent
of a hippopotamus playing the piccolo.
So in summary, who can benefit from this book, and how? The trainee will
certainly not outgrow it—even the most experienced paediatrician, seeing
a child with an uncommon condition, or preparing a teaching session on a
common one, will benefit from taking a surreptitious peek at the relevant
section here, to ensure nothing has been forgotten. For example, I would
challenge the reader to list the totality of the associated conditions which
need to be detected in a baby with a PHOX2b mutation before turning to
Chapter 26. Review copies of books come into three categories: ‘throw
away’, ‘give away’ and ‘chain it to the wall’—this Handbook is definitely in
the last category. Departmental thieves, hands off!
Andy Bush
Professor of Paediatric Respirology
Royal Brompton Hospital
London
viii
Acknowledgements
Contents
Appendices
Blood gases and acid–base balance 759
2 Fitness to fly 763
xii CONTENTS
3 Polysomnography 767
4 Measuring lung function 777
5 Lung function: reference values 795
Index 798
xiii
± plus/minus
= equal to
> more than
< less than
≥ equal to or more than
≤ equal to or less than
% per cent
β beta
γ gamma
£ pound sterling
°C degree Celsius
®
registered
™ trademark
AAD adaptive aerosol delivery
AAT alpha- antitrypsin
ABG arterial blood gas
ABPA allergic bronchopulmonary aspergillosis
ACBT active cycle of breathing technique
ACD alveolar capillary dysplasia
ACE angiotensin-converting enzyme
Ach acetylcholine
AChE acetylcholinesterase
AChR acetylcholine receptor
ACM Arnold–Chiari malformation
ACS acute chest syndrome
ADA adenosine deaminase
ADHD attention-deficit/hyperactivity disorder
AHI apnoea hypopnoea index
AIDS acquired immune deficiency syndrome
ALL acute lymphoblastic leukaemia
ALT alanine aminotransferase
ALTE apparent life-threatening event
ANA antinuclear antibody
ANCA antineutrophil cytoplasmic antibody
ANSD autonomic nervous system dysregulation
APTT activated partial thromboplastin time
xiv SYMBOLS AND ABBREVIATIONS
V/Q ventilation/perfusion
VSD ventricular septal defect
VZV varicella-zoster virus
WASP Wiskott–Aldrich syndrome protein
WBC white blood count
WG Wegener’s granulomatosis
WHO World Health Organization
XDT-TB extreme drug resistance
ZIG zoster immunoglobulin
Part I
Approach to
clinical problems
Examining the
respiratory system
Introduction 4
Clubbing 4
Chest shape 4
Palpation 5
Percussion 6
Stridor 6
Stertor 6
Auscultation 7
Listen to the cough 9
4 Chapter Examining the respiratory system
Introduction
Examining the chest is part of the routine physical examination of all chil-
dren who are unwell. Most doctors are expert at identifying the abnormal
signs that indicate disease. This short section provides the background to
a common language so that clear descriptions can be given to colleagues.
Clubbing
• Gross clubbing is easy to recognize. Early clubbing is more subtle with
an impression of fullness and ‘floating’ of the root of the nail bed on
compression.
• The mechanisms that underlie the development of clubbing remain
unclear. Possibilities include circulating mediators of vasodilatation
released in response to hypoxia, and effects of the vagal nerve resulting
from the observation of the association between clubbing and disease in
organs with vagal innervation.
• In children with respiratory symptoms, clubbing usually suggests
suppurative lung disease or cyanotic cardiac disease. It can also be seen
in children with chronic hypoxaemia from a respiratory cause such as
interstitial lung disease (ILD) or bronchiolitis obliterans (BO).
Chest shape
• Fixed variations of the chest shape are relatively common in children,
affecting 0.5% of the population. They usually have no significant
functional consequences. The commonest variation is pectus excavatum
(see E Chapter 40). It is best to avoid the term ‘chest deformity’ in
describing normal variations of the chest shape.
• Hyperinflation (Fig. .) is a reversible change in the chest shape and
indicates air trapping, usually as a result of small airways obstruction. In
the context of asthma, it suggests poor control. It is most easily seen
from the side.
• Harrison’s sulci (Fig. .2) refer to an indentation of the lower chest wall
with the apparent splaying of the costal margins. They may be seen
in association with hyperinflation. They are associated with chronic
respiratory disease associated with increased work of breathing and
may be caused by the necessarily increased power of diaphragmatic
contraction on relatively soft costal cartilage.
Palpation 5
Back Front
Normal Hyperinflated
Palpation
• Placing the hands on the chest can give valuable information about the
presence of secretions and wheeze. It can also help determine whether
chest expansion is symmetrical.
• In older children who can perform a vital capacity manoeuvre,
measuring the chest expansion, using a tape measure at the level of
the xiphoid cartilage, can be predictive of lung volumes measured by
spirometry. Depending on the height and sex of the subject, normal
values for chest expansion can range from 3 cm to 9 cm.
6 Chapter Examining the respiratory system
Percussion
Despite occasional views to the contrary, it is always useful to percuss the
chest in children. A dull percussion note is consistent with extensive consoli-
dation or pleural disease, either pleural thickening or pleural fluid.
Stridor
Stridor (from the Latin stridere, to make harsh sounds) is a harsh mono-
phonic noise that comes from the trachea or larynx as a result of narrowing.
It may be heard with or without a stethoscope. When the extrathoracic
airway is affected, the noise always has an inspiratory component but can
be biphasic if the narrowing is severe. When the intrathoracic trachea is
affected, the stridor will usually be biphasic, with a relatively loud expiratory
phase. A purely inspiratory stridor indicates narrowing of the extrathoracic
airway. Stridor is louder when airflows are increased, such as with crying or
exertion, and quieter when flows are reduced, e.g. during sleep.
Stertor
This term is used to describe the harsh coarse noises generated by turbu-
lent airflow in the supraglottic space. It may be caused by spasticity of the
pharyngeal muscles, e.g. in a child with cerebral palsy, by retained secre-
tions, or by adenotonsillar hypertrophy. Stertor is often louder during sleep,
because of reduced tone of the pharyngeal muscles.
Another random document with
no related content on Scribd:
ERICA spicata.
CHARACTER SPECIFICUS.
DESCRIPTIO.
REFERENTIA.
1. Calyx et Corolla.
2. Calyx et Bractea lente aucta.
3. Stamina et Pistillum.
4. Stamina a Pistillo diducta, anthera una lente aucta.
5. Stylus et Stigma lente aucta.
SPECIFIC CHARACTER.
Heath, with bearded tips, within the blossoms; which are numerous, on a
close spike, nearly terminating the branches; leaves growing mostly by
sixes.
DESCRIPTION.
REFERENCE.
1. The Empalement with the Blossom.
2. The Empalement and Floral-leaves magnified.
3. The Chives and Pointal.
4. The Chives detached from the Pointal, one tip magnified.
5. The Shaft and its Summit magnified.
ERICA spuria.
CHARACTER SPECIFICUS.
DESCRIPTIO.
REFERENTIA.
1. Calyx et Corolla.
2. Calyx lente auctus.
3. Stamina et Pistillum.
4. Stamina a Pistillo diducta; anthera una lente aucta.
5. Stylus et Stigma, lente aucta.
SPECIFIC CHARACTER.
Heath, with beardless tips, within the blossom; shaft without; blossoms
nearly cylindrical, of a light purple; segments of the border rolled back;
flowers terminate the smaller branches, mostly by fours; leaves grow by
fours, a little hairy at the edges.
DESCRIPTION.
Stem shrubby, grows a foot high, and branching; the small branches are
numerous, and nearly upright.
Leaves grow by fours, are linear, a little hairy, furrowed beneath; with
very short foot-stalks pressed to the branches.
Flowers grow at the end of the small branches, generally by fours, and
sitting close upon them; footstalks very short, having three very small floral
leaves.
Empalement. Cup of four leaves, which are awl-shaped, keeled, and
pressed to the blossom.
Blossom nearly cylindrical, an inch long, and of a light purple; segments
of the border nearly egg-shaped, sharp-pointed, and rolled back; the mouth a
little narrowed.
Chives. Eight hair-like threads, shorter than the blossom; tips beardless,
and within the blossom.
Pointal. Seed-bud egg-shaped and furrowed. Shaft thread-shaped, and
without the blossom. Summit four-cornered.
Native of the Cape of Good Hope.
Flowers from the month of April till August.
REFERENCE.
CHARACTER SPECIFICUS.
DESCRIPTIO.
REFERENTIA.
1. Calyx et Corolla.
2. Calyx, lente auctus.
3. Corolla.
4. Stamina, et Pistillum.
5. Stamina a Pistillo diducta, anthera una lente aucta.
6. Pistillum, auctum.
SPECIFIC CHARACTER.
Heath with beardless tips, within the blossom; the flowers terminate the
branches in umbels, forming a spike; blossom swelled at the base, pinched
in at the top, and almost covered by a coloured cup, with the border
spreading; leaves grow by threes, harsh, three-sided, sharp-pointed, and tiled
in six divisions round the stem.
DESCRIPTION.
Stem shrubby, upright, very branching; the larger and smaller branches
grow spreading, and upright, and are very much notched.
Leaves grow by threes, three-sided, smooth, linear, harsh, sharp-pointed,
and tiled in six divisions; with very short foot-stalks, pressed to the
branches.
Flowers grow at the ends of the small branches in umbels, numerous
and upright; foot-stalks coloured, the length of the blossoms; two floral
leaves upon the middle of the foot-stalk, and the third at its base.
Empalement. Cup four-leaved, leaflets egg-shaped, pointed, concave,
skinny, and coloured, nearly the length of the blossom.
Blossom swelled at the base, narrowed at the mouth, and flesh coloured;
segments spreading, pointed, and half egg-shaped.
Chives. Threads eight, hair-like, and curved. Tips beardless, within the
blossom, and yellow.
Pointal. Seed-bud roundish, and furrowed. Shaft within the blossom,
and thread-shaped. Summit between shield and four-cornered.
Native of the Cape of Good Hope.
Flowers from August till November.
REFERENCE.
CHARACTER SPECIFICUS.
DESCRIPTIO.
REFERENTIA.
1. Folium, auctum.
2. Calyx.
3. Calyx, auctus.
4. Corolla.
5. Stamina, et Pistillum, anthera una lente aucta.
6. Pistillum.
7. Pistillum, auctum.
SPECIFIC CHARACTER.
Heath, with beardless tips, within the blossom; flowers grow mostly singly
at the end of the branches; blossoms between club and cylindar-shaped,
curved and downy; leaves grow by fours, thin and fringed with hairs.
DESCRIPTION.
Stem flexible, upright, and downy at the upper part; branches scattered,
thread shaped, numerous and hairy, the smaller branches are very numerous
and very short.
Leaves grow by fours, are thin, blunt, fringed with hair at the edges, and
furrowed beneath.
Flowers sit close upon the ends of the small branches, mostly solitary,
and spreading, appearing like a long bunch.
Empalement. Cup four leaves, leaflets spatula-shape and fringed, with
scarce any foot-stalks; three floral leaves pressed to the blossom.
Blossom club-shaped, curved, hairy and flesh-coloured; the segments of
the border pointed, and reflexed.
Chives. Eight hair-like threads. Tips beardless, and just without the
blossom.
Pointal. Seed-bud growing on a foot-stalk nearly globular, and deeply
furrowed. Shaft thread-shaped, curved at the end, and without the blossom.
Summit obscurely four-cornered.
Native of the Cape of Good Hope.
Flowers from April till July.
REFERENCE.
1. A Leaf, magnified.
2. The Empalement.
3. The Empalement, magnified.
4. The Blossom.
5. The Chives, and Pointal, one tip magnified.
6. The Pointal.
7. The Pointal, magnified.
ERICA ventricosa.
CHARACTER SPECIFICUS.
DESCRIPTIO.
REFERENTIA.
1. Calyx, et Corolla.
2. Calyx, et Bractea lente aucta.
3. Stamina, et Pistillum.
4. Stamina a Pistillo diducta; anthera una lente aucta.
5. Stylus, et Stigma, lente aucta.
SPECIFIC CHARACTER.
DESCRIPTION.
The Stem shrubby, about half a foot high, cylindrical, thickish, very
much branched, the branches bend downward, then ascend.
Leaves growing by fours, linear, pointed with a recurved base, and an
ascending point, ciliate, flat on their upper, and rolled back on their under,
surface, shining, the leaf-stems very short, and pressed to the branches.
Flowers terminal, numerous, upright, level, and in bunches; the
footstalks purple, having two small opposite floral leaves at their base.
Empalement. Cup four-leaved, erect, keel-shaped, and ciliate.
Blossom of an oblong-ovate form, narrowed toward the top, with a
slightly reflexed border, of a whitish purple, smooth, and shining.
Chives. Eight hair-like threads, nearly of a length with the blossomed,
fixed into the receptacle. Tips within the blossom small, cleft, and two-
horned at their base.
Pointal. Seed-vessel oblong and furrowed. Shaft thread-shaped, of an
equal length with the threads. Summit nearly four-cornered.
Native of the Cape of Good Hope.
In bloom from April till September.
REFERENCE.