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Applied
Knowledge in
Paediatrics
MRCPCH Mastercourse
Martin Hewitt
BSc BM MD MRCP FRCPCH
Consultant Paediatric Oncology & Paediatric Medicine
Nottingham Children’s Hospital
Nottingham University Hospital NHS Trust
Nottingham UK
Senior Theory Examiner (AKP)
RCPCH
London UK
Roshan Adappa
MB BS MD FRCPCH
Senior Attending Physician Neonatology
Sidra Medicine
Doha Qatar
Honorary Senior Lecturer Cardiff University
Cardiff UK
© 2022, Elsevier Inc. All rights reserved.

First edition 2022

No part of this publication may be reproduced or transmitted in any form or by any means, electronic or
mechanical, including photocopying, recording, or any information storage and retrieval system, without per-
mission in writing from the publisher. Details on how to seek permission, further information about the Pub-
lisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center
and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions

The book entitled figures from Levine MRCPCH Mastercourse is in the public domain.
The following figures are from MRCPCH: 2.4, 2.7-2.9, 2.11-2.13, 4.2, 4.3, 5.1, 5.2, 5.4, 8.3, 16.5, 16.10- 16.12, 18.1,
19.2, 20.1, 20.2, 23.1,23.3, 23.5-23.8, 23.11, 25.1, 25.3, 25.5, 28.5.

Notices

Practitioners and researchers must always rely on their own experience and knowledge in evaluating and
using any information, methods, compounds or experiments described herein. Because of rapid advances in
the medical sciences, in particular, independent verification of diagnoses and drug dosages should be made.
To the fullest extent of the law, no responsibility is assumed by Elsevier, authors, editors or contributors for
any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or
from any use or operation of any methods, products, instructions, or ideas contained in the material herein.

ISBN: 978-0-7020-8037-1

Content Strategist: Alexandra Mortimer

Content Project Manager: Shivani Pal

Printed in China

Last digit is the print number: 9 8 7 6 5 4 3 2 1

 Contributors

Ibtihal Abdelgadir, MB BS MD MSc Usama Al-­Kanani, MB ChB FRCEM Barbara Blackie, MD MEd FRCPC
FRCPCH PEM Dip Senior Attending Physician Paediatric
Attending Physician Paediatric Attending Physician Paediatric Emergency Medicine
Emergency Medicine Emergency Medicine Sidra Medicine
Sidra Medicine Sidra Medicine Assistant Professor
Assistant Professor Doha Qatar Weill Cornell Medicine
Weill Cornell Medicine Doha Qatar
Doha Qatar Louise Allen, MB BS MD FRCOphth
Consultant Paediatric Ophthamology Gillian Body, BSc MB BS MMedSci
Roshan Adappa, MB BS MD FRCPCH Cambridge University NHS Trust FRCPCH
Senior Attending Physician Neonatology Associate Lecurer Consultant Paediatric Medicine
Sidra Medicine University of Cambridge Noah’s Ark Children’s Hospital for Wales
Doha Qatar Cambridge UK Cardiff UK
Honorary Senior Lecturer Cardiff
University Roona Aniapravan, MB BS FRCPCH Subarna Chakravorty, MB BS PhD
Cardiff UK Attending Physician Paediatric MRCPCH FRCPath
Emergency Medicine Consultant Paediatric Haematology
Sudhakar Adusumilli, MB BS DCH, Sidra Medicine King’s College Hospital
MRCP FRCPCH Assistant Professor Weill Cornell Medical London UK
Senior Attending Physician Paediatric College
Emergency Medicine Doha Qatar Vince Choudhery, MB ChB FRCS
Sidra Medicine MRCPCH
Doha Qatar Karen Aucott, MB ChB MRCPCH Consultant Paediatric Emergency
Consultant Paediatric Medicine Medicine
Shakti Agrawal, MB BS MRCP Nottingham Children’s Hospital Royal Hospital for Children
MRCPCH Nottingham University Hospital NHS Glasgow UK
Consultant Paediatric Neurology Trust
Birmingham Children’s Hospital Nottingham UK Angus Clarke, BM BCh DM MA FRCP
Birmingham UK FRCPCH
Ramnath Balasubramanian, Professor Medical Genetics
Juliana Chizo Agwu, MB BS MSc MB BS DNB MRCPCH University of Cardiff
MRCP FRCPCH PCME Consultant Paediatric Nephrology Cardiff UK
Consultant Paediatric Medicine & Birmingham Children’s Hospital
Diabetes Birmingham UK Lucy Cliffe, MB ChB FRCPCH
Sandwell and West Birmingham NHS Consultant Paediatric Immunology &
Trust Srini Bandi, MB BS MD MSc FRCPCH Infectious Diseases
West Bromwich UK Consultant Paediatric Medicine Nottingham Children’s Hospital
Leicester Royal Infirmary Nottingham University Hospital NHS
Rulla Al-­Araji, MB ChB MRCPCH Leicester UK Trust
Consultant Paediatric Gastroenterology Nottingham UK
Great Ormond Street Hospital for Sybil Barr, MB BCh MSc FRCPCH
Children Senior Attending Physician Neonatology
London UK Sidra Medicine
Doha Qatar

iii
 Contributors

Madhumita Dandapani, MB BS Richard Hain, MB BS MSc MD Elisabeth Jameson, BSc MB BCh

PhD MRCPCH FRCPCH FRCPE DipPalMed PGCert FHEA MSc MRCPCH
Clinical Associate Professor Consultant Paediatric Palliative Care Consultant Paediatric Metabolic Disease
University of Nottingham Children’s Hospital for Wales Willink Biochemical Genetics Unit
Honorary Consultant Paediatric Oncology Cardiff UK St Mary’s Hospital
Nottingham Children’s Hospital Honorary Professor Clinical Ethics Manchester UK
Nottingham University Hospital NHS Trust Swansea University
Nottingham UK Swansea UK Sundaram Janakiraman, MB BS
FRCPCH
Corinne de Sousa, BSc MB BS Martin Hewitt, BSc BM MD MRCP Consultant Neonatology
MRCPsych FRCPCH James Cook University Hospitals
Consultant Child Psychiatry Consultant Paediatric Oncology & Middlesbrough
Hopewood CAMHS Paediatric Medicine Associate Lecturer
Nottinghamshire Healthcare NHS Trust Nottingham Children’s Hospital Newcastle University UK
Nottingham UK Nottingham University Hospital NHS Trust
Nottingham UK Agnieszka Jarowska-­Ganly, BSc
David Devadason, MB BS FRCPCH Senior Theory Examiner (AKP) MSc
Consultant Paediatric Gastroenterology RCPCH Pychometric Analyst
Nottingham Children’s Hospital London UK RCPCH
Nottingham University Hospital NHS Trust London UK
Nottingham UK Syed Haris Huda, MB BS IMRCS
FRCEM ACEP Nicola Jay, MB BS BSc MSc MRCPCH
Yazeed Eldos, MD Attending Physician Paediatric PGDip Ethics
Senior Attending Physician Paediatric Emergency Medicine Consultant Paediatric Allergy
Emergency Medicine Sidra Medicine Sheffield Children’s Hospital
Sidra Medicine Doha Qatar Sheffield UK
Doha Qatar
Matthew Hurley, BSc MB BCh PhD Tawakir Kamani, MD MSc DOHNS
Elhindi Elfaki, MB BS, FRCPCH MRCPCH FRCS
Senior Attending Physician Neonatology Consultant Paediatric Respiratory Consultant Surgeon Ear Nose Throat
Sidra Medicine Medicine Nottingham University Hospital NHS Trust
Doha Qatar Nottingham Children’s Hospital Nottingham UK
Nottingham University Hospital NHS Trust
Lucy Foard, BSc MBPsS Nottingham UK Rohit Kumar, MB BS MRCPCH
Psychometric Analyst Consultant Neonatology
RCPCH Amna Hussain, BSc MB BS MRCPCH James Cook University Hospital
London UK Attending Physician Paediatric South Tees Hospitals NHS Trust
Emergency Medicine Middlesbrough UK
Sohail Ghani, MB BS FCPS FRCPCH Sidra Medicine
FRCPE Doha Qatar Mithilesh Lal, MD MRCP FRCPCH
Attending Physician Paediatric Consultant Neonatology
Emergency Medicine Muhammad Islam, MB BS MRCEM James Cook University Hospital
Sidra Medicine Attending Physician Paediatric South Tees Hospitals NHS Trust
Doha Qatar Emergency Medicine Middlesborough UK
Sidra Medicine Senior Theory Examiner
Graeme Hadley, MB ChB DCH Doha Qatar RCPCH
FRCPCH London UK
Consultant Paediatric Medicine and Nadya James, BSc MB BS MRCPCH
Emergency Mediine Consultant Community Paediatrics Prakash Loganathan, MB BS MD
Rondebosch Medical Centre Nottingham Children’s Hospital Consultant Neonatology
Cape Town South Africa Nottingham University Hospital NHS Trust James Cook University Hospital
Nottingham UK South Tees Hospitals NHS Trust
Middlesbrough UK

iv
 Contributors

Kah Yin Loke, MB BS MMed(Paed) Moriam Mustapha, BSc RD Jane Ravescroft, MB ChB MRCGP
MD MRCP FRCPCH Neonatal Dietitian MRCP
Associate Professor Paediatric Sidra Medicine Consultant Paediatric Dermatology
Endocrinology Doha Qatar Nottingham Children’s Hospital
National University of Singapore Nottingham University Hospital NHS Trust
Singapore Vrinda Nair, MB BS MD FRCPCH Nottingham UK
Consultant Neonatology
Andrew Lunn, BM MRCPCH James Cook University Hospital Muthukumar Sakthivel, MB BS
Consultant Paediatric Nephrology South Tees Hospitals NHS Trust MD FRCPCH
Nottingham Children’s Hospital Middlesborough UK Attending Physician Paediatric
Nottingham University Hospital NHS Trust Emergency Medicine
Nottingham UK Khuen Foong Ng, MB BS MRCPCH Sidra Medicine
Registrar Paediatric Infectious Diseases & Assistant Professor
Prashant Mallya, MB BS MD Immunology Weill Cornell Medicine
MRCPCH Bristol Royal Hospital for Children Doha Qatar
Consultant Neonatology University Hospitals Bristol NHS
James Cook University Hospital Foundation Trust Nafsika Sismanoglou, Ptychio
South Tees Hospitals NHS Trust Bristol UK Iatrikes, (MD) MSc MRCPCH
Middlesborough UK Registrar Paediatric Immunology &
Amitav Parida, BSc MB BS MRCPCH Allergy
Stephen Marks, MB ChB MD MSc Consultant Paediatric Neurology Northern General Hospital
MRCP DCH FRCPCH Birmingham Children’s Hospital Sheffield, UK
Reader Paediatric Nephrology Birmingham UK
University College London Elisa Smit, MD FRCPCH
Consultant Paediatric Nephrology Sathya Parthasarathy, MB BS Consultant Neonatology
Great Ormond Street Hospital for MRCOG Cardiff and Vale University Health Board
Children Consultant Obstetrician (Fetal Medicine) Clinical Senior Lecturer Cardiff University
London UK James Cook University Hospital Cardiff UK
South Tees Hospitals NHS Trust
Eleanor Marshall, BSc MB BCh PhD Middlesbrough UK Alan Smyth, MA MB BS MD MRCP
MRCPCH FRCPCH
Consultant Paediatric Allergy Colin Powell, MB ChB MD DCH Professor of Child Health
Sheffield Children’s Hospital FRACP MRCP FRCPCH University of Nottingham
Sheffield UK Senior Attending Physician Paediatric Honorary Consultant Paediatric
Emergency Medicine Respiratory Medicine
Katherine Martin, BSc MB ChB Sidra Medicine Nottingham University Hospitals NHS
MRCPCH Doha Qatar Trust
Consultant Paediatric Neurodisability Honorary Professor of Child Health Nottingham UK
Nottingham Children’s Hospital Cardiff University
Nottingham University Hospital NHS Trust Cardiff UK Sibel Sonmez-­Ajtai, MD MSc
Nottingham UK MRCPCH Dip Clin Ed
Andrew Prayle, BMedSci BM BS Consultant Paediatric Allergy
Flora McErlane, MB BCh MSc PhD MRCPCH DipStat Sheffield Children’s Hospital
MRCPCH Clinical Associate Professor Sheffield UK
Consultant Paediatric Rheumatology Paediatric Respiratory Medicine
Great North Children’s Hospital University of Nottingham Jothsana Srinivasan, MB BS DCH
Newcastle upon Tyne UK Nottingham UK MRCPCH
Consultant Paediatric Medicine &
Nazakat Merchant, MBBS MD DCH Ruth Radcliffe, BMedSci BM BS Paediatric Dermatology
FRCPCH MRCPCH Nottingham Children’s Hospital
Consultant Neonatology Consultant Paediatric Medicine Nottingham University Hospital NHS Trust
West Hertfordshire NHS Trust University Hospitals of Leicester NHS Nottingham UK
Hon Senior Clinical Lecturer Trust
King’s College London Leicester UK
London UK

v
 Contributors

Richard Stewart, MB BCh BAO MD Sunitha Vimalesvaran, MB BS Lisa Whyte, MB ChB MSc MRCPCH
FRCS FRCS(Paed) MSc MRCPCH Consultant Paediatric Gastroenterology
Consultant Paediatric Surgery GRID Registrar Paediatric Hepatology Birmingham Children’s Hospital
Nottingham Children’s Hospital King’s Colle Hospital Birmingham UK
Nottingham University Hospital NHS Trust London UK
Nottingham UK Kate Adel Wilson, MNutrDiet BSc
Joanna Walker, MBE BA FRCP Dietitian
Amy Taylor, BMedSci MB ChB FRCPCH Sidra Medicine
MRCPCH Consultant Paediatric Endocrinology Doha Qatar
Consultant Paediatric Neurodisibility Portsmouth Hospitals University NHS
Nottingham Children’s Hospital Trust Damian Wood, MB ChB DCH FRCPCH
Nottingham University Hospital NHS Trust Portsmouth UK Consultant Paediatric Medicine
Nottingham UK Senior Theory Examiner (AKP) Nottingham Children’s Hospital
RCPCH Nottingham University Hospital NHS Trust
Robert Tulloh, BA BM BCh DM London UK Nottingham UK
FRCPCH FESC
Professor Congenital Cardiology Timothy Warlow, MB ChB, BMedSc,
University of Bristol FRCPCH DipPallMed
Consultant Congenital Cardiology Consultant Paediatric Palliative Medicine
University Hospitals Bristol and Weston University Hospitals
NHS Trust Southampton UK
Bristol UK

vi
 Acknowledgements

The editors would like to thank the following individuals for their helpful comments on the text or
their contribution of images and clinical scenarios.
Dr Gillian Body
Dr Will Carroll
Dr Mark Fenner
Dr Amy Kinder
Sheran Mahal (Question Bank and Quality Assurance Manager RCPCH)
Dr Eloise Shaw
Professor Harish Vyas
Dr Joanna Walker
Dr David White
Dr Nigel Broderick provided many of the radiological images and appropriate explanations of
the appearances.
We are also grateful to Sue Hampshire (Director of Clinical and Service Development Resuscita-
tion Council UK) for her support and the permission to use the management flow-­charts produced
by the Resuscitation Council UK.
Some of the images used in this book are taken from MRCPCH Mastercourse (volumes 1 and 2),
edited by Professor Malcolm Levene published by Churchill Livingstone/Elsevier in 2007. We are
grateful to the many paediatricians who sourced the images in that publication and trust that the
images continue to contribute to their educational aims.
Martin Hewitt
Roshan Adappa

vii
 Foreword

This book forms part of the Mastercourse in Paediatrics series produced by the Royal College of
Paediatrics and Child Health with each book aimed at covering the topics outlined in the relevant
RCPCH examination syllabus. It has been written by experienced specialist authors and outlines
core information of presentation, assessment and management of conditions affecting all systems
plus information on ethics, UK law, clinical governance and evidence-­based paediatrics. Although
written for candidates preparing for the Applied Knowledge in Paediatrics examination, the book
will also provide useful information and knowledge for the practicing paediatrician.
Supporting children and young people and helping them achieve their full potential requires
many skills. These include the ability to engage with the patient and their carers, the need to assess
the extent and type of problems presented and the knowledge to provide current and effective
treatments.
The training of a paediatrician must, therefore, aim to develop these skills and ensure a sound
knowledge of clinical conditions and their management. The practicalities of such management also
require the recognition of the urgency and priority of any proposed investigation and treatment.
This book covers many of these important topics.
The authors have provided a presentation of many of the common conditions seen in clinical
practice at an appropriate level for the paediatric trainee. It is well recognised, however, that such
basic knowledge requires continued revision. Consequently, every encounter with a child or young
person must be seen as an opportunity for the paediatrician to learn and improve their understand-
ing of the patient and their family, the problem presented and the appropriate management for that
problem.
This book, therefore, contributes to that growth of clinical skills and professional development
of the paediatric trainee and will help them as they prepare for the AKP exam.
Dr Camilla Kingdon
President of the Royal College of Paediatrics and Child Health

viii
 Preface

This book, Applied Knowledge of Paediatrics: MRCPCH Mastercourse, has been written specifically for
trainees in paediatrics who have around 18–24 months of clinical experience and who may be pre-
paring for the Applied Knowledge in Practice (AKP) examination. It forms part of the Mastercourse
in Paediatrics series that was established by the RCPCH and joins the Science of Paediatrics book
edited by Lissauer and Carroll. Both books are written with the prime aim of helping trainees pre-
pare for specific RCPCH theory examinations but they will also be of value to paediatric trainees as
part of their everyday practice.
Membership of the Royal College of Paediatrics and Child Health (MRCPCH) is a postgraduate
qualification in Paediatric Medicine that is recognised in the UK and internationally. The award of
the qualification indicates that a trainee has achieved a high standard of practice and is able to start
Higher Specialist Training.
The AKP examination assesses the candidate’s knowledge of the presentation, investigation and
management of a wide range of conditions affecting children and young people. This level of under-
standing comes from clinical exposure to patients, reading about the details of the clinical condi-
tions presented and taking the opportunity to discuss issues with experienced colleagues.
The chapters in Applied Knowledge of Paediatrics cover all the systems and each starts with the
points listed in the RCPCH AKP syllabus. The subsequent chapter was then written by the specialist
authors to ensure these topics were addressed.
Within each chapter there are Practice Points that capture important issues relevant to clinical
practice or that may include explanations using examples.
Clinical Scenarios are also presented and are based on known, but modified, clinical stories to
outline some of the issues that may present themselves to the clinician. Some of the issues presented
are not resolved and so reflect the reality of current practice.
At the end of each chapter there are Important Clinical Points that provide a list of some of the
significant points raised in the chapter.
Images are used throughout the chapters. These will demonstrate many important features that
may appear in the examination but they should also act as a prompt to the reader to seek out further
examples of the appearances shown. The adage ‘One swallow doesn’t make a summer’ could be
adapted to make the point that ‘One image doesn’t capture all the relevant features’.
Each chapter finishes with a short list for Further Reading to allow the reader to explore reviews
and topics in more detail. Many chapters have drawn information from current guidelines pub-
lished by the National Institute for Health and Care Excellence (NICE). Clearly these guidelines may
change over time and it is the responsibility of every clinician to ensure that the most up-­to-­date
version is consulted.
The first chapter provides some advice on preparing for the AKP examination including some
insight into the process of producing the actual exam papers, assessing the questions and the post-­
examination review. The aim is that by understanding how the papers are constructed from items in
the RCPCH Question Bank and how the results are reviewed after the exam, the candidate will gain
some understanding of the structure of the examination.
The final chapter provides 50 AKP exam-­style questions presented in random order along with
itemised answers that aim to provide clarification on how answers can be assessed and the correct
ones chosen. The reader may wish to use this as a practice examination but must remember that

ix
 Preface

questions in the real examination have different weightings allocated depending on various factors
such as length, complexity and format.
We would like to thank all the authors of each of the chapters for sharing their knowledge and
expertise, and for their understanding as we made changes and requested further reviews of their
text.
Our thanks also to Alexandra Mortimer, Shivani Pal and the larger team at Elsevier for their sup-
port and guidance during the production of this book.
Finally, our thanks must go to our families for their patience and tolerance as we committed time
to working on this project.
We hope the book proves valuable to all trainees and contributes to the improvement in the care
and treatment of the many children who will come under their care.

Martin Hewitt
Roshan Adappa

x
 Chapter |1|
Preparing for the AKP exam
Martin Hewitt

After reading this chapter you should understand: Preparation

• the structure and format of the AKP examination
• the range of topics covered by the syllabus
Candidates can sit the RCPCH theory examinations in
• the style of questions presented in the
any order although most progress from FoP though TaS
examination
• how each question is produced and reviewed to the AKP exam. After six attempts, further attempts are
• advice on some aspects of examination preparation only permitted if evidence of further study is provided.
The AKP exam is usually first attempted by trainees who
have accrued at least 12 months of paediatric training.
However, for those in UK training schemes, there is a pres-
sure to complete the theory exams during 3 years of full-­
The Applied Knowledge in Practice (AKP) examination time training as failure to do so will usually require an
is one of the three theory exams that must be passed extension of training time. The AKP exam assesses clinical
before a candidate is allowed to present themselves for knowledge and decision-­making skills, and an exposure to
the clinical examination—Foundation of Practice (FoP) a broad range of general and specialty clinical paediatrics
and Theory and Science (TaS) being the other two. Pass- is therefore needed.
ing all four examinations leads to the award of Mem- This is a UK examination and assesses understanding
bership of the Royal College of Paediatrics and Child of UK laws, expectations and clinical practice. Question
Health. writers aim to produce questions that can be supported
This chapter will describe the exam format and so by published evidence, national guidelines of accepted
allow the candidate to prepare in an appropriate man- practice and information in established and respected text-
ner. Some details of the exam may change over time and books and journals.
it is therefore imperative that the candidate consults the It is important to remember that much of the AKP
RCPCH exam website at an early stage to identify possible exam is aimed at assessing clinical understanding of pae-
updates and new advice. diatric medical practice encountered by candidates dur-
This book is not a comprehensive paediatric textbook. ing their normal daily duties. Questions will ask about
It does, however, aim to cover practically all of the topics the presentation of clinical problems, possible differen-
outlined in the RCPCH Examination Syllabus and there- tial diagnoses, appropriate common investigations and
fore forms the basis of questions in the examination. the interpretation of results. Answers to other questions
Although the syllabus and examination aim to cover the will require a knowledge of management and long-­term
main topics of clinical paediatric practice that a Specialist consequences of a broad range of conditions. The exami-
Trainee with 18 months of experience might encounter, it nation will also test the ability of candidates to establish
should also be expected that some less-­common condi- clinical priorities and ensure that time-­critical decisions
tions will appear. A paediatrician should be able to iden- are recognised and acted upon. All paediatricians will rec-
tify uncommon conditions within common presentations, ognise that care occurs within the context of the family,
which is a point illustrated by the phrase “all that wheezes and therefore an understanding of professional and legal
isn’t asthma.” obligations is necessary and assessed in the examination.

1
 Chapter |1| Preparing for the AKP exam

Candidates will obviously need to build their knowledge for example, most candidates choose an answer differ-
base by further background reading. ent from the allocated correct answer, this suggests that
the phrasing of the question is ambiguous and points
to more than one acceptable valid answer. The panel
Examination structure would review that question in detail and, if it is agreed
that phrasing of any part of the question is ambiguous
and therefore unfair, then the question is removed from
The examination is a curriculum-­driven, computer-­based that examination. The question is sent for review rather
assessment that takes place three times each year. There than returned to the question bank.
are two separate papers sat on the same day with each
paper lasting 2.5 hours. In total there are 120 questions
across the two papers. The exam does not use negative Question types
marking—a wrong answer scores zero. The allocation of
topics across the various syllabus headings is set by the
“Theory Examination Blueprint” that allows specific map- There are different question types used in the examina-
ping of questions to the syllabus and aims to provide a tion. Examples of these can be seen on the RCPCH web-
balanced selection of questions across the entire syllabus site and in Chapter 35 of this book. Some information on
in each exam. each question type is offered here.

Single best answer (SBA)

Question journey
Most of the questions in the exam follow this format. The
stem may include a clinical history, examination, results
The development of questions used and the building of and images and the question is posed. There are then five
each exam involves many separate steps to ensure that answers offered with only one being correct. The most
the questions are relevant and current and that the important point for a candidate to understand is that all
exam is well balanced. Before appearing in an exam, answers shown will be plausible but only one is the most
each and every question will have been individually appropriate for the question asked. If the candidate reads
scrutinised by at least nine separate, experienced pae- a question and concludes that all answers are correct then
diatricians and will be reviewed again by a panel of the question is a good example of the single best answer
another six to eight paediatricians after the exam has format. In this situation, the candidate is advised to re-­
taken place. read the question and clarify the exact phrase of the ques-
The questions are generated at Question Setting tion posed. Examples of the different types of phrasing
Group meetings that occur throughout the year at vari- are:
ous locations in the UK and abroad. The meetings are ..the next most appropriate step in management
organised by RCPCH staff and are open to all paedia- ..the most appropriate initial management
tricians who already hold the MRCPCH diploma. Sen- ..the treatment to be given immediately
ior exam facilitators are part of the meeting and guide ..the test which will provide a diagnosis
small groups to create the questions. Each question ..the most likely diagnosis
is then assessed by the Senior Theory Examiner for For example, a question may describe a child present-
AKP and is reviewed again by two senior clinicians at ing in extremis to the emergency department and, in real
RCPCH Examination Board. Following this review, the life, multiple interventions will be undertaken. Each of
approved question is placed in the question bank for these interventions will be appropriate and necessary and
future use. therefore will be listed in the answer list. The question
Following every exam, and before the results are may then ask which one intervention from this list must
finalised, a panel of paediatricians meet with the be undertaken as a priority.
RCPCH psychometrician at the “post examination
Angoff meeting” and review the performance of every
question. This meeting scores the level of difficulty
Multiple best answer (MBA)
for each question and reviews any possible discrepan- Some questions will ask for more than one answer such
cies or problem questions identified in the exam. If, as needing two investigations to support a diagnosis. The

2
 Preparing for the AKP exam Chapter |1|

questions do not ask for more than three answers and the commonly in certain ethnic groups or in certain geograph-
list of options provided will be up to a maximum of 10 ical locations.
answers available. An awareness of the indications, contraindications and
long-­term consequences of some of the drugs adminis-
tered to children is important. Candidates are advised to
Multipart question (MPQ) use the BNFc during their normal working day as part of
This question structure follows the format of the single best their revision and to look at contraindications and com-
answer but there are usually two questions joined to the ini- mon side effects.
tial scenario and each question is independent of the other. Many questions will contain images such as clinical
The answer to part one does not give a clue to the answer of photos, radiographs and ECGs.
part two. Clinical photographs will cover a range of features
including specific syndromes and disease-­related abnor-
malities. The ability to identify a series of clinical features
Extended matching questions (EMQ) in a child and recognise the underlying syndrome is a skill
This format provides an introductory statement that that many geneticists and paediatricians take many years
explains the general topic for the question. Examples to develop. However, recognising the features of a small
would include cardiac diagnoses, drugs for epilepsy or group of syndromes is required for the AKP exam and
investigations for hypernatraemia. these are presented in this book. Trainees are advised to
The question then presents the first statement or clini- review as many images as possible of these syndromes
cal scenarios followed by a list of 10 potential answers. to ensure they can identify the major features. Similarly,
The second scenario is then presented followed by the wherever a condition which has recognised clinical fea-
same list of 10 answers and finally the third question with tures is described in the text, the candidate should seek
the same 10. It is possible that one of the answers may be out example images or descriptions.
chosen for more than one of the questions—each ques- There are questions that require the ability to inter-
tion is independent of the other two. pret radiographs although it is accepted practice that it
is the radiologist who will provide the definitive opin-
ion and final report. Trainees, however, do need to iden-
Detailed advice tify common radiological abnormalities that require
Some questions may seem to have one or two obvious an immediate response and management such as the
answers and the candidate needs to look for further clues presence of a pneumothorax, a pneumonia, or necrotis-
in the question stem to support one or the other. For ques- ing enterocolitis. MRI and CT scans must be reported
tions where there is no obvious answer, one approach by radiology staff but an AKP candidate must have an
would be to ask the ‘reverse question’ and identify those understanding of the common abnormalities to allow
answers which clearly do not fit the clinical scenario in the explanation of the findings to patients, carers and col-
question stem. Having removed these answers, the can- leagues usually following a discussion with the radiolo-
didate can then work on those remaining to identify the gist. Trainees should attend as many radiology meetings
appropriate and correct answer. as possible and be prepared to ask radiologists to explain
All questions in the examination that include results important features.
of laboratory investigations will also show the normal
ranges for each of the listed test. These ranges may be
slightly different from those used at the candidate’s insti-
Evidence-­based paediatrics
tution but these are the ones agreed for examination. In The AKP examination will contain two questions about
practice, this is not a problem as the provided results, evidence-­based practice in each paper. The questions usu-
where appropriate, are obviously abnormal or obviously ally present information from a published paper but with
normal. the methodology and results summarised. The data can
The candidate should read the stem very carefully be complex and each answer should be compared in turn
as it often contains specific details to guide the candi- with the given results to determine whether the answer
date towards particular conditions that may occur more statement can be supported.

3
 Chapter |1| Preparing for the AKP exam

IMPORTANT CLINICAL POINTS

Marking Results
•  arks awarded for each question are different and
m • normal ranges are shown for each test
weighted for complexity
• a wrong answer scores zero Images
• r eview as many images as possible of syndromes
Single best answer • review as many images as possible of clinical signs and
• understand the exact phrase of the question being asked described lesions

Multi-­part question Radiographs

• answer to each question is independent of any other part • attend as many radiology meetings as possible

Extended matching question

• t he same answer may apply to more than one of the
three questions

Further reading

RCPCH Theory Examinations. https://www.rcpch.ac.uk/resources/

theory-­examinations-­structure-­syllabus.

4
 Chapter |2|
Neonatology
Authors: Mithilesh Lal, Elisa Smit, Nazakat Merchant

Contributions from: Sunitha Vimalesvaran, Vrinda Nair, Prakash Loganathan, Prashant Mallya, Rohit Kumar,
Janakiraman Sundaram, Sathya Parthasarathy

After reading this chapter you should be able to diagnose age in the second half of pregnancy. This definition does
and manage: not distinguish the normal, constitutionally small fetus
• birth injury (small for gestational age [SGA]) from the small fetus
• short and long-­term consequence of preterm birth whose growth potential is restricted. The latter fetus is
• common medical conditions at increased risk of perinatal morbidity and mortality
• common surgical conditions whereas the former is not.
• congenital anomaly   
• common postnatal problems IUGR may be:
and Symmetrical IUGR (20%–30% of small fetuses) that re-
• know the effect of prenatal and perinatal events on
fers to a growth pattern in which all fetal organs are
neonates
decreased proportionally and is thought to result from
a pathological process manifesting early in gestation.
Asymmetrical IUGR (70%–80 % of small fetuses) where
Antenatal assessment of fetal there is a relatively greater decrease in abdominal size
growth (liver volume and subcutaneous fat tissue) than in head
circumference and is thought to occur late in gestation.  

Antenatal assessment of the mother and fetus requires a

comprehensive history and examination, investigations Screening for intrauterine growth
for potential congenital infections and chromosomal
anomalies and ultrasound monitoring. Ultrasonography restriction
will assess the breathing pattern, muscle tone, body move-
ment and amniotic fluid volume. The main aim is to iden-
tify early intrauterine growth restriction—IUGR—(also
Fundal height (FH) measurement
referred to as fetal growth restriction [FGR]). Measurement of the distance between the upper edge of
IUGR is defined as the failure of the fetus to achieve its the pubic symphysis and the top of the uterine fundus is
full growth potential and could be due to maternal, fetal performed during antenatal care to detect IUGR.
or neonatal causes. It is the biggest risk factor for stillbirth,
perinatal and neonatal morbidity and mortality.
Selective ultrasonography
Indications for a growth scan are:
Definition and classification
• first FH measurement below 9th centile at between 26–
The most common obstetric definition of IUGR is an 28 weeks
estimated weight below the 9th centile for gestational • no increase in sequential measurements

5
 Chapter |2| Neonatology

Amniotic fluid volume assessment can identify preg-

Table 2.1 Indications for serial growth measurements nancies with the most severe oligohydramnios as these
during pregnancy.
have high rates of perinatal mortality, congenital anoma-
lies and IUGR.
Current Past medical Past obstetric Doppler velocimetry looks for abnormal doppler wave
pregnancy history history forms in maternal uterine arteries and fetal vessels (umbil-
maternal age over chronic previous birth ical arteries, middle cerebral arteries, ductus venosus) that
40 years hypertension weight <9th will indicate poorer neonatal outcomes.
centile Abdominal circumference is the most sensitive single
biometric indicator of IUGR and is usually performed at
maternal smoking diabetes previous stillbirth
approximately 34 weeks of gestation.
drug misuse renal
impairment
maternal BMI > 35 Perinatal events and birth injury
multiple
pregnancy Hypoxic-­ischaemic encephalopathy
hypertension or Hypoxic-­ischaemic encephalopathy (HIE) is the result of
preeclampsia a significant lack of oxygen and reduced blood flow to the
unexplained APH fetal brain and other organs during labour and delivery.
concerns related Causes include:
to growth • underlying conditions producing circulatory compro-
measurements mise in the mother
•  utero-­
placental problems—umbilical cord prolapse,
placental abruption
• fetal conditions—cardiac failure, feto-­maternal haemor-
rhage
• sequential measurements do not follow the expected Sometimes the insult is more prolonged and chronic in
growth nature, but both acute and chronic asphyxia can lead to
• sequential measurements cross centiles in an upward HIE.
direction Infants with HIE are often in poor condition at birth
All women should be assessed at booking for risk fac- and invariably require resuscitation, and the condition
tors to identify those who need increased surveillance. is described as mild, moderate or severe encephalopathy
Some will be at increased risk of developing fetal growth (see Sarnat stages in Table 2.1).
restriction because of factors in the current pregnancy, Relevant information contributing to the diagnosis of
in the past medical history or the past obstetric history. HIE includes:
Those women with such risk factors will need serial scans • evidence of fetal distress
at least every 3 weeks from 26–28 weeks until delivery •  sentinel events—cord prolapse, antepartum haemor-
(Table 2.1). rhage, shoulder dystocia
• low Apgar scores
• placental report indicating dysfunction
Growth indices • Kleihauer-­Betke test—if feto-­maternal haemorrhage is
Estimated fetal weight is the most common method of suspected
identifying the growth-­restricted fetus as it combines mul-
tiple biometric measurements including abdominal cir-
Investigations
cumference, biparietal diameter, head circumference and
femur length. •  lood gas—identify degree of acidosis
b
Customised growth charts are used to plot both fun- • electrolytes, glucose, calcium if seizures
dal height measurements obtained during clinical exami- • amplitude integrated electroencephalography (aEEG)
nation and estimated fetal weight following an ultrasound • infection screen
examination. They are customised to each individual tak- • cranial ultrasound (oedema or areas of parenchymal or
ing into account the height, weight, ethnicity, and parity basal ganglia/thalamic damage)
of the mother. • doppler studies of cerebral flow velocity

6
 Neonatology Chapter |2|

should be avoided by an adequate glucose infusion rate.

Table 2.2 Stages of hypoxic-­ischaemic encephalopathy Liver dysfunction can cause coagulopathy and severe
(Sarnat)
bleeding, which may require treatment with blood and
clotting products. Hepatic and renal drug clearance is
mild – moderate – severe – stage 3 often impaired and doses may need adjustment and
stage 1 stage 2 monitoring.
hyperalert lethargic coma
eyes wide reduced tone weak or absent Important sequelae
open respiratory drive
Moderate and severe HIE have a high mortality and mor-
does not diminished no response to stimuli bidity rate and many who do survive have significant
sleep brainstem intellectual disability and poor motor function. Despite
reflexes therapeutic cooling, the combined outcome of death or
(pupil/gag/ disability at 18–24 months is around 50% with 25% mor-
suck) tality and 25% disability.
irritable clinical seizures floppy Several features can help to define the prognosis and
seizures diminished brainstem those with poor feeding and decreased tone at 2 weeks
absent reflexes (pupil/gag/ will have a poor neurodevelopmental outlook. MRI of the
suck) brain between day 5–14 can identify abnormal signals in
the posterior limb of the internal capsule, which again
usually diminished tendon
is indicative of a poor outcome. Epilepsy, vision or hear-
lasts reflexes
ing difficulties, as well as behaviour and learning difficul-
<24
ties are all recognised consequences of hypoxic ischaemic
hours
encephalopathy.
EEG severely abnormal

Brachial plexus injury

Brachial plexus palsy is flaccid paralysis of the upper limb
Treatment and managment seen at birth, due to stretching, rupture or avulsion of
Supportive treatment is required for those babies with some, or all, of the cervical and first thoracic nerve roots
multisystem involvement including ventilation in view of (Table 2.3).
poor respiratory effort. Periods of hypo-­and hyperoxia as Risk factors:
well as hypo-­and hypercapnia may develop and indicate a • shoulder dystocia
worse neurological outcome. • birth weight over 4 kg
Therapeutic hypothermia is the only neuroprotec- • maternal diabetes—associated with macrosomia
tive treatment effective in term infants with moderate • breech delivery—difficulty in extracting the trailing
or severe HIE. This involves a reduction of core body arm
temperature to 33.5°C for 72 hours followed by a slow • instrumental delivery
rewarming phase (0.5°C/hour increase) over 6 hours.
The treatment must be initiated within 6 hours after Other findings which may be seen are:
birth for it to be beneficial. • f racture of humerus or clavicle with crepitus or swell-
Infants with HIE may develop overt seizures, whilst ing
others will have seizures on aEEG without a clinical com- • Horner’s syndrome—sympathetic nerve damage
ponent—’electrical seizure activity’. There is no clear con- • respiratory distress—phrenic nerve injury resulting in
sensus on management, but many would treat seizures diaphragmatic paralysis
with a duration of over 3 min or greater than 3 seizures in • encephalopathy—associated hypoxic ischaemic event
1 hour.
Cardiac dysfunction may require inotropic support
Investigations
and the issue may become evident during cooling when
many infants show a hypothermia-­induced bradycardia of Usually limited to chest x-­ray to identify a clavicle or
around 70–90 bpm. humeral fracture or diaphragmatic palsy. Nerve conduc-
Transient renal impairment is common and careful tion studies or MRI may be required if surgical interven-
fluid management is required. Potential hypoglycaemia tion is indicated.

7
 Chapter |2| Neonatology

Table 2.3 Types of brachial palsy and diagnostic features

Erb’s palsy Klumpke’s palsy total palsy

nerve roots C5, 6 and sometimes T1 C8, T1 C5-­T1
clinical presentation weakness of arm weakness of intrinsic weakness of
decreased arm movements muscles of hand entire arm
arm is adducted and internally rotated with elbow leading to ‘claw
extended, hand’
forearm is in pronation and wrist is flexed (‘waiter’s
tip’) (Figure 2.1).
Moro reflex absent present absent
biceps reflex absent present absent
radial reflex absent present absent
grasp reflex present absent absent

Treatment and management

Parents are advised of the need for careful handling of
infants in the first 1–2 weeks till the inflammation sub-
sides but after this time, a formal exercise programme
will be initiated by the physiotherapy team. About 70%
to 80% of infants make a full recovery without interven-
tion in 6 weeks to 3 months. The child should be referred
to colleagues with expertise in nerve injuries if there is no
improvement by 6 weeks.

Antenatal management of preterm

labour

Accurate identification of women in true preterm labour

allows appropriate application of interventions that can
improve neonatal outcome such as the administration of
antenatal corticosteroid therapy, prophylaxis against group
B streptococcal infection or necessary transfer to a facility
with an appropriate level of newborn care.
Risk factors for preterm labour include:
• previous preterm delivery
• multiple pregnancy
• advanced maternal age
• teenage mother
• smoking or drug abuse
• deprivation
Interventions aimed at reducing the risk of preterm
Fig. 2.1 Brachial plexus injury (Erb’s palsy) following shoulder birth include:
dystocia. Image shows internal rotation at shoulder and flexion •  education and health promotion programmes in-
of digits cluding smoking cessation, treatment of drug misuse,

8
 Neonatology Chapter |2|

maintenance of a normal body mass index and longer that clamping the cord after a good respiratory effort is
intervals between pregnancies established is more effective than time based delayed cord
• low-­dose aspirin may reduce the risk of spontaneous clamping. Positive End Expiratory Pressure (PEEP) sup-
preterm birth port has been shown to be beneficial by establishing a
• cervical cerclage placement may prolong gestation for functional residual capacity in the lungs. Routine airway
women with a history of preterm birth suction with or without meconium has no benefit and is
therefore not recommended.
Management of preterm labour
The diagnosis of preterm labour is based on clinical cri- Medical conditions in the preterm
teria of regular painful uterine contractions accompanied
by cervical dilation or effacement. Tocolytics can be used
neonate
to try and delay preterm labour so that antenatal steroids
and magnesium sulphate can be given.
Respiratory distress syndrome
Management of the high-­risk Respiratory distress syndrome (RDS) is primarily seen
in premature babies and is the result of surfactant defi-
pregnancy ciency and immature lung development and therefore
Preterm birth can result in significant health consequences in the incidence decreases with increasing gestational age.
both the short and long term. Pregnancies that are likely to Risk factors for RDS include prematurity, maternal dia-
produce infants at high risk of problems include those with: betes, absence of labour and lack of antenatal steroids.
• intrauterine growth restriction—from maternal, placen- Antenatal steroids, surfactant therapy and noninvasive
tal or fetal causes respiratory support have resulted in reduced mortality
• prolonged preterm rupture of membranes, presenting as from RDS.
infection or risk of infection or related poor lung growth The preterm infant with RDS will have tachypnoea,
• congenital malformations from syndromic association grunting, chest wall retractions, nasal flaring and ‘head
• chronic maternal illness—maternal diabetes and other bobbing’. As the condition becomes more severe the baby
medical conditions becomes cyanotic and pale and may have apnoeic epi-
• acute fetal compromise—placental abruption, cord pro- sodes.
lapse
• twin or higher order pregnancy
Differential diagnosis
Initial assessment and intervention in the delivery room:
• pregnancies at risk of difficulties should occur in a hos- • t ransient tachypnoea of newborn (TTN)
pital with a level 3 NICU. Antenatal steroid administra- • aspiration
tion for lung maturation and magnesium sulphate for • pneumonia or sepsis
neuroprotection should also be administered to the ex- • cyanotic congenital heart disease
pectant mother.
• delivery room temperature needs to be kept above 25oC
Investigations
and the use of a plastic covering for the preterm infant
will help maintain better thermal control. Each degree The chest x-­ray will show the recognised changes of RDS
below 36.5oC is associated with increased mortality in with the reticulogranular pattern (ground glass) in the
preterm babies of about 28%. lung fields, an air bronchogram and low lung volumes
(Figure 2.3).
Following birth
Treatment and management
Most preterm or term infants will not need any inter-
vention. The management outlined in the Resuscitation Antenatal steroids reduce the incidence and severity of
Council UK algorithm should be followed if intervention RDS and the consequent need for mechanical ventilation.
is required (Figure 2.2). Current recommendation is for them to be offered to all
Specific aspects of the assessment of the newborn women between 24+0 and 33+6 weeks of pregnancy who
require consideration. are at risk of preterm delivery within 7 days. The ideal
Clamping of the cord can be delayed for up to 3 min- therapeutic window for administration is when delivery is
utes in the preterm infant, although there is good evidence expected 1 to 7 days after a complete course of treatment.

9
Chapter |2| Neonatology

Newborn life support

(Antenatal counselling)
Team briefing and equipment check

Birth
Preterm Delay cord clamping if possible

Approx 60 seconds

Maintain temperature

At all times ask “is help needed”

< 32 weeks
Start clock / note time
Dry / wrap, stimulate, keep warm
Place undried in
plastic wrap +
radiant heat Assess
Colour, tone, breathing, heart rate

Ensure an open airway

Inspired oxygen
Preterm: consider CPAP
28–31 weeks 21–30%
< 28 weeks 30%
If gasping / not breathing
• Give 5 inflations (30 cm H2O) – start in air
If giving inflations, • Apply PEEP 5–6 cm H2O, if possible
start with 25 cm H2O • Apply SpO2 +/– ECG

Acceptable Reassess
pre-ductal SpO2 If no increase in heart rate, look for chest movement

2 min 65%
If the chest is not moving
5 min 85%
• Check mask, head and jaw position
10 min 90% • 2 person support
• Consider suction, laryngeal mask/tracheal tube
• Repeat inflation breaths
• Consider increasing the inflation pressure

Reassess
Titrate oxygen to achieve target saturations

If no increase in heart rate, look for chest movement

Once chest is moving continue ventilation breaths

If heart rate is not detectable or < 60 min–1

after 30 seconds of ventilation
• Synchronise 3 chest compressions to 1 ventilation
• Increase oxygen to 100%
• Consider intubation if not already done or laryngeal
mask if not possible

Reassess heart rate and chest movement

every 30 seconds

If the heart rate remains not detectable or < 60 min–1

• Vascular access and drugs
• Consider other factors e.g. pneumothorax,
hypovolaemia, congenital abormality

Update parents and debrief team

complete records

Fig. 2.2 Newborn Life Support algorithm (Reproduced with permission from the Resuscitation Council UK 2021)

10
 Neonatology Chapter |2|

High flow nasal cannulae (HFNC) have a similar effi-

cacy as other forms of noninvasive respiratory support.
Indication for use of invasive mechanical ventila-
tion include deteriorating respiratory function, recurrent
apnoea, increasing oxygen requirement and worsening of
hemodynamic status. Volume-­targeted ventilation results
in reduction of broncho-­ pulmonary dysplasia (BPD),
pneumothorax and days of ventilation when compared to
pressure limited modes. Early extubation to noninvasive
respiratory support should be planned if a ventilated neo-
nate has a good response to surfactant, but if conventional
support fails then high frequency ventilation is used as
rescue mode.
Ventilatory support aims to maintain oxygen satura-
tions between 91% to 95%, but the high oxygen flows
required leads to an increase in the incidence of retinopa-
thy of prematurity and BPD. The use of caffeine citrate in
preterm infants under 31 weeks reduces ventilation days,
the incidence of BPD and cerebral palsy.

Fig. 2.3 Chest x-­ray of 28-­week gestation neonate with

clinical signs suggestive of respiratory distress syndrome
Bronchopulmonary dysplasia
(Copyright – Dr Mithilesh Lal -­ used with permission) Bronchopulmonary dysplasia (BPD) is defined as the need
for respiratory support and supplemental oxygen at 36
weeks postmenstrual age, and its development is inversely
Surfactant can be given prophylactically to those proportional to gestational age and birth weight.
infants who are at risk of RDS, even before signs of The most important risk factors for BPD are prema-
RDS develops, and is usually given within 10–30 mins turity and low birth weight although some antenatal risk
of delivery. Rescue surfactant treatment is usually given factors include maternal smoking and maternal hyperten-
within 12 hours when specific criteria for RDS severity sion. Neonates with persistent ductus arteriosus or who
are met (e.g., receiving noninvasive support and FiO 2 need mechanical ventilation with high pressures and vol-
over 30%–40%). Both animal-­ derived and synthetic umes are also at risk of developing the condition.
surfactants are available, although the former is usually
used. New techniques for surfactant administration are
Treatment and management
minimally invasive surfactant therapy (MIST) and less
invasive surfactant administration (LISA). These tech- Antenatal interventions including the administration of
niques administer surfactant in spontaneously breathing, antenatal steroids will make a significant impact of the
nonintubated neonates by using a specialised catheter. development of BPD. If the preterm baby needs ventila-
These methods are associated with reduced incidence of tory support then noninvasive techniques should be con-
BPD, duration of invasive ventilation and incidence of sidered, whilst surfactant, caffeine and steroids will all
pneumothorax. have a protective effect although the potential side effects
of each needs to be considered.
The long-­term management of BPD will vary depend-
Mechanical respiratory support ing upon the extent of residual lung dysplasia. Those babies
Continuous positive airway pressure (CPAP) is a form with mild BPD may only require supplemental oxygen for
of noninvasive respiratory support which delivers con- a short period of time and may be discharged with a home
stant positive pressure and is delivered through fitted nasal oxygen supply. Those with more severe disease may require
devices. CPAP can be used from birth in a preterm baby continued oxygen and home ventilation supported by a
to aid respiratory effort or after extubation following a dedicated community respiratory team. Further informa-
period of ventilation. tion is presented in Chapter 17, Respiratory.
Noninvasive positive pressure ventilation (NIPPV) The aim of any such treatment will be to ensure maxi-
provides support using a face mask or prongs and pro- mal growth and development with the intention that
vides a positive pressure at preset intervals. It is thought to developing lung tissue will be sufficient to allow the child
be superior to CPAP in small neonates. to dispense with ventilator support.

11
 Chapter |2| Neonatology

Patent ductus arteriosus Necrotising enterocolitis (NEC)

The ductus arteriosus in term babies usually closes within Necrotising enterocolitis describes an inflammatory pro-
1–3 days after birth but in the preterm neonate about 40% cess in the small and large bowel that can affect preterm
fail to close spontaneously and usually leads to clinical prob- and, occasionally, term babies. The pathogenesis remains
lems. Neonates at risk of developing a clinically significant unclear but is multifactorial although there are recognised
patent ductus arteriosus (PDA) include those who are small risk factors that include IUGR, prolonged resuscitation
for gestational age, those with late onset sepsis and those with low APGAR scores and neonatal sepsis. Some fac-
who are given excessive fluids during the first days after birth. tors have been identified that reduce the risk of develop-
A persistent ductus arteriosus is defined as one that is ing NEC and these include antenatal steroids, breast milk
still present one month after the baby should have been feeding and probiotics.
born and therefore excludes preterm babies who are still In preterm neonates, NEC presents around 2–3 weeks
within their due dates. This persistence is associated with of age with features suggestive of sepsis and gastrointesti-
BPD, NEC and intraventricular haemorrhage. nal obstruction. Abdominal distension, bilious aspirates
Echocardiography is required to assess the presence and blood with mucus in the stool are all characteristic
and impact of a PDA. findings. Disease progression leads to peritonitis, hypoten-
The symptomatology of PDA is dependent on ductal sion, DIC and shock.
size, shunt volume, and the extent of a ‘circulatory steal’ A differential diagnosis would include spontaneous
effect. Clinical features of a hemodynamically significant isolated intestinal perforation, congenital bowel anoma-
PDA include: lies and a food-­protein induced enterocolitis.
•  pan-­ systolic, pan-­
diastolic murmur (continuous, ma-
chinery murmur)
Investigations
• active precordium with a wide pulse pressure
• features of cardiac failure An abdominal x-­ray will identify bowel wall oedema and
• pulmonary oedema thickening along with intramural and portal venous gas
• high oxygen requirement (Figure 2.4). Pneumatosis intestinalis is a pathognomonic
finding and indicates that the mucosal surface is damaged
Treatment and management
There is limited consensus regarding the treatment of a
PDA. Conservative management includes fluid restriction,
diuretics and the application of positive end-­expiratory
ventilatory pressure.
Prophylactic treatment refers to treating all babies
within 24 hours, without any screening and prior to any
symptoms or signs. Treatment is usually started after 6
hours but before 24 hours of birth with medications such
as indomethacin, ibuprofen or paracetamol. However,
there is no difference in mortality or composite outcome
of death or neuro-­disability at 18–36 months with this
approach. It is estimated that up to 60% of these infants
would close their duct spontaneously.
Though there are advantages of prophylactic treatment
in avoiding unnecessary drug exposure, the concern is that
the damage could have already been done by the time the
PDA becomes symptomatic. Both indomethacin and ibu-
profen have been reported to have similar effectiveness
for ductal closure (60%–80%), and ibuprofen has been
reported to have a better side effect profile. Paracetamol
can be used for ductal closure if there is contraindication
(NEC, IVH) to use indomethacin or ibuprofen.
Surgical ligation of the PDA is performed in infants Fig. 2.4 Abdominal x-­ray in a preterm neonate with
with hemodynamically significant PDA where medical necrotising enterocolitis. Air is visible below the diaphragm
treatment has failed or is contraindicated, particularly if (open arrows), on either side of the bowel wall (black triangles)
the baby remains ventilated and weaning is difficult. and outlining the falciform ligament (black arrow)
12
 Neonatology Chapter |2|

and bowel gases have tracked along the tissue planes. Sen- recognised by the onset of apnoeic episodes, seizures, or
tinel bowel loops (fixed loops) may indicate bowel necro- cardiovascular collapse with significant acidosis due to the
sis without pneumatosis. acute blood loss.

Treatment and management Treatment and management

Conservative management includes pain relief and seda- Once identified, the initial management is mainly support-
tion, cessation of all oral intake and use of parenteral ive. The appearance of posthaemorrhagic hydrocephalus
nutrition to allow resting of the bowel. Broad spectrum may require surgical intervention in the form of insertion
antibiotics with anaerobic cover for 10–14 days should of a ventricular reservoir or ventriculo-­peritoneal shunt.
be given along with circulatory and ventilatory support
if needed. Bowel perforation is the only absolute indica-
Neurodevelopmental outcome
tion for surgery in the acute phase. Failure of conserva-
tive management also warrants surgery and is considered Infants with severe GM-­IVH had increased risk of cerebral
with extensive NEC prior to impending bowel perforation. palsy, cognitive delay and moderate to severe neurode-
Surgery usually involves laparotomy with resection of velopmental impairment. Preterm babies born before 32
necrotic bowel, with or without stoma formation. weeks’ gestation are at particular risk of developing long
term neuro-­developmental problems.
Germinal matrix and intraventricular Most neonatal units will follow preterm babies under
32 weeks’ gestation until 24 months corrected age to mon-
haemorrhage (GM-­IVH) itor neurodevelopmental progress. These babies will often
Intracranial haemorrhage occurs mainly in the germinal require the ongoing support of all members of the com-
matrix or extends into ventricular system and is an impor- munity neurodevelopmental team.
tant cause for neurological morbidity in the preterm neo-
nate. The haemorrhages tend to develop within the first 6
hours after birth and almost 90% have appeared by day 4.
Periventricular leukomalacia
Consequently, perinatal and delivery room interventions Periventricular leukomalacia (PVL), or white matter injury
and early neonatal care are likely to have impact on inci- (WMI), represents one of the most common forms of brain
dence of IVH. injury in the preterm infants resulting from focal necrosis
Risk factors for intraventricular haemorrhage include and gliosis of white matter around lateral ventricles. Risk
hypoxia, severe RDS and hypotension. Practice aimed at factors include NEC, significant hypotension, neonatal sep-
preventing intraventricular haemorrhage include antenatal sis, severe hypoxia, hypocapnia and chorioamnionitis.
steroids, maintaining normal oxygenation, volume ventila- Three common forms of PVL are described:
tion to avoid hypocapnia, in-­utero transfer to tertiary centre • cystic PVL—most severe type due to focal cystic necrosis
and indomethacin prophylaxis. •  microcystic PVL—cystic lesions are only detected on
The neonate with a GM-­IVH may be asymptomatic and high resolution MRI
the bleed is then detected incidentally on cranial ultrasound • diffuse WMI—the most frequent type of injury
examination (Figure 2.6a and 2.6b). Most, however, will be

Table 2.4 Grading systems for germinal matrix and

intraventricular haemorrhage

Severity
grade 1 germinal matrix haemorrhage with or
without IVH (less than 10% of ventricle
filled with blood) (Figure 2.5).
grade 2 IVH (10%–50% of ventricle filled with blood),
typically without ventricular dilation.
grade 3 IVH (greater than 50% of ventricle filled with
blood) typically with ventricular dilation
(Figure 2.6a and 2.6b).
grade 4 periventricular haemorrhagic infarction. Fig. 2.5 Para-­sagittal view of a small GMH-­IVH (grade 1 IVH)
(Copyright – Dr Mithilesh Lal -­ used with permission)
13
 Chapter |2| Neonatology

A B
Fig 2.6a and Fig. 2.6b. Coronal and parasagittal view of grade 3 IVH (Copyright – Dr Mithilesh Lal -­ used with permission)

The condition is initially asymptomatic and only iden- severe ROP, and 1% will develop blindness. Classification
tified on routine cranial USS but can later lead to abnor- of ROP is based on anterior-­posterior location (zone),
malities of tone, poor feeding and seizures. severity (stage), extent (number of clock hours of ROP
along the circumference of retina), dilation and tortuosity
of posterior pole vessels.
Treatment and management Signs indicative of ROP activity including increased
Strategies to prevent PVL include antenatal steroids and venous dilation and arteriolar tortuosity of the posterior
antenatal magnesium sulphate given to the mother when retinal vessels. These features may later increase in severity
preterm delivery is imminent. Once born, management to include iris vascular engorgement, poor pupillary dila-
will include delayed clamping of cord, optimum resusci- tion (rigid pupil) and vitreous haze.
tation and stabilisation and gentle ventilation to avoid Screening criteria in UK advise that all babies less than
extremes in CO2 and O2 levels. Adequate management 32 weeks gestational age (up to 31 weeks and 6 days) or
to maintain normal levels of blood pressure, temperature less than 1501 g birth weight should be screened for ROP.
and blood glucose are important steps in early manage-
ment that help prevent neurological damage.
Treatment and management
Laser ablation is the current treatment of choice as this
Neurodevelopmental outcome prevents progression of the lesions to retinal detachment
Cystic PVL often leads to spastic diplegia. It is easier to and blindness. Intraocular injections of anti-­VEGF agents
predict neuromotor outcomes than other domains of such as bevacizumab are considered as first-­line treatment
neurodevelopmental problems, including cognitive and for localised central ROP as laser ablation is ineffective in
behavioural issues relating to more common and wide- the prevention of retinal detachment.
spread diffuse WMI.

Retinopathy of prematurity (ROP)

Medical conditions in the term
Retinopathy of prematurity is a disease of immature and
developing retinal vasculature and was associated with
neonate
increasing use of supplemental oxygen and improved sur-
vival in the preterm infant. Restrictive use of supplemental
Meconium aspiration syndrome
oxygen led to reduction in ROP but an increase in deaths
and disability due to cerebral palsy. Up to 60% to 70% of This condition is a relatively common cause of respira-
VLBW babies can develop ROP, 5% require treatment for tory distress in term or near-­term infants born through

14
 Another Random Document on
Scribd Without Any Related Topics
back passage, kicked Snap; who forthwith flew at the
gardener as he was bringing in the horse-radish for the
beef; who stepping backwards trode upon the cat; who spat
and swore, and went up the pump with her tail as big as a
fox’s brush.

To avoid this domestic scene, the hot-tempered gentleman

withdrew to the breakfast-room and took up a newspaper.
By-and-by, Harry and Polly came in, and they were soon
snapping comfortably over their own affairs in a corner.

The hot-tempered gentleman’s umber eyes had been looking

over the top of his newspaper at them for some time, before
he called, “Harry, my boy!”

And Harry came up to him.

“Shew me your tongue, Harry,” said he.

“What for?” said Harry; “you’re not a doctor.”

“Do as I tell you,” said the hot-tempered gentleman; and as

Harry saw his hand moving, he put his tongue out with all
possible haste. The hot-tempered gentleman sighed. “Ah!”
he said in depressed tones; “I thought so!—Polly, come and
let me look at yours.”

Polly, who had crept up during this process, now put out
hers. But the hot-tempered gentleman looked gloomier still,
and shook his head.
“What is it?” cried both the children. “What do you mean?”
And they seized the tips of their tongues in their fingers, to
feel for themselves.

But the hot-tempered gentleman went slowly out of the

room without answering; passing his hands through his hair,
and saying, “Ah! Hum!” and nodding with an air of grave
foreboding.

Just as he crossed the threshold, he turned back, and put

his head into the room. “Have you ever noticed that your
tongues are growing pointed?” he asked.

“No!” cried the children with alarm. “Are they?”

“If ever you find them becoming forked,” said the gentleman
in solemn tones, “let me know.”

With which he departed, gravely shaking his head.

In the afternoon the children attacked him again. “Do tell us

what’s the matter with our tongues.”

“You were snapping and squabbling just as usual this

morning,” said the hot-tempered gentleman.

“Well, we forgot,” said Polly. “We don’t mean anything, you

know. But never mind that now, please. Tell us about our
tongues. What is going to happen to them?”

“I’m very much afraid,” said the hot-tempered gentleman, in

solemn measured tones, “that you are both of you—fast—
going—to—the—”

“Dogs?” suggested Harry, who was learned in cant

expressions.

“Dogs!” said the hot-tempered gentleman, driving his hands

through his hair. “Bless your life, no! Nothing half so
pleasant! (That is, unless all dogs were like Snap, which
mercifully they are not.) No, my sad fear is, that you are
both of you—rapidly—going—to the Snap-Dragons!”
And not another word would the hot-tempered gentleman
say on the subject.

Christmas Eve.

In the course of a few hours Mr and Mrs Skratdj recovered

their equanimity. The punch was brewed in a jug, and tasted
quite as good as usual. The evening was very lively. There
were a Christmas-tree, Yule cakes, log, and candles,
furmety, and snap-dragon after supper. When the company
was tired of the tree, and had gained an appetite by the
hard exercise of stretching to high branches, blowing out
“dangerous” tapers, and cutting ribbon and pack-thread in
all directions, supper came, with its welcome cakes and
furmety and punch. And when furmety somewhat palled
upon the taste (and it must be admitted to boast more
sentiment than flavour as a Christmas dish), the Yule
candles were blown out and both the spirits and the palates
of the party were stimulated by the mysterious and pungent
pleasures of snap-dragon.

Then, as the hot-tempered gentleman warmed his coat-tails

at the Yule-log, a grim smile stole over his features as he
listened to the sounds in the room. In the darkness the blue
flames leaped and danced, the raisins were snapped and
snatched from hand to hand, scattering fragments of flame
hither and thither. The children shouted as the fiery
sweetmeats burnt away the mawkish taste of the furmety.
Mr Skratdj cried that they were spoiling the carpet; Mrs
Skratdj complained that he had spilled some brandy on her
dress. Mr Skratdj retorted that she should not wear dresses
so susceptible of damage in the family circle. Mrs Skratdj
recalled an old speech of Mr Skratdj on the subject of
wearing one’s nice things for the benefit of one’s family, and
not reserving them for visitors. Mr Skratdj remembered that
Mrs Skratdj’s excuse for buying that particular dress when
she did not need it, was her intention of keeping it for the
next year. The children disputed as to the credit for courage
and the amount of raisins due to each. Snap barked
furiously at the flames; and the maids hustled each other for
good places in the doorway, and would not have allowed the
man-servant to see at all, but he looked over their heads.

“St! St! At it! At it!” chuckled the hot-tempered gentleman in

undertones. And when he said this, it seemed as if the
voices of Mr and Mrs Skratdj rose higher in matrimonial
repartee, and the children’s squabbles became louder, and
the dog yelped as if he were mad, and the maids’ contest
was sharper; whilst the snap-dragon flames leaped up and
up, and blue fire flew about the room like foam.

At last the raisins were finished, the flames were all but out,
and the company withdrew to the drawing-room. Only Harry
lingered.
“Come along, Harry,” said the hot-tempered gentleman.

“Wait a minute,” said Harry.

“You had better come,” said the gentleman.

“Why?” said Harry.

“There’s nothing to stop for. The raisins are eaten, the

brandy is burnt out—”

“No, it’s not,” said Harry.

“Well, almost. It would be better if it were quite out. Now

come. It’s dangerous for a boy like you to be alone with the
Snap-Dragons to-night.”

“Fiddle-sticks!” said Harry.

“Go your own way, then!” said the hot-tempered gentleman;

and he bounced out of the room, and Harry was left alone.
He crept up to the table, where one little pale blue flame
flickered in the snap-dragon dish.

“What a pity it should go out!” said Harry. At this moment

the brandy bottle on the side-board caught his eye.
“Just a little more,” murmured Harry to himself; and he
uncorked the bottle, and poured a little brandy on to the
flame.

Now of course, as soon as the brandy touched the fire, all

the brandy in the bottle blazed up at once, and the bottle
split to pieces; and it was very fortunate for Harry that he
did not get seriously hurt. A little of the hot brandy did get
into his eyes, and made them smart, so that he had to shut
them for a few seconds.

But when he opened them again, what a sight he saw! All

over the room the blue flames leaped and danced as they
had leaped and danced in the soup-plate with the raisins.
And Harry saw that each successive flame was the fold in
the long body of a bright blue Dragon, which moved like the
body of a snake. And the room was full of these Dragons. In
the face they were like the dragons one sees made of very
old blue and white china; and they had forked tongues, like
the tongues of serpents. They were most beautiful in colour,
being sky-blue. Lobsters who have just changed their coats
are very handsome, but the violet and indigo of a lobster’s
coat is nothing to the brilliant sky-blue of a Snap-Dragon.

How they leaped about! They were for ever leaping over
each other, like seals at play. But if it was “play” at all with
them, it was of a very rough kind; for as they jumped, they
snapped and barked at each other, and their barking was like
that of the barking Gnu in the Zoological Gardens; and from
time to time they tore the hair out of each other’s heads
with their claws, and scattered it about the floor. And as it
dropped it was like the flecks of flame people shake from
their fingers when they are eating snap-dragon raisins.

Harry stood aghast.

“What fun!” said a voice close by him; and he saw that one
of the Dragons was lying near, and not joining in the game.
He had lost one of the forks of his tongue by accident, and
could not bark for awhile.

“I’m glad you think it funny,” said Harry, “I don’t.”

“That’s right. Snap away!” sneered the Dragon. “You’re a

perfect treasure. They’ll take you in with them the third
round.”

“Not those creatures?” cried Harry.

“Yes, those creatures. And if I hadn’t lost my bark, I’d be the
first to lead you off,” said the Dragon. “Oh, the game will
exactly suit you.”

“What is it, please?” Harry asked.

“You’d better not say ‘please’ to the others,” said the
Dragon, “if you don’t want to have all your hair pulled out.
The game is this. You have always to be jumping over
somebody else, and you must either talk or bark. If anybody
speaks to you, you must snap in return. I need not explain
what snapping is. You know. If anyone by accident gives a
civil answer, a claw-full of hair is torn out of his head to
stimulate his brain. Nothing can be funnier.”

“I dare say it suits you capitally,” said Harry; “but I’m sure
we shouldn’t like it. I mean men and women and children. It
wouldn’t do for us at all.”

“Wouldn’t it?” said the Dragon. “You don’t know how many
human beings dance with dragons on Christmas Eve. If we
are kept going in a house till after midnight, we can pull
people out of their beds, and take them to dance in
Vesuvius.”

“Vesuvius!” cried Harry.

“Yes, Vesuvius. We come from Italy originally, you know. Our

skins are the colour of the Bay of Naples. We live on dried
grapes and ardent spirits. We have glorious fun in the
mountain sometimes. Oh! what snapping, and scratching,
and tearing! Delicious! There are times when the squabbling
becomes too great, and Mother Mountain won’t stand it, and
spits us all out, and throws cinders after us. But this is only
at times. We had a charming meeting last year. So many
human beings, and how they can snap! It was a choice
party. So very select. We always have plenty of saucy
children, and servants. Husbands and wives too, and quite
as many of the former as the latter, if not more. But besides
these, we had two vestry-men, a country postmaster, who
devoted his talents to insulting the public instead of to
learning the postal regulations, three cabmen and two
‘fares,’ two young shop-girls from a Berlin wool shop in a
town where there was no competition, four commercial
travellers, six landladies, six Old Bailey lawyers, several
widows from almshouses, seven single gentlemen and nine
cats, who swore at everything; a dozen sulphur-coloured
screaming cockatoos; a lot of street children from a town; a
pack of mongrel curs from the colonies, who snapped at the
human beings’ heels, and five elderly ladies in their Sunday
bonnets with Prayer-books, who had been fighting for good
seats in church.”

“Dear me!” said Harry.

“If you can find nothing sharper to say than ‘Dear me,’” said
the Dragon, “you will fare badly, I can tell you. Why, I
thought you’d a sharp tongue, but it’s not forked yet, I see.
Here they are, however. Off with you! And if you value your
curls—Snap!”

And before Harry could reply, the Snap-Dragons came on on

their third round, and as they passed they swept Harry with
them.

He shuddered as he looked at his companions. They were as

transparent as shrimps, but of this lovely cerulaean blue.
And as they leaped they barked—“Howf! Howf?”—like
barking Gnus; and when they leaped Harry had to leap with
them. Besides barking, they snapped and wrangled with
each other; and in this Harry must join also.

“Pleasant, isn’t it?” said one of the blue Dragons.

“Not at all,” snapped Harry.

“That’s your bad taste,” snapped the blue Dragon.

“No, it’s not!” snapped Harry.

“Then it’s pride and perverseness. You want your hair
combing.”

“Oh, please don’t!” shrieked Harry, forgetting himself. On

which the Dragon clawed a handful of hair out of his head,
and Harry screamed, and the blue Dragons barked and
danced.

“That made your hair curl, didn’t it?” asked another Dragon,
leaping over Harry.

“That’s no business of yours,” Harry snapped, as well as he

could for crying.

“It’s more my pleasure than business,” retorted the Dragon.

“Keep it to yourself, then,” snapped Harry.

“I mean to share it with you, when I get hold of your hair,”

snapped the Dragon.

“Wait till you get the chance,” Harry snapped, with desperate
presence of mind.

“Do you know whom you’re talking to?” roared the Dragon;
and he opened his mouth from ear to ear, and shot out his
forked tongue in Harry’s face; and the boy was so frightened
that he forgot to snap, and cried piteously,—

“Oh, I beg your pardon, please don’t!”

On which the blue Dragon clawed another handful of hair out

of his head, and all the Dragons barked as before.

How long the dreadful game went on Harry never exactly

knew. Well practised as he was in snapping in the nursery,
he often failed to think of a retort, and paid for his
unreadiness by the loss of his hair. Oh, how foolish and
wearisome all this rudeness and snapping now seemed to
him! But on he had to go, wondering all the time how near it
was to twelve o’clock, and whether the Snap-Dragons would
stay till midnight and take him with them to Vesuvius.

At last, to his joy, it became evident that the brandy was

coming to an end. The Dragons moved slower, they could
not leap so high, and at last one after another they began to
go out.

“Oh, if they only all of them get away before twelve!”

thought poor Harry.

At last there was only one. He and Harry jumped about and
snapped and barked, and Harry was thinking with joy that
he was the last, when the clock in the hall gave that whirring
sound which some clocks do before they strike, as if it were
clearing its throat.

“Oh, please go!” screamed Harry in despair.

The blue Dragon leaped up, and took such a claw-full of hair
out of the boy’s head, that it seemed as if part of the skin
went too. But that leap was his last. He went out at once,
vanishing before the first stroke of twelve. And Harry was
left on his face on the floor in the darkness.
 Conclusion.

When his friends found him there was blood on his forehead.
Harry thought it was where the Dragon had clawed him, but
they said it was a cut from a fragment of the broken brandy
bottle. The Dragons had disappeared as completely as the
brandy.

Harry was cured of snapping. He had had quite enough of it

for a lifetime, and the catch-contradictions of the household
now made him shudder. Polly had not had the benefit of his
experiences, and yet she improved also.

In the first place, snapping, like other kinds of quarrelling,

requires two parties to it, and Harry would never be a party
to snapping any more. And when he gave civil and kind
answers to Polly’s smart speeches, she felt ashamed of
herself, and did not repeat them.

In the second place, she heard about the Snap-Dragons.

Harry told all about it to her and to the hot-tempered
gentleman.
“Now do you think it’s true?” Polly asked the hot-tempered
gentleman.

“Hum! Ha!” said he, driving his hands through his hair. “You
know I warned you, you were going to the Snap-Dragons.”

Harry and Polly snubbed “the little ones” when they

snapped, and utterly discountenanced snapping in the
nursery. The example and admonitions of elder children are
a powerful instrument of nursery discipline, and before long
there was not a “sharp tongue” amongst all the little
Skratdjs.

But I doubt if the parents ever were cured. I don’t know if

they heard the story. Besides, bad habits are not easily
cured when one is old.

I fear Mr and Mrs Skratdj have yet got to dance with the
Dragons.
 Chapter Two.
Old Father Christmas.

“Can you fancy, young people,” said Godfather Garbel,

winking with his prominent eyes, and moving his feet
backwards and forwards in his square shoes, so that you
could hear the squeak-leather half a room off—“can you
fancy my having been a very little boy, and having a
godmother? But I had, and she sent me presents on my
birthdays too. And young people did not get presents when I
was a child as they get them now. Grumph! We had not half
so many toys as you have, but we kept them twice as long. I
think we were fonder of them too, though they were neither
so handsome, nor so expensive as these new-fangled affairs
you are always breaking about the house. Grumph!

“You see, middle-class folk were more saving then. My

mother turned and dyed her dresses, and when she had
done with them, the servant was very glad to have them;
but, bless me! your mother’s maids dress so much finer than
their mistress, I do not think they would say ‘thank you’ for
her best Sunday silk. The bustle’s the wrong shape.
Grumph!
“What’s that you are laughing at, little miss? It’s pannier, is
it? Well, well, bustle or pannier, call it what you like; but only
donkeys wore panniers in my young days, and many’s the
ride I’ve had in them.

“Now as I say, my relations and friends thought twice before

they pulled out five shillings in a toy-shop, but they didn’t
forget me, all the same.

“On my eighth birthday my mother gave me a bright blue

comforter of her own knitting.

“My little sister gave me a ball. My mother had cut out the
divisions from various bits in the rag bag, and my sister had
done some of the seaming. It was stuffed with bran, and had
a cork inside which had broken from old age, and could no
longer fit the pickle jar it belonged to. This made the ball
bound when we played ‘prisoner’s base.’

“My father gave me the broken driving-whip that had lost

the lash, and an old pair of his gloves, to play coachman
with; these I had long wished for, since next to sailing in a
ship, in my ideas, came the honour and glory of driving a
coach.

“My whole soul, I must tell you, was set upon being a sailor.
In those days I had rather put to sea once on Farmer
Fodder’s duck-pond than ride twice atop of his hay-waggon;
and between the smell of hay and the softness of it, and the
height you are up above other folk, and the danger of
tumbling off if you don’t look out—for hay is elastic as well
as soft—you don’t easily beat a ride on a hay-waggon for
pleasure. But as I say, I’d rather put to sea on the duck-
pond, though the best craft I could borrow was the pigsty-
door, and a pole to punt with, and the village boys jeering
when I got aground, which was most of the time—besides
the duck-pond never having a wave on it worth the name,
punt as you would, and so shallow you could not have got
drowned in it to save your life.

“You’re laughing now, little master, are you? But let me tell
you that drowning’s the death for a sailor, whatever you may
think. So I’ve always maintained, and have given every
navigable sea in the known world a chance, though here I
am after all, laid up in arm-chairs and feather-beds, to wait
for bronchitis or some other slow poison. Grumph!

“Well, we must all go as we’re called, sailors or landsmen,

and as I was saying if I was never to sail a ship, I would
have liked to drive a coach. A mail coach, serving His
Majesty. (Her Majesty now God bless her!) carrying the
Royal Arms, and bound to go, rough weather and fair.
Many’s the time I’ve done it (in play you understand) with
that whip and those gloves. Dear! dear! The pains I took to
teach my sister Patty to be a highwayman, and jump out on
me from the drying ground hedge in the dusk with a ‘Stand
and deliver!’ which she couldn’t get out of her throat for
fright, and wouldn’t jump hard enough for fear of hurting
me.

“The whip and the gloves gave me joy, I can tell you; but
there was more to come.

“Kitty the servant gave me a shell that she had had by her
for years. How I had coveted that shell! It had this
remarkable property: when you put it to your ear you could
hear the roaring of the sea. I had never seen the sea, but
Kitty was born in a fisherman’s cottage, and many an hour
have I sat by the kitchen fire whilst she told me strange
stories of the mighty ocean, and ever and anon she would
snatch the shell from the mantelpiece and clap it to my ear,
crying, ‘There child, you couldn’t hear it plainer than that.
It’s the very moral!’

“When Kitty gave me that shell for my very own I felt that
life had little more to offer. I held it to every ear in the
house, including the cat’s; and, seeing Dick the sexton’s son
go by with an armful of straw to stuff Guy Fawkes, I ran out,
and in my anxiety to make him share the treat, and learn
what the sea is like, I clapped the shell to his ear so smartly
and unexpectedly, that he, thinking me to have struck him,
knocked me down then and there with his bundle of straw.
When he understood the rights of the case, he begged my
pardon handsomely, and gave me two whole treacle sticks
and part of a third out of his breeches’ pocket, in return for
which I forgave him freely, and promised to let him hear the
sea roar on every Saturday half-holiday till farther notice.

“And, speaking of Dick and the straw reminds me that my

birthday falls on the fifth of November. From this it came
about that I always had to bear a good many jokes about
being burnt as a Guy Fawkes; but, on the other hand, I was
allowed to make a small bonfire of my own, and to have
eight potatoes to roast therein, and eight-pennyworth of
crackers to let off in the evening. A potato and a pennyworth
of crackers for every year of my life.

“On this eighth birthday, having got all the above named
gifts, I cried, in the fulness of my heart, ‘There never was
such a day!’ And yet there was more to come, for the
evening coach brought me a parcel, and the parcel was my
godmother’s picture-book.

“My godmother was a gentlewoman of small means; but she

was accomplished. She could make very spirited sketches,
and knew how to colour them after they were outlined and
shaded in Indian ink. She had a pleasant talent for
versifying. She was very industrious. I have it from her own
lips that she copied the figures in my picture-book from
prints in several different houses at which she visited. They
were fancy portraits of characters, most of which were
familiar to my mind. There were Guy Fawkes, Punch, his
then Majesty the King, Bogy, the Man in the Moon, the Clerk
of the Weather Office, a Dunce, and Old Father Christmas.
Beneath each sketch was a stanza of my godmother’s own
composing.
“My godmother was very ingenious. She had been mainly
guided in her choice of these characters by the prints she
happened to meet with, as she did not trust herself to
design a figure. But if she could not get exactly what she
wanted, she had a clever knack of tracing an outline of the
attitude from some engraving, and altering the figure to suit
her purpose in the finished sketch. She was the soul of
truthfulness, and the notes she added to the index of
contents in my picture-book spoke at once for her honesty in
avowing obligations, and her ingenuity in availing herself of
opportunities.

“They ran thus:—

“Number 1.—Guy Fawkes. Outlined from a figure of a

warehouseman rolling a sherry cask into Mr Rudd’s wine
vaults. I added the hat, cloak, and boots in the finished
drawing.

“Number 2.—Punch. I sketched him from the life.

“Number 3.—His Most Gracious Majesty the King. On a quart

jug bought in Cheapside.

“Number 4.—Bogy, with bad boys in the bag on his back.

Outlined from Christian bending under his burden, in my
mother’s old copy of the ‘Pilgrim’s Progress.’ The face from
Giant Despair.

“Number 5 and Number 6.—The Man in the Moon, and The

Clerk of the Weather Office. From a book of caricatures
belonging to Dr James.

“Number 7.—A Dunce. From a steel engraving framed in

rosewood that hangs in my Uncle Wilkinson’s parlour.
“Number 8.—Old Father Christmas. From a German book at
Lady Littleham’s.”
 Chapter Three.

“My sister Patty was six years old. We loved each other
dearly. The picture-book was almost as much hers as mine.
We sat so long together on one big footstool by the fire, with
our arms round each other, and the book resting on our
knees, that Kitty called down blessings on my godmother’s
head for having sent a volume that kept us both so long out
of mischief.

”‘If books was allus as useful as that, they’d do for me,’ said
she; and though this speech did not mean much, it was a
great deal for Kitty to say; since, not being herself an
educated person, she naturally thought that ‘little enough
good comes of larning.’

“Patty and I had our favourites amongst the pictures. Bogy,

now, was a character one did not care to think about too
near bed-time. I was tired of Guy Fawkes, and thought he
looked more natural made of straw, as Dick did him. The
Dunce was a little too personal; but Old Father Christmas
took our hearts by storm; we had never seen anything like
him, though now-a-days you may get a plaster figure of him
in any toy-shop at Christmas-time, with hair and beard like
cotton-wool, and a Christmas-tree in his hand.

“The custom of Christmas-trees came from Germany. I can

remember when they were first introduced into England, and
what wonderful things we thought them. Now, every village
school has its tree, and the scholars openly discuss whether
the presents have been ‘good,’ or ‘mean,’ as compared with
other trees of former years.

“The first one that I ever saw I believed to have come from
good Father Christmas himself; but little boys have grown
too wise now to be taken in for their own amusement. They
are not excited by secret and mysterious preparations in the
back drawing-room; they hardly confess to the thrill—which.
I feel to this day—when the folding-doors are thrown open,
and amid the blaze of tapers, Mamma, like a Fate, advances
with her scissors to give every one what falls to his lot.

“Well, young people, when I was eight years old I had not
seen a Christmas-tree, and the first picture of one I ever
saw was the picture of that held by Old Father Christmas in
my godmother’s picture-book.

”‘What are those things on the tree?’ I asked.

”‘Candles,’ said my father.

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