PasTest Paediatrics

Series

MRCPCH 1:
Essential Questions in Paediatrics

Second Edition
PasTest
Dedicated to your success

Mark Beattie Mike Champion

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978 1905635 535

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Contents

Contributors

Introduction

1.
Cardiology
iv

viii

2. Child Development, Child Psychiatry and Community

Paediatrics

3. Clinical Pharmacology and Toxicology

13

26

4.
Dermatology
37

5. Emergency Medicine
48

6. Endocrinology
59

7. Ethics, Law and Governance

74

8. Gastroenterology and Nutrition

88

9. Genetics
107

10. Haematology and Oncology

117

11. Hepatology
129

12. Immunology
147

13. Infectious Diseases

14. Metabolic Medicine

15. Neonatology

16. Nephrology
 156

169

182

194

17. Neurology
209

18. Ophthalmology
220

19. Paediatric Surgery

228

20. Respiratory Medicine

240

21. Rheumatology
251

22. Statistics

Index
265

279

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Contributors

Jim Baird BM MRCPCH

SPR in Paediatric Endocrinology, Paediatric Medical Unit, Southampton General Hospital,

Southampton (Endocrinology)

R Mark Beattie BSc MBBS FRCPCH MRCP

 Consultant Paediatric Gastroenterologist, Paediatric Medical Unit, Southampton General Hospital,
Southampton (Gastroenterology and Nutrition)

Natalie L E Canham MBChB BA (Hons) MRCP (Paeds)

Consultant in Clinical Genetics, North West Thames Regional Genetics Service, Northwick Park
Hospital, Harrow, Middlesex (Genetics)

Michael L Capra MBBCh DCH Dip Obs MRCP MMedSci (Clinical education)
Consultant Paediatric Oncologist, Our Lady's Children's Hospital, Dublin (Clinical Pharmacology;
Haematology and Oncology)

Mike Champion BSc MBBS MRCP FRCPCH

Consultant in Paediatric Inherited Metabolic Disease, Evelina Children's Hospital, Guy's and St Thomas' NHS
Foundation Trust, London (Metabolic Medicine; Ophthalmology)

Andrew Clark MBBS MRCP MD

Consultant in Paediatric Allergy, Addenbrookes Hospital, Cambridge

University Hospitals NHS Foundation Trust, Cambridge (Respiratory Medicine)

Serena Cottrell MBBS BSc (Hons) MMedSci MRCPI FRCPCH

Consultant Paediatrician and Lead for Resuscitation Service, Department of Paediatrics, Southampton
General Hospital, Southampton (Emergency Medicine)

Justin Davies MD FRCPCH MRCP

Consultant Paediatric Endocrinologist, Paediatric Medical Unit, Southampton General

Hospital, Southampton (Endocrinology)

Jane C Davies MB ChB MRCP MRCPCH MD

Senior Lecturer in Gene Therapy, The Department of Gene Therapy, NHLI, Imperial College, Emmanuel
Kaye Building, London (Respiratory Medicine)

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CONTRIBUTORS
V

Anil Dhawan MBBS MD FRCPCH

Professor of Paediatric Hepatology, Paediatric Liver Centre, King's College Hospital,

Denmark Hill, London (Hepatology)
 Katy Fidler BSc MBBS MRCPCH PhD
Consultant in General Paediatrics and Paediatric Infectious Diseases, Royal Alexandra Children's Hospital,
Brighton (Infectious Diseases)

Grenville F Fox MBChB MRCP FRCPCH

Consultant Neonatologist and Clinical Director, Children's Services and Genetics, Evelina Children's
Hospital Neonatal Unit, Guy's and St Thomas' NHS Foundation Trust, London (Neonatology)

Helen M Goodyear MBChB FRCP FRCPCH MD MMEd

Consultant Paediatrician and Associate Postgraduate Dean, Department of Child Health,
Birmingham Heartlands Hospital, Bordesley Green East, Birmingham (Dermatology)

Juliet C Gray MBBS MA PhD MRCP (Paeds)

Clinical Lecturer in Paediatric Oncology, Southampton General Hospital, Southampton
(Haematology and Oncology)

Steve Halford MBBS MRCP FCEM

Consultant in Emergency Medicine, Southampton General Hospital, Southampton

(Emergency Medicine)

James W Hart MBBS BSc (Hons) MRCP (UK) MRCPCH

SPR, Paediatric Medical Unit, Southampton General Hospital, Southampton (Nephrology)

Nathan Hasson MBChB FRCPCH

Consultant in Paediatric Rheumatology, The Portland Hospital, London (Rheumatology)

Tammy Hedderly MBBS BSc (Hons) MRCPCH

Consultant Paediatric Neurologist, Evelina Children's Hospital and King's College Hospital, London
(Neurology)

Lara Kitteringham MBChB FRCS (Paed)

Consultant Paediatric Surgeon, Paediatric Surgical Unit, Southampton General Hospital,
Southampton (Paediatric Surgery)

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 VI
CONTRIBUTORS

Vic Larcher MB BChir MA FRCPCH FRCP

Consultant in Paediatrics and Clinical Ethics, Great Ormond Street Hospital, London (Ethics, Law and
Governance)

Emma J Lim BMBS MSc MRCP

SPR in Paediatric Infectious Diseases, Department of Paediatric Infectious Disease, St Mary's

Hospital, London (Infectious Diseases)

Anne-Marie McMahon BSc MBBS MRCPCH MSc

Consultant in Paediatric Rheumatology, The Leeds Teaching Hospitals NHS Trust and Sheffield Children's
NHS Foundation Trust (Rheumatology)

Heather Mitchell BM BCh MA MRCP MRCPCH MRCGP DCH DRCOG SPR in Paediatric Endocrinology,
Department of Paediatric Endocrinology, University College London Hospitals, London
(Endocrinology)

Reynella Anne Morenas MBBS BSc MRCPCH

SPR in Paediatric Gastroenterology, Paediatric Medical Unit, Southampton General Hospital,
Southampton (Gastroenterology and Nutrition)

Karyn Moshal MBChB MRCP MRCPCH DTMH

Consultant and Lead Clinician in Infectious Diseases, Department of Clinical Infectious Diseases, Great
Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London (Infectious
Diseases)

Joanne Philpot BA MBBS MD MRCPCH

Consultant Paediatrician, Paediatric Department, Wexham Park Hospital, Slough (Child
Development)

Stephen Playfor DM
Consultant Paediatric Intensivist, Paediatric Intensive Care Unit, Royal Manchester Children's Hospital,
Manchester (Emergency Medicine)

Waseem Qasim BMedSci MRCP MRCPCH PhD

Molecular Immunology, Institute of Child Health, London (Immunology)

Nicolas Regamey MD
Assistant Professor, Division of Paediatric Respiratory Medicine, University Children's Hospital,
Bern, Switzerland (Respiratory Medicine)
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Fiona M Regan MB ChB MRCP

CONTRIBUTORS

Consultant Paediatrician with a special interest in endocrinology and diabetes, Hillingdon Hospital,
Uxbridge, London Endocrinology

Christopher Reid MRCP (UK) FRCPCH

Consultant Paediatric Nephrologist, Evelina Children's Hospital, Guy's and St Thomas' NHS
Foundation Trust, London (Nephrology)
VII

Nancy Tan MBBS MMed (Paeds) MRCPCH (Edin) Dip (FP) Derm (S'pore) Consultant,
Department of Paediatric Medicine, KK Women's and Children's Hospital, Singapore
(Hepatology)

Stephen Tomlin BPharm MRPharmS ACPP

Consultant Pharmacist, Children's Services, Evelina Children's Hospital, Guy's and St Thomas' NHS
Foundation Trust, London (Clinical Pharmacology)

Robert M R Tulloh BM BCh MA DM FRCP FRCPCH

Consultant Paediatric Cardiologist, Department of Congenital Heart Disease, Bristol Royal

Hospital for Children, Bristol (Cardiology)
Angie Wade BSc MSc PhD CStat ILTM
Senior Lecturer in Biostatistics, Centre for Paediatric Epidemiology and Statistics, Institute of Child
Health, London (Statistics)

Robert A Wheeler MS FRCS LLB (Hons) FRCPCH

Consultant Paediatric and Neonatal Surgeon, Hon. Senior Lecturer in Medical Law,
Southampton University Hospitals Trust, Southampton (Ethics, Law and Governance;
Paediatric Surgery)

Amy Whiting BSc (Hons) MB BS MRCPCH

SPR in Paediatrics, Children's Hospital Oxford, John Radcliffe Hospital, Oxford (Child
Development)

Louise C Wilson BSc MBChB FRCP

Consultant in Clinical Genetics, Department of Genetics, Institute of Child Health, London (Genetics)

Every effort has been made to acknowledge the contributors for this book. However, if there are any
omissions this will be rectified at reprint.

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Introduction

The new edition of this revision text has been written to accompany Essential Revision Notes in
Paediatrics for MRCPCH (2nd edition). The questions, in the new examination format, are designed to
help facilitate revision for the MRCPCH Part One examination and are also relevant for part one of the DCH
examination. The book is split by subject to aid revision planning. Each question has a detailed
explanation and so the text can be used as a stand-alone revision aid.

The candidate is advised to consult the RCPCH website for up-to-date information regarding the exam
(www.rcpch.ac.uk). The information below is taken from the website and is correct at the time of going to
press.

The MRCPCH Part One examination consists of two papers:

 Paper One A (Basic Child Health), which focuses on the areas of child health that are relevant to those
who will be working with children in their medical careers, not just those entering mainstream
hospital-based paediatrics. The areas tested are those conditions likely to be seen in 6 to 12 months of
hospital, community or primary care practice.

Paper One B (Extended Paediatrics) focuses on the more complex paediatric problem-solving skills
not tested in Paper One A, and on the scientific knowledge underpinning paediatrics.

Candidates for MRCPCH must successfully complete both Paper One A and One B before being allowed to
enter MRCPCH Part Two.

Paper One A (Basic Child Health) replaces the previous Diploma in Child Health (DCH)
written paper.

The papers consist of:

Multiple true-false questions used to test knowledge when there is an absolute Yes/No answer.

Best of Five questions used to test judgement and experience. A simple statement or short clinical
scenario leads into five options. All could be possible but only one is completely or the most correct. The
candidate has to choose the best option.

Extended matching questions (EMQs) are used in the same way as Best of Five questions. In this case a list of
6-12 possible answers is offered with two or three statements or clinical scenarios. The candidate chooses the

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INTRODUCTION

best option from the introductory list. Again, all could be possible but only one is completely or the most
correct.

Further details on the make-up of the three types of questions and on the proportion of different
types are available on the college website along with downloadable sample papers.
IX

We would like to acknowledge the many authors who have enthusiastically contributed chapters
to this book and Kirsten Baxter and Lily Martin at PasTest for their enthusiasm and expertise in
helping pull this new edition together.

Finally, we hope the book helps candidates to get through the exam!

Mark Beattie Mike Champion August 2009

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 1. Cardiology

Multiple True-False Questions

1.1
Muscular ventricular septal defects (VSDs)

A do not require SBE prophylaxis

B usually cause a heart murmur in the first day of life
C if large, are closed by catheter procedures in 10% of cases
D do not have conduction tissue running on their inferior margin Eif large, usually cause
heart failure before the child is 4 days old

1.2 Scimitar syndrome

A is usually associated with a hypoplastic left lung
B
can be palliated by coil occlusion in the cardiac catheter laboratory C will show dextrocardia on the X-ray
as a result of situs inversus D is associated with abnormal pulmonary arterial supply
E is usually associated with abnormal radii

1.3 On the first day of life, the following may be found in neonates
with congenital heart disease:

A a harsh pansystolic murmur with the diagnosis of ventricular septal

defect

B severe cyanosis in unobstructed total anomalous pulmonary venous

connection

Ca harsh systolic murmur in transposition of the great arteries

without associated defect

D severe acidosis and poor pulses with hypoplastic left heart syndrome E severe cyanosis
and acidosis in a baby with Down syndrome and
atrioventricular septal defect

1.4 The following is true of persistent ductus arteriosus:

A it is defined as persistence of ductal patency beyond 1 week after

the date the baby should have been born
B on auscultation, a continuous murmur in the right infraclavicular
area is heard

C it may present as heart failure with poor peripheral pulses

D closure is usually undertaken in the catheter laboratory with coil or
device at 1 year
E if it is large, surgical ligation is recommended at 1-3 months
1
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CARDIOLOGY - QUESTIONS

1.5 The following statements about transposition of the great

1.6
arteries are true:

A there is an association with coarctation of the aorta

B arterial switch is the operation of choice, undertaken before

2 weeks

C the condition is detected antenatally in 50% of cases

D presentation can occur upon closure of the ductus arteriosus
E the arrangement of the coronary arteries is a major factor in deter-
mining the success of the surgical repair

The following is true of Eisenmenger syndrome:

A affected children are typically teenagers
B it can be seen in children with Down syndrome
Cit is usually secondary to an untreated ventricular septal defect or
atrioventricular septal defect
D the pulmonary component of the second heart sound is quiet on
auscultation

E the ECG shows left ventricular hypertrophy

Best of Five Questions

1.7
1.8
You are asked to review an ECG of a baby on the intensive care unit. The baby was well at birth,
but soon became unwell and cyanosed. There was no heart murmur. ECG findings reveal
a superior axis, absent right ventricular voltages, and a large P wave. What is the MOST likely
diagnosis?

A Complete atrioventricular septal defect

B Tricuspid atresia
C Critical pulmonary stenosis
D Transposition of the great arteries
E Total anomalous pulmonary venous connection (TAPVC)

You are asked to see in clinic a 6-year-old girl with a diagnosis of right atrial isomerism. Which
one of the following features would you expect her to have?
A Asplenia and a midline liver
B Polysplenia
C Two functional left lungs
D T-cell deficiency
E Trisomy 21

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1.9
CARDIOLOGY
QUESTIONS

You are asked to review a child on the ward who is known to have short stature and renal abnormalities. On
left sternal edge.
examination, she has micrognathia and an ejection systolic murmur at the upper
Her notes show that she has recently seen an ophthalmologist. What is the MOST likely
 underlying diagnosis? A Williams syndrome B DiGeorge syndrome C Alagille syndrome
D Noonan syndrome
E Left atrial isomerism
3

1.10 A 1-day-old baby who is otherwise asymptomatic presents with a

loud harsh heart murmur at the left sternal edge. There are no features of heart failure present, the
oxygen saturations are normal, and the ECG performed by the resident speciality
registrar is reported to be normal. What is the MOST likely diagnosis in this case?

A Atrial septal defect

B Small muscular ventricular septal defect C Large muscular ventricular
septal defect D Pulmonary stenosis
E Persistent ductus arteriosus

1.11 A newborn baby presents cyanosed and unwell with a heart

murmur at the left sternal edge. The chest X-ray shows massive cardiomegaly with a dilated right atrium and
reduced pulmonary vascular markings. You are informed that the baby's mother has a history of
bipolar depression and that she had been taking lithium during pregnancy. What is the
MOST likely diagnosis? A Transposition of the great arteries
B Tetralogy of Fallot
C Tricuspid atresia
D Ebstein anomaly
E Pulmonary atresia, ventricular septal defect and collaterals
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4
CARDIOLOGY - QUESTIONS

1.12 A 2-year-old boy presents with a murmur, heard in both systole

and diastole at the upper sternal edge, which disappears on lying down. Physical examination is
otherwise normal. He is a well, asymptomatic child and there are no signs of cardiac failure.
You are told that his second cousin had a small ventricular septal defect, which closed
spontaneously, and that his uncle had a heart attack aged 45. What do you consider to be the
BEST management plan?

A Refer for echocardiography and specialist opinion from a consultant

paediatric cardiologist
B Perform an ECG, chest X-ray and oxygen saturations, and then refer
for echocardiography
C Refer for genetic counselling and, possibly, gene-mapping studies D Reassure them that
the murmur is innocent

E Say that you suspect the murmur is caused by a persistent arterial duct, which should be
coil-occluded to avoid the development of heart failure in the future

1.13 You are asked to review a 4-month-old girl in clinic. Her ECG
shows a short P-R interval and giant QRS complexes. Echocardiography
reveals evidence of hypertrophic cardiomyopathy. What is the MOST likely
diagnosis? A Pompe disease
B Lown-Ganong-Levine syndrome
C Hurler syndrome
D Noonan syndrome
E Wolff-Parkinson-White syndrome
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CARDIOLOGY QUESTIONS

Extended Matching Questions

1.14 Theme: Surgical procedures in paediatric cardiology

A Arterial switch procedure
B Hemi-Fontan
C Fontan
D Norwood
E Rastelli

F Blalock-Taussig shunt
G Pulmonary artery (PA) band

I
H Ductus arteriosus ligation
Coarctation of the aorta repair

From the list above, choose the most appropriate procedure for the children in the scenarios below.
Each option may be used once, more than once, or not at all.

1. A 4-day-old baby who presented with absent femoral and brachial pulses, no heart murmur,
and severe acidosis. ECG had revealed absent left ventricular forces.
2. A 3-year-old child with complex cardiac problems, which were not
 suitable for repair, including two separate ventricles. He had under- gone a previous heart
operation at the age of 7 months and had oxygen saturations of 80-85%. His cardiologist felt
that he required a further operation, as there was insufficient blood flow to the lungs, causing
exercise limitation.
3. A severely cyanosed baby with tetralogy of Fallot and a loud heart. murmur at the upper left
sternal edge, and a recent history of severe spells of cyanosis.
5
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6
CARDIOLOGY
QUESTIONS

1.15 Theme: The sick newborn infant

A Pulmonary atresia
B Tetralogy of Fallot
C Coarctation of the aorta

D Hypoplastic left heart syndrome

E Transposition of the great arteries
F Interrupted aortic arch
G Obstructed total anomalous pulmonary venous connection
H Critical aortic stenosis
Ebstein anomaly

Choose the most likely diagnosis from the histories and findings detailed below. Each option may be
used once, more than once, or not at all.
1. A 6-day-old baby presents cyanosed, with a severe metabolic acido-
sis. On examination, there is a large liver but no audible heart murmur. ECG and chest X-ray
were both reported to be normal. 2. A breathless baby with a cleft palate, absent left
brachial and
femoral pulses, and a normal ECG.
3. A very unwell baby, with a loud heart murmur, a superior axis on the
ECG, and reduced pulmonary vascular markings on the chest X-ray.
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CARDIOLOGY ANSWERS
7

MT-F Answers

1.1 Muscular ventricular septal defects (VSDs): D

Ventricular septal defects are the most common form of congenital heart disease, comprising 30% of the
total number of cases. Muscular VSDs occur in the muscular part of the ventricular septum. Subacute
bacterial endocarditis (SBE) prophylaxis is no longer indicated, now only being required in rare and
specific cases. The pulmonary resistance is high at birth, and hence there is little shunt between the two
ventricles and therefore no audible murmur in the first 24 hours. Only 25% of VSDs require cardiac
surgery, and this is usually performed when the child is 3-5 months of age. Very few patients have
interventional catheter closure, usually for smaller defects and at a later age. The conduction tissue
is located inferiorly in a perimembranous septal defect, which means that surgeons need to
avoid that area when suturing a patch in place to close the defect. If the VSD is large, patients
present with symptoms of heart failure after the first week of life and at that age have a right
 ventricular heave, a soft systolic murmur accompanied by an apical mid-diastolic murmur, and a
loud pulmonary second heart sound on examination.

1.2
Scimitar syndrome: B D

Scimitar syndrome is a form of anomalous pulmonary venous drainage in which the veins from the lower right
lung drain into the inferior vena cava. The right lung itself is hypoplastic, and there is an associated
dextrocardia due to the heart moving over to the right side of the chest, but with normal situs. Situs is
the orientation of the organs, situs solitus being normal, and situs inversus being mirror image. The
arterial supply to the lung is from branches of the descending aorta. The right upper lobe
pulmonary vein draining into the inferior vena cava may be seen as a vertical line on a chest X-ray
and is known as the 'scimitar sign'. There may be an atrial septal defect, and children can suffer with
recurrent chest infections, which may require right lower lobectomy.

1.3 Congenital heart disease on the first day of life: D E Babies presenting with left-to-right shunt will
have no murmur or symptoms on the first day of life, because the pulmonary vascular
resistance has yet to fall. Similarly, any common mixing disease, such as atrioventricular septal
defect, can present with severe cyanosis on the first day of life, with high pulmonary vascular
resistance, before breathlessness and heart failure develop at 1 week of age or more. All the
obstructed left heart lesions, such

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00
8
CARDIOLOGY
ANSWERS

as coarctation of the aorta and hypoplastic left heart syndrome, tend to present with acidosis and
weak pulses in the first few days of life.

1.4
Persistent ductus arteriosus: D E

There is abnormal persistence of the ductus arteriosus beyond 1 month after the date the baby should
have been born. Those children affected are usually asymptomatic and rarely develop heart failure.
On auscultation, a continuous 'machinery' or systolic murmur at the left infraclavicular area is heard. The
murmur is initially systolic but, as the pulmonary vascular resistance falls, it becomes continuous in nature
because there is a continual run-off of blood from the aorta to the pulmonary artery (as the pressure in the
aorta is greater than in the pulmonary artery throughout the cardiac cycle). Other clinical features
include bounding pulses and wide pulse pressure. If the duct is large, chest radiography can
demonstrate cardiomegaly and pulmonary plethora. Management is usually by closure in the
cardiac catheter laboratory with a coil or device when the infant is 1 year of age. However, if the duct is
 large, surgical ligation can be undertaken when the infant is aged 1-3 months. The presence of a
ductus arteriosus in a pre-term baby is not congenital heart disease, but these children have a higher
incidence of persistent ductus arteriosus.

1.5 Transposition of the great arteries: A B DE

In this condition, the aorta usually arises anteriorly from the right ventricle and the pulmonary artery
arises posteriorly from the left ventricle. Deoxygenated blood is therefore returned to the body, while
oxygenated blood goes back to the lungs. If these two parallel circuits were completely separate,
the condition would be incompatible with life. These children have high pulmonary blood flow and are
very cyanosed, unless there is an atrial septal defect, a ductus arteriosus, or a ventricular septal
defect, allowing mixing of the two circulations. Babies become cyanosed when the duct closes,
thus reducing the mixing between the systemic and pulmonary circulations, but there is usually
no murmur. Transposition of the great arteries may be associated with ventricular septal defect,
coarctation of the aorta or pulmonary stenosis. Management is to resuscitate the baby, followed by a
balloon atrial septostomy (preferably via the umbilical vein) at a cardiac centre in about 20% of
cases. In the sick, cyanosed newborn baby, a continuous intravenous infusion of prostaglandin E, or
E2 should be commenced to keep the duct open. Definitive repair in the form of the arterial switch
operation will usually be undertaken before the baby is 2 weeks of age.

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CARDIOLOGY ANSWERS

1.6 Eisenmenger syndrome: A B C

Eisenmenger syndrome was first described in 1897, and occurs secondary to a large left-to-right shunt
(usually a ventricular septal defect or atrioventricular septal defect) in which the pulmonary
hypertension leads to pulmonary vascular disease (increased resistance over many years).
Eventually, the flow through the defect is reversed (right-to-left) so the child becomes blue, typically at
10-15 years of age. There is not usually a significant heart murmur. Eventually, they develop right heart
failure. The ECG shows right ventricular hypertrophy and strain pattern, with peaked P waves
indicating right atrial hypertrophy. Management is largely supportive, as surgical closure is not
possible when there is a right-to-left shunt. They may be commenced on sildenafil or an
endothelin-receptor antagonist on the advice of a specialist in pulmonary hypertension.
9
 Best of Five Answers

1.7 B: Tricuspid atresia

Tricuspid atresia is the condition in which there is no tricuspid valve and usually the right ventricle
is very small. There is right-to-left shunt at atrial level, as the blood cannot pass into the right
ventricle. Babies become very cyanosed when the ductus arteriosus closes if they are
duct-dependent, and they usually have no heart murmur. Management options include a
Blalock-Taussig shunt if the child is very blue, a pulmonary artery (PA) band if they are in heart
failure, a hemi-Fontan procedure after they reach 6 months of age, and a Fontan procedure at 3-5
years of age. The ECG shows a superior axis, as the atrioventricular junction is located inferiorly, the P
waves are large as a result of right atrial hypertension, and there is a small right ventricle,
reducing the forces visible on the ECG.

1.8 A: Asplenia and a midline liver

Right atrial isomerism is a multifactorial genetic defect. Right atrial isomerism is associated with asplenia,
small-bowel malrotation, and complex heart disease with abnormalities of connection, in which the
pulmonary veins always connect abnormally because there is no morphological left atrium with which to
connect. In left atrial isomerism, there is polysplenia, small-bowel malrotation (less common
than in right atrial isomerism), two left lungs and complex heart disease.

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 10
CARDIOLOGY ANSWERS

1.9
C: Alagille syndrome
Alagille syndrome is a genetic defect of the JAG-1 gene in 70% of cases. Features include
peripheral pulmonary artery stenosis, a prominent forehead, wide-apart eyes, small chin,
butterfly vertebrae, intrahepatic biliary hypoplasia, embryotoxon (slit lamp for cornea), and renal
and growth abnormalities. Posterior embryotoxon occurs when there is a prominent Schwalbe's line
visible just inside the temporal limbus. It occurs in approximately 15% of normal eyes and is visible
through a clear cornea as a sharply defined, concentric white line or opacity anterior to the
limbus. Williams syndrome is due to a 1.5-Mb deletion on chromosome 7 and leads to typical
facial features, behavioural abnormalities and cardiac features of supravalvar aortic stenosis and branch
pulmonary artery stenosis. DiGeorge syndrome is associated with conotruncal defects (tetralogy of Fallot,
common arterial trunk and interrupted aortic arch), typical facial features, cleft palate, absent
thymus and absent parathyroids. Noonan syndrome is associated with mutations in PTPN11, SOS1,
KRAS or RAF1 genes, with cardiac features of hypertrophic cardiomyopathy, atrial septal defect,
pulmonary stenosis and pulmonary hypertension.

1.10 D: Pulmonary stenosis

Those children with left-to-right shunts have no signs or symptoms on the first day of life.
However, those with outflow obstruction have a murmur from birth. Pulmonary stenosis usually causes no
cyanosis, and all neonates have a dominant right ventricle, thus revealing no evidence of right ventricular
hypertrophy.

1.11 D: Ebstein anomaly

Ebstein anomaly is signified by an abnormal and regurgitant tricuspid valve, which is set further
down into the right ventricle than normal. The affected child will be cyanosed at birth, with a pansystolic
murmur of tricuspid regurgitation at the lower sternal edge. This congenital heart condition has been
associated with maternal ingestion of lithium.

1.12 D: Reassure them that the murmur is innocent

This is typical of a venous hum, an innocent heart murmur. It may be easy to hear the venous blood
flow returning to the heart, especially at the upper sternal edge. This characteristically occurs in both
systole and diastole, and disappears when the child lies flat. Innocent murmurs are the most
common murmurs heard in children, occurring in up to 50% of normal children. They are often
discovered in children with a co-existing infection or with anaemia.
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CARDIOLOGY
ANSWERS
11

Innocent murmurs all relate to a structurally normal heart and it is clearly important to reassure the parents
that their child's heart is normal. Types of innocent murmur include those caused by increased flow
across the branch pulmonary artery, Still's murmur, and venous hums. The murmur should be
soft (no thrill), systolic (diastolic murmurs are not innocent) and short, never pansystolic. The child
is always asymptomatic. The murmur may change with posture, as in venous hums.

1.13 A: Pompe disease

Pompe disease is a glycogen storage disorder that can affect the heart. It results in an autosomal
recessive hypertrophic cardiomyopathy, and is rare. Glycogen accumulates in skeletal muscle, the
tongue and diaphragm, and the liver. The heart enlarges as glycogen is deposited in the ventricular
muscle. It is a progressive disease. The ECG reveals a short P-R interval with giant QRS complexes.
Chest radiography shows an enlarged heart and often congested lung fields. Treatment is
largely supportive.

EMQ Answers

1.14 Surgical procedures in paediatric cardiology

1. D: Norwood procedure
The Norwood procedure is used to palliate hypoplastic left heart syndrome. It is performed
when the infant is aged 3-5 days. The right ventricle is intended to pump blood to the body, so
the pulmonary artery is sewn onto the aorta. The atrial septum is excised so that pulmonary
venous blood can return to the right ventricle and, to ensure adequate pulmonary blood flow, either a
Blalock-Taussig shunt is inserted or a conduit is constructed from the right ventricle to the pulmonary
arteries. As there is a small left ventricle, there is little voltage from this chamber.

2. C: Fontan procedure

The Fontan operation is a palliative procedure and is usually performed when the patient is 3-5 years of
age. A channel is inserted to drain blood from the inferior vena cava to the right pulmonary artery.
Blue deoxygenated blood then flows directly to the lungs and bypasses the heart. The oxygenated
 blood comes back from the lungs and is pumped by the ventricle to the body.

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CARDIOLOGY ANSWERS

3. F: Blalock-Taussig shunt

A Blalock-Taussig systemic-to-pulmonary shunt will increase pulmonary blood flow in the

severely cyanosed baby with tetralogy of Fallot and a recent history of severe spells of cyanosis. Most of
these children go on to have elective surgical repair at 6-9 months to close the ventricular septal defect
and widen the right ventricular outflow tract.

1.15 The sick newborn infant

1. G: Obstructed total anomalous pulmonary venous connection In total anomalous pulmonary

venous connection, the pulmonary veins do not make the normal connection with the left atrium.
Instead, they can drain upwards to the innominate vein, to the liver or to the coronary sinus. If
the connection becomes obstructed, the baby will present at 1-7 days of life with cyanosis,
acidosis, breathlessness, collapse, and signs of right heart failure, and will require emergency
resuscitation, ventilation and
surgery.

2. F: Interrupted aortic arch

Interrupted aortic arch can occur at any site from the innominate artery as far as distal to the left
subclavian artery. It is a duct-dependent condition and is associated with DiGeorge syndrome. In
DiGeorge syndrome, there is cardiac disease, 22q11.2 gene deletion, absent thymus, absent
parathyroids, abnormal facies, and cleft palate. Affected babies usually present with absent left
brachial and femoral pulses, and signs of heart failure when the ductus arteriosus closes.

3. I: Ebstein anomaly
In Ebstein anomaly, the tricuspid valve is abnormal: the posterior leaflet of the tricuspid valve
originates within the right ventricular cavity, which is thus 'atrialised'. A huge right atrium
results from the inability of the right ventricle to pump blood forward into the pulmonary artery and
therefore sometimes a palliative Blalock-Taussig shunt has to be inserted. Affected babies present with a
loud heart murmur (tricuspid regurgitation), a superior axis on the ECG, and reduced pulmonary
vascular markings on chest X-ray, with an enlarged cardiac silhouette.
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2. Child Development, Child

Psychiatry and Community Paediatrics

Multiple True-False Questions

2.1
Characteristics of fragile X syndrome include:

A micro-orchidism in postpubertal males

B male preponderance
C typical facial features of micrognathia and long facies D poor eye contact
and social impairment
E absence of learning disability in all females

2.2 Regarding gross motor development and posture,

A a 6-week-old lying in the supine position will show extension at the
elbows, hips and knees
B a 2-month-old held in ventral suspension will not be able to hold
his head in line with his body
C a 3-month-old in the prone position will lift his head and upper
chest, supported by his forearms
D a 6-month-old would be expected to roll over
E a 6-month-old would be expected to sit unsupported and reach out
 for nearby objects

2.3 A typical 1-year-old is able to

2.4
A feed himself with a biscuit

B use the index finger to point to what he wants

C pick up small crumbs with the thumb and index finger
D look for toys he has dropped
E bang together two objects in the midline

Criteria for a successful screening test include:

A the condition is important and rare
Ban identified and available treatment pathway
C moderate numbers of true positives (sensitivity)
D early intervention to be as effective as late intervention at
improving outcome
E acceptability to the patient and family

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2.5
CHILD DEVELOPMENT
-

QUESTIONS

A typical 5-month-old should display the following visually directed behaviours:

A grasp a small-sized object

B follow large objects to the midline
C look at a face

D follow large objects beyond the midline

E follow large objects over 180°
 2.6 Medical complications of bulimia nervosa include:

A fluid and electrolyte imbalance

B thyroid gland enlargement
C peri-oral callus formation
D enamel erosion of the teeth Eoesophagitis

Best of Five Questions

2.7 A 7-year-old girl attends the outpatient clinic with a history of

day- and night-time wetting. She has never been fully continent of urine; she passes small
amounts of urine regularly throughout the day, becoming more frequent in the
afternoon. Her mother's pregnancy and the girl's delivery had been uneventful. The girl
has not had any significant illnesses, and is developmentally normal. She lives with her
parents and two older siblings. She says she is happy at home and at school. She is
progressing well with her school work. She doesn't enjoy sports at school and often misses
physical education lessons. She appears to be embarrassed by her problem, and her
parents seem appropriately concerned. On examination she looks well. Her general examination
and neurological examination are normal. Her spine is normal. There is no palpable bladder or
kidneys. Her urine dip is negative for leukocytes and nitrites. Which of the following is the
MOST likely diagnosis?
A Ectopic ureter
B Neurogenic bladder
C Urinary tract infection
D Delayed maturation of urethral sphincter control
E Detrusor instability

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QUESTIONS
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2.8
An 8-year-old girl attends the outpatients department with a 3- month history of
 non-attendance at school. She says little in the clinic room. Parents describe how she had seemed happy
very happy with her
at school at the end of the previous term, and her teacher had been
academic progress and behaviour in school. She is the younger of two children and
lives with both her parents. The parents do not report any difficulties at home. They have
both worked full-time since she was a baby. Her child- minder has been taking her to school with
her sister, but when they arrive at the school gate she becomes distressed and will not enter the
playground. Her mother's pregnancy and the girl's delivery had been uneventful. Her general
health is fine. Developmentally, she appears to be age-appropriate. She has not had her full
schedule of immunisations because her parents did not want her to have her MMR vaccine at
the pre-school vaccinations. Her examination is unremarkable. Which of the following is the MOST likely
cause of her school non-attendance?

A Truancy
BSchool phobia
C Conduct disorder

D Depression
E Bullying

2.9 A 3-year-old boy is referred to the outpatient department with concerns about his development.
The pregnancy and neonatal history were uneventful, and he has had no serious illnesses. He has
always been slow in his development. His mother also had mild learning difficulties, and needed
extra help in school. On assessment, he can walk but cannot jump. He has poor eye contact,
has no clear words, and can not identify parts of his body. He is unable to copy a horizontal
line, and is still in nappies. Examination is very difficult, because he becomes very distressed and
demonstrates hand flapping. His head circumference is on the 90th centile. Which of the following is the
MOST useful investigation?

A Thyroid studies
B Magnetic resonance imaging (MRI) brain scan
C Urinary organic acids
D Genetic studies

E Electroencephalogram (EEG)

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CHILD DEVELOPMENT
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QUESTIONS
2.10 A 3-year-old boy is referred to the outpatient department with
concerns about speech and language development. The
pregnancy and neonatal history were uneventful and he has had no serious illnesses. At 15
months he had five clear words that he has since stopped using. He enjoys puzzles, is able to
feed using a spoon and fork, and can dress himself with help. There has been difficulty settling him
into nursery. He becomes very distressed and runs around being disruptive and sometimes
physically aggressive towards other children. On assessment he is very difficult to settle and
tries to escape from the room. He does not demonstrate any clear words or understanding of simple
commands and refuses to engage in any activities. Which of the following is the MOST likely
diagnosis?
A Attention deficit disorder

B Specific speech and language delay

C Autistic spectrum disorder
D Deafness

E Global developmental delay

2.11 A 14-year-old girl is seen in clinic with a history of a severe sore

throat and fever 4 months previously. Since then she has had headaches, generalised muscle
pain, tiredness, and poor concentration of increasing severity. For the past 2 months she has been
unable to attend school because of her muscle pain and lethargy, and her sleep pattern has become
disturbed. She finds it very difficult to fall asleep at night, and then has difficulty getting up in the
morning. On examination she has mild lymphadenopathy and looks pale. Her weight is
continuing along the 25th centile. Examination is otherwise unremarkable, except for the
marked muscle pain she experiences on movement of her limbs. Which of the following is
the MOST likely diagnosis? A Depression
B Chronic fatigue syndrome
C Hypothyroidism
D Crohn's disease
E Anorexia nervosa

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 CHILD DEVELOPMENT QUESTIONS

2.12 A 13-year-old girl is seen in clinic with a history of weight loss

over the previous 6 months. Her weight has gone from the 75th to the 3rd centile and her height has
continued along the 50th. Apart from her periods, which have stopped, her general health
has been fine. She is in the county's gymnastic squad, but over the past 2 months she has
missed training sessions. Her examination is unremarkable. Which of the following is the MOST
likely diagnosis?

A Depression
B Chronic fatigue syndrome
CHyperthyroidism
D Crohn's disease

E Anorexia nervosa

2.13 A 15-month-old child is seen in clinic with a 3-month history of collapsing. The episodes occur
when he has been hurt. He
17

suddenly looks very pale, loses consciousness, and falls to the floor. He then appears to stiffen, and has
some clonic movements of his arms and legs before quickly recovering. Examination is
unremarkable. Which of the following is the MOST likely diagnosis?
A Prolonged QT syndrome
B Reflex anoxic seizures C Complex partial epilepsy D
Tonic-clonic seizures

E Myoclonic seizures
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CHILD DEVELOPMENT
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QUESTIONS

Extended Matching Questions

2.14 Theme: Speech delay

A Electroencephalogram (EEG)
B Urine organic acids
C Creatine kinase

D Brain magnetic resonance imaging (MRI)

E Hearing assessment
F Lactate

G Genetic studies for fragile X

H Electrocardiogram (ECG)
| Ammonia

For each patient below, select the most appropriate investigation from those listed above. Each option
may be used once, more than once, or not at all.

1. A 3-year-old boy presents with loss of language skills. Speech devel-

opment appeared to be normal until his 3rd birthday, following which he has become progressively more
aphasic.
2. A 3-year-old boy presents with delay in expressive speech and
comprehension. Questioning reveals that he walked at 21 months and had his first clear
word at 2 years. His eye contact is normal, but it is difficult to keep him on task. On developmental
assessment, he can copy vertical lines, but not a circle. He can identify animals in a book, and
one colour. Expressively, he is not putting two words together. He is still wearing nappies.
3. A 3-year-old boy presents with delay in expressive speech and
comprehension. On questioning, he walked at 24 months and had his first clear word at 2
years. His eye contact is poor, and it is very difficult to keep him on task. On
developmental assessment, he can scribble, but prefers fiddling with the crayons and
chewing them. He cannot point to parts of his body, and grunts in response to questions.
No clear words are demonstrated. He is still wearing nappies. He has an uncle, now aged
28, who is known to have developmental delay and was very slow to walk.

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QUESTIONS
19

2.15 Theme: Children Act

A
Place of Safety Order
B
Care Order

C
Wardship
D
Child Assessment Order

F
Police Protection Order
E Supervision Order
 G Emergency Protection Order (EPO)
H Statementing Order
Identify the following orders of the Children Act 1989 below from those listed above. Each option may be
used once, more than once, or not at all.

1. This order lasts for a maximum of 8 days, with a possibility of exten-

sion for a further 7 days. This order may be granted by the court if there is reason to believe that a child may be
harmed if not removed from their present accommodation.
2. This order confers parental responsibility on the social services (in
addition to that of the parents), and usually involves removal from home. It may be
applied for in cases of non-accidental injury in which enquires will take some time, and
when the child is not regarded as being safe at home.
3. This order may be used if there is a situation of persistent but non-
urgent suspicion of risk. It overrides the objections of a parent to whatever examination or assessment is needed to
see whether the child is at significant risk of harm. This order lasts up to 7 days.

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ANSWERS

MT-F Answers
 2.1
Characteristics of fragile X syndrome: B D

Fragile X syndrome is an X-linked condition affecting 1 in 4000 males and is characterised by

learning disability. It was first recognised following an observation that there were many more males
than females with learning disability. The fragile X chromosome marker is a breakage gap at the distal q
arm of the X chromosome. At this site, a CGG triplet expansion inactivates distal genes. Fragile X
syndrome causes moderate to severe learning disability in males, and can cause learning disability
in carrier females. It is characterised by a long face, prominent ears and jaw, and macro-orchidism in
postpubertal males. Behavioural characteristics include social impairment, poor attention, and
difficulty in making and sustaining eye contact.

2.2 Gross motor development and posture: CD

At 6 weeks, a baby placed in the supine position will classically show a flexed posture at the elbows,
hips and knees. An extended posture may indicate cerebral palsy. A 6-week-old held in ventral
suspension will be expected to be able to hold his head in line with his body. At 3 months, a baby
placed face-down should be able to lift their head and upper chest, supported by their
forearms. Rolling over is usually achieved by 6 months. It is not until 9 months that a baby would
be expected to sit totally unsupported while reaching out to grasp objects.

2.3 Typical 1-year-old: A CDE

At 9 months of age a child will look for toys they have dropped and will have developed a mature grip
between the thumb and index finger. By 1 year of age, they can use this pincer grip to pick up small
crumbs. At 1 year, a child is expected to be able to bang together two objects in the midline and
to feed himself a biscuit. By 15 months a child should be able to point to what he wants, using his
index finger.

2.4
Criteria for a successful screening test: B E

Successful screening tests require the condition to be important, with a well-understood natural history and
epidemiology. The test itself should be cost-effective, acceptable to the patient and family, and
sensitive (high number of true positives) and specific (low number of false negatives); the pathway for
further diagnostic evaluation should have been agreed. The treatment pathway should be clearly
understood, available, evidence- based, and should provide an early intervention that improves
outcome. The screening programme should be effective at reducing mortality and

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ANSWERS
21

morbidity, cost-effective, and acceptable to the community as a whole. It will require continuing quality
assurance and audit.

2.5 Visually directed behaviours: B C D E

In the first year, visually directed behaviours develop in response to normal visual system
maturation. It is normal for an infant to look at a face before 6 weeks, follow large objects to the
midline from 2 weeks to 2 months, and beyond the midline at 2-3 months of age. Following large
objects over 180° occurs between 3 and 5 months. Grasping small objects is a later skill, developed from
9 months of age. Visually directed behaviours are crude measures of the visual system, but do
highlight more severe visual impairments.

2.6 Medical complications of bulimia nervosa: A D E

The repetitive binge eating and vomiting in bulimia nervosa can result in fluid and electrolyte imbalance.
Excessive use of laxatives and diuretics can also exacerbate metabolic disturbance. Salivary gland
enlargement and oesophagitis are common. Callus formation on the knuckles occurs from using the
hand to induce vomiting. Enamel erosion occurs as a result of the acidity of regurgitated stomach
contents. Thyroid gland enlargement is not characteristic of bulimia nervosa.

Best of Five Answers

2.7 E: Detrusor instability

Primary daytime enuresis is a lack of attainment of bladder control in the daytime and is often
accompanied by night-time incontinence of urine. An organic aetiology is present in fewer than
5% of cases. In the majority of cases, there is a delay in the maturation of normal urethral
sphincter control mechanisms. There are often co-existent psychological or developmental problems.
In this patient an ectopic ureter is unlikely, as incontinence would be worse in the morning
from pooling of the urine in the bladder overnight. Normal neurology, spine, and a non-distended
bladder make neurogenic bladder unlikely. Urinary tract infection in light of a negative urine dip for
leukocytes and nitrites is unlikely, although in this case a clean-catch urine sample should be sent
for microscopy, culture and sensitivity. Delayed maturation of sphincter control is possible,
although there seem to be no psychological, developmental, or behavioural problems, and no
obvious family stressors. Detrusor instability is a result of

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CHILD DEVELOPMENT
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ANSWERS

intermittent contraction of the detrusor muscle during the filling and storage stage of the bladder when it
should be relaxed, producing urine leakage and urge incontinence. It is more common in girls, and
produces minor degrees of wetness that becomes worse in the afternoon. Treatment involves regular
bladder emptying and positive reinforcement. Oxybutynin therapy has been used to stabilise
the detrusor muscle.

2.8 B: School phobia

Non-attendance at school is a complex problem. There are three main types of school non-attendance:

2.9
Children who have a good reason not to attend, for example because of bullying or an inability to
cope with school work.
The so-called neurotic disorders of school refusal. These can result from separation anxiety (for example, fear
of being away from parents or carers) or school phobia (a fear of school). School refusal is more
common in children of primary school age who come from stable family backgrounds. They tend
to be the only or youngest child, and are usually doing very well academically. In this girl,
school phobia would be the most likely diagnosis, as the fear and difficulty arise on her
arrival at school, as opposed to on separation from the parents. Truancy, a conduct disorder
that is more common in children of secondary school age. They tend to exhibit antisocial behaviours
both at home and in school, and are generally lower achievers. Children who truant tend to come
from larger and more chaotic families with poor adult supervision. School refusal carries
a more positive prognosis than school truancy.

D: Genetic studies

The history tells us that the child is a boy with a mother with learning difficulties. This should always
raise concern regarding fragile X and Duchenne muscular dystrophy. The examination reveals a
 child with significant developmental delay, autistic features and a large head, all in keeping with a
diagnosis of fragile X. The other investigations are reasonable in a child with developmental
delay, but in this individual are less likely to provide a diagnosis.

2.10 C: Autistic spectrum disorder

From the history, some of this child's development is age-appropriate, excluding global
developmental delay. The history and his behaviour in clinic make specific speech and
language delay and deafness unlikely. Attention deficit disorder is a possibility, but it would
not explain his

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CHILD DEVELOPMENT
ANSWERS
23

regression of language at around 15 months of age and the severe delay in speech and language
since. It is also unusual to make a diagnosis of attention deficit disorder in such a young child. Autistic
spectrum disorder would explain all the findings and is the most likely diagnosis.

2.11 B: Chronic fatigue syndrome

The lack of weight loss excludes anorexia nervosa and makes Crohn's disease unlikely. If hypothyroidism
was severe enough to cause her to take 2 months off school, her weight would have been expected to
increase, thus excluding hypothyroidism as a diagnosis. Depression is a possibility, but the sleep
pattern is not typical. Chronic fatigue syndrome often develops after a viral illness. Headaches,
generalised muscle pains, poor concentration, and sleep disturbance are all features of the illness.
Pallor and mild cervical lymphadenopathy are common. Weight is usually static or can increase slightly as
a result of lack of exercise.

2.12 E: Anorexia nervosa

Anorexia is the most likely diagnosis. Crohn's disease is a possibility, but there is no history of any bowel
disturbance, despite a dramatic loss in weight. If the patient had hyperthyroidism there should have been
some clinical signs on examination, such as tachycardia, sweaty palms or goitre. The history is
also not typical of chronic fatigue syndrome.

2.13 B: Reflex anoxic seizures

Although the child appears to stiffen and has some clonic movements of his limbs, complex partial
epilepsy, tonic-clonic seizures, and myoclonic seizures are unlikely as the events always occur after
a painful stimulus. Reflex anoxic seizures have rapid onset, and the child looks pale, becomes
bradycardic, and sometimes asystolic; there is a brief convulsion and a quick recovery. Tiredness
and pallor may persist for a short time following a seizure. Reflex anoxic seizures are also known as
pallid breath-holding spells. Cyanotic breath-holding spells occur in children younger than 3
years and involve a prolonged expiratory apnoea and cerebral anoxia. The child becomes cyanotic,
collapses, and has brief tonic-clonic movements. This condition tends to be self-limiting.

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ANSWERS

EMQ Answers

2.14 Speech delay

1. A: Electroencephalogram (EEG)
The diagnosis of Landau-Kleffner syndrome should be considered in a child who loses language skills.
The onset is usually between 3 and 9 years and is characterised by rapid loss of language skills after
initial normal language development. There are typical EEG abnormalities, and the syndrome has a
variable prognosis. Most patients will still have language difficulties as adults, ranging from
those with almost no verbal ability to those with mild deficits in verbal communication.

2. C: Creatine kinase

This child is showing mild delay in all areas of his development, more marked in speech and language.
The diagnosis that needs to be excluded is Duchenne muscular dystrophy, particularly as he only walked
at 21 months. Boys with Duchenne muscular dystrophy usually have associated learning difficulties, and
often present to the medical professional with concerns about speech and language before any
muscle weakness is apparent.

3. G: Genetic studies for fragile X

This child is showing significant developmental delay in all areas of his development. He is also
demonstrating some autistic features, with poor eye contact, chewing, and fiddling. The
information about the uncle suggests a genetic disease such as fragile X. Duchenne muscular
 dystrophy is not likely, as affected individuals do not usually survive into their late 20s.

2.15 Children Act

1. G: Emergency Protection Order (EPO)

This order lasts for a maximum of 8 days, with a possibility of extension for a further 7 days.
This order may be granted by the court if there is reason to believe that a child may be harmed
if not removed from their present accommodation.
The Emergency Protection Order replaced the Place of Safety Order, and may be granted by
the court if one of the following is satisfied:
There is reasonable cause to believe that the child is likely to suffer appreciable harm if not removed
from their present accommodation. Enquiries by the local authority are being frustrated by lack of
access. In addition, a child likely to suffer significant harm may also be taken into police
protection for 72 hours using a Police Protection Order; this involves a decision internal to the
police force, and is quicker than applying for an EPO.

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2. B: Care Order
CHILD DEVELOPMENT ANSWERS
25

This order confers parental responsibility on the social services (in addition to that of the parents) and
usually involves removal from home. It may be applied for in cases of non-accidental injury
where enquires will take some time, and when the child is not regarded as being safe at home. Care
orders can be taken out by a court if they are convinced that the threshold for appreciable harm to the
child has been met.

3. D: Child Assessment Order

This order may be used if there is a situation of persistent but non-urgent suspicion of risk. It
overrides the objections of a parent to whatever examination or assessment is needed to see whether the child
is at significant harm. This order lasts up to 7 days.
A Supervision Order gives the social services the power and duty to visit the family and also to impose
conditions, such as attendance at a clinic, nursery, school, or outpatient visits. If insufficient powers are
available via the Children Act, then Wardship via the High Court may be applied for. This gives the
court virtually unlimited powers, and is used in exceptional circumstances, such as when a family
objects to medical treatment because of religious reasons. The Statementing Order does not exist.

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3. Clinical Pharmacology and

Toxicology

Multiple True-False Questions

3.1 Concerning the suitability of the excipients in a medicine for use

in neonates,

A benzyl alcohol should be avoided in injectable preparations

B propylene glycol must be avoided at any concentration
 C lactose is contraindicated
D sugar-free medicines are not essential
E alcohol concentration can cause intoxication in infants

3.2 The following statements relating to paediatric dosing that

requires alteration of a product designed for adults are true: A it is never acceptable to cut a
coated tablet
B dispersible tablets always form a uniform concentration when
dissolved in water
C injectable product may occasionally be administered orally
D not all 'non-coated' tablets are dispersible in water
E suppositories are easily cut to give accurate dosing to children

3.3 Concerning accurate paediatric dosing,

A to obtain any child's dose, divide the adult dose by 70 and multiply
by the child's weight
B the surface area equates more accurately with the extracellular fluid
than does weight
C the adult dose is usually taken as the upper dose for children D doses should never be
rounded to avoid using decimal points E adult dosing is always more logical than
children's dosing

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CLINICAL PHARMACOLOGY AND TOXICOLOGY QUESTIONS

3.4 Prescribing outside a product licence:

A it is poor practice to prescribe outside a licence

B information from the manufacturer that must be given out with a
 product may state that the product is not suitable in children
27

C all information in the British National Formulary (BNF) is covered by

the licence

D all information in the BNF for Children is covered by the licence E carers must give formal consent for
children in their care to receive
medicines that are outside their licence

3.5 Therapeutic drug monitoring may be indicated in the following

circumstances:

A to confirm toxicity
B to check compliance
C to confirm steady state
D to monitor plasma levels if an interacting drug has been added
E to monitor plasma levels of a hepatically cleared drug if renal
function has changed

3.6 The following drug interactions are known:

A phenytoin increases the plasma concentrations of theophylline

B erythromycin increases the chances of bleeding in children stabilised
on warfarin

C carbamazepine is an enzyme inducer

D girls taking the oral contraceptive pill may need extra protection if
rifampicin is started
E omeprazole can decrease the level of some drugs that are
metabolised by the liver
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CLINICAL PHARMACOLOGY AND TOXICOLOGY
QUESTIONS

Best of Five Questions

3.7

3.8
 3.9

A neonate of 2 kg requires amphotericin B at a dose of 1 mg/kg to treat a systemic

fungal infection. A single-lumen peripheral line is in situ. What would be the BEST option for
administering the medicine?

A Insert a central line

B Mix the amphotericin with 0.9% sodium chloride

C Give the amphotericin undiluted
D Mix the amphotericin with 5% glucose
E Mix the amphotericin with 5% glucose and a buffer

A 5-year-old requires a dose of oral liquid nifedipine at a dose of 5 mg twice a day. The
preparations available to prescribe are: liquid-filled capsules, modified-release tablets, injection,
and an imported liquid not licensed in the UK. What would be the BEST option for
administering the nifedipine?
A Crush the tablets and sprinkle over food
B Crush the tablets and put in 5 mL of water
C Remove the liquid from the capsules
D Give the content of the injection orally
E Use the imported liquid which is not licensed in the UK

A pre-term neonate who weighs 2.5 kg and is 3 days old requires a higher dose of
benzylpenicillin on a mg/kg basis than an adult to achieve the same plasma concentration.
Which of the following BEST describes why this should be?

A Benzylpenicillin crosses the blood-brain barrier in neonates more

readily
B Neonates have a greater volume of distribution for water-soluble
drugs
C Benzylpenicillin is water-soluble and distributes to the kidneys at a
faster rate in neonates

D Benzylpenicillin is excreted from the kidney more slowly in neonates E The protein binding of
benzylpenicillin is lower in neonates
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QUESTIONS

3.10 The therapeutic plasma range of phenytoin is lower in neonates

than in children. What is the MOST likely reason for this?
A Less phenytoin is needed to treat seizures on a mg/kg basis
29

B Bilirubin displaces phenytoin from albumin more readily in neonates C Decreased albumin levels lead to
increased 'free' phenytoin
D Phenytoin is more toxic in neonates
E Phenytoin is excreted more slowly in neonates

3.11 Chloramphenicol may cause 'grey baby syndrome' in neonates.

What is the MOST likely reason for this?

A Chloramphenicol is able to cross the blood-brain barrier B Neonatal livers are

unable to glucuronidate high doses of
chloramphenicol
C Chloramphenicol is glucuronidated to a toxic metabolite in
neonates

D Grey baby syndrome is an idiosyncratic reaction to chloramphenicol

in neonates

E At birth, neonatal sulfation is more established than is

glucuronidation

3.12 A neonate born at 37 weeks of gestation presents at 2 weeks old.

The baby was commenced on phenytoin 2 days previously and the plasma level of
 phenytoin 8 hours post-dose was 8 mg/L. At presentation the infant is still having seizures.
What would be the MOST appropriate clinical approach?

A Leave the prescription the same, because steady state hasn't been
reached

B Double the dose and monitor in another 3 days

C Increase the dose by about 20% and monitor the child
D Monitor the albumin to see if it is a true level

E Stop the phenytoin and start another anticonvulsant

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CLINICAL PHARMACOLOGY AND TOXICOLOGY
QUESTIONS

3.13 A 10-kg 14-month-old boy is brought to the accident and

emergency department by his parents. His parents claim that he possibly ingested
paracetamol, as six 500-mg capsules were found to be missing from the family's
paracetamol container. This possible ingestion occurred within a 60-minute window before
their presentation to the Emergency Department. Of the five choices below, which is the
MOST appropriate immediate management action?
A Measure plasma paracetamol levels
B Administer ipecacuanha to induce emesis C Perform a gastric lavage
D Administer N-acetylcysteine
E Administer activated charcoal

Extended Matching Questions

 3.14 Theme: Interactions

A Drug/drug
B Drug/diluent
C Drug/flush
D Drug / plastic
E Drug/acid
F Drug/food G Drug/water
H Drug/alkali

For each of the scenarios below, select the most likely interaction to account for the loss of efficacy of the
drug. Each option may be used once, more than once, or not at all.

1. Gentamicin is being given to a neonate for a Gram-negative

infection. After the addition of benzylpenicillin co-administered down the same line, the levels of
gentamicin are decreased.
2. A child receives insulin that was mixed in a 500-mL bag of sodium chloride. Over the course of
24 hours, the insulin becomes less effective.
3. A child is receiving omeprazole tablets that are crushed and
dissolved in water before administration. A nasogastric tube becomes necessary during
the child's management. After its insertion, the effect of the administered omeprazole is
seen to be much less than before the tube was inserted.

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CLINICAL PHARMACOLOGY AND TOXICOLOGY QUESTIONS

3.15 Theme: Neonatal pharmacology

A Gut motility
B Gut acidity
C Hepatic glucuronidation
D Hepatic sulfation
E Renal function

F Protein binding
G Volume of distribution

H Permeability of the blood-brain barrier

‫اليا‬
Select the process which is most likely to be altered (from that of children and adults) and so be
responsible for the following situations in neonates. Each option may be used once, more than
once, or not at all.
1. A 2-week-old term baby requires a higher dose (on a mg/kg basis)
of a water-soluble drug in order to achieve the same plasma concentration.
31

2. Addition of co-trimoxazole to a neonate with high plasma levels of

bilirubin can quickly become life-threatening.
3. Dosing of benzylpenicillin in a 1-week-old, 30-week gestation
neonate is usually at a frequency of two or three times a day, as opposed to four times a day in
children.
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CLINICAL PHARMACOLOGY AND TOXICOLOGY ANSWERS

MT-F Answers

3.1 Drug excipients: A E

Benzyl alcohol can cause severe respiratory problems in neonates, and thus should be avoided. Some medicines
contain so much alcohol that even normal doses in the very young can cause intoxication. Propylene glycol is
hypertonic, and fast administration of high quantities can cause haemodynamic instability.
However, small amounts infused slowly are acceptable. Lactose may be used in any patient, providing
they are not lactose intolerant. Sugar-free medicines are desirable in all children, because sugar can
cause dental caries; however, the administration of small amounts of sugar-containing medicines for short
periods of time is acceptable.

3.2
Alteration of a product for paediatric dosing: C D

Although it is rarely desirable, injectable products may occasionally be used orally or

 intranasally, although the pH often means that the liquid is not very palatable. Plain tablets with
no special release mechanisms may be suitable for crushing, but the particles may not disperse in
water at all, leaving all the particles on the bottom of the vessel. It is never desirable to cut a
coated tablet, but in the absence of any other product it may be acceptable, if the coat has no
modifying-release properties such as a basic sugar coat. Just because a tablet is dispersible, it doesn't
mean that it dissolves to form a uniform solution. Suppositories may not have the medication
dispersed evenly throughout the product, and thus cutting is not desirable in terms of accurate dosing.

3.3
Paediatric dosing: B C
Most drugs are distributed in the extracellular fluid, and so basing dosing on a child:adult surface area ratio of
an adult dose is more accurate than using a weight ratio, as the extracellular fluid is more closely related to
the surface area than to the weight. Although some children may theoretically require
larger doses than an adult because of their size and liver function, the adult dose is usually taken
as the maximum dose. Most adults get the same dose of a drug independent of their size (eg
paracetamol); their plasma levels may therefore vary enormously. Dosing like this only works because
most drugs have wide therapeutic ranges. Because of the wide therapeutic ranges, rounding of
doses to avoid decimal points is usually good practice; however, this sometimes causes large
variance in doses, and might not be acceptable for dosing within a narrow therapeutic range.

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CLINICAL PHARMACOLOGY AND TOXICOLOGY ANSWERS

3.4 Prescribing outside a product licence: B

33

Prescribers can prescribe irrespective of whether something has a licence or not, but the prescriber
must be aware of the clinical evidence and the quality of the product they are prescribing. A
company's patient information leaflets must legally be given out with a licensed product, even if
it is contrary to what the product is being used for. Most information in the BNF is covered by the
manufacturer's licence, but not all. The BNF for Children contains a lot more information that is not covered by the
licence. It is good practice to discuss with a patient all their medicines and their evidence base.

3.5 Therapeutic drug monitoring: A B D

Therapeutic drug monitoring should only be carried out if there is a real need to do so. Toxicity is
often diagnosed by symptoms, and can occur at normal plasma levels. However, if toxicity is
 expected, therapeutic drug monitoring should be carried out to ascertain the current level. If non-
compliance is expected, therapeutic drug monitoring may confirm the situation. If a patient is
stable and their plasma levels known, then therapeutic drug monitoring should be carried out after
an interacting medicine has been added, to observe what is happening to the patient. Steady state is a
stable plasma level reached after about 5 half-lives of the drug. Therapeutic drug monitoring is
normally carried out after steady state has been reached, rather than to confirm steady state. If a
drug is hepatically cleared, its levels should not be altered if renal function changes, and thus no
additional monitoring is needed.

3.6 Drug interactions: B C D

Erythromycin is an enzyme inhibitor and thus increases the levels of warfarin, leading to potential
bleeding. Carbamazepine is an enzyme inducer. Rifampicin is an enzyme inducer that increases the
metabolism of the oral contraceptive pill, leading to a greater chance of conception. Phenytoin is an
enzyme inducer, and thus decreases the levels of the hepatically cleared theophylline. Omeprazole
is a weak enzyme inhibitor, and thus increases the levels of some drugs that are metabolised by the
liver.

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CLINICAL PHARMACOLOGY AND TOXICOLOGY ANSWERS

Best of Five Answers

3.7 E: Mix the amphotericin with 5% glucose and a buffer

Amphotericin B does not need to be administered through a central line, although if treatment is
long-term it may be preferable. It must be diluted and given as an infusion. It is not stable in acid
solutions, so 5% glucose is not appropriate; however, it reacts with chloride ions, making it
incompatible with sodium chloride. Ideally, it is put in glucose that has been neutralised with buffer, and this is
the normal method of administration.

3.8 E: Use the imported liquid which is not licensed in the UK Sprinkling crushed tablets over food
for children is not advisable, as not all the food may be eaten. Crushing the modified-release tablets
 in water will change the properties of the modified release, but also these tablets don't disperse
very well, and thus the whole dose may not be given. The liquid may be withdrawn from the
capsules, but this is a difficult process and knowledge is needed of how much of the liquid holds the
actual dose, and this will vary with different brands. It may be possible to give the injection orally,
but this is expensive and not at all pleasant. An imported product that is licensed in its country
of origin is often the best option, providing care is taken if documents need translation.

3.9 B: Neonates have a greater volume of distribution for water-

soluble drugs

All the answers are true for benzylpenicillin in the neonate, but the reason for the higher dose is
only due to the higher volume of distribution caused by the increased extracellular fluid.

3.10 C: Decreased albumin levels lead to increased 'free' phenytoin

The main reason for the lower plasma phenytoin levels is the decreased
albumin concentrations. The phenytoin excretion is reasonable from birth. Albumin binding is not as
tight as it is in children, but bilirubin rarely plays a part in displacing large amounts, unless levels
are very high. The overall plasma concentration of phenytoin needs to be lower because there is
more free (non-bound) drug and thus dosing can be reduced compared with that in adults and
children.

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CLINICAL PHARMACOLOGY AND TOXICOLOGY ANSWERS

3.11 B: Neonatal livers are unable to glucuronidate high doses of

chloramphenicol

Glucuronidation is one of the last metabolic processes to be established in neonates. Some drugs are
sulfated in preference, but chloramphenicol is not. Thus high doses of chloramphenicol are not able
to be metabolised, and toxic levels pass into the central nervous system and other organs, to
produce the often fatal effects of the grey baby syndrome.
35
3.12 C: Increase the dose by about 20% and monitor the child Phenytoin demonstrates saturable
kinetics within the therapeutic range (10-20 mg/L). This means that large dose adjustments just
outside the range or within the range are likely to lead to very large variations in plasma levels and to
render the child toxic. Although the levels are likely to increase a little over the next few days until steady
state is reached, this is unlikely to be adequate or fast enough for this patient. Albumin may well be
affecting the level, but the patient needs to be treated now, irrespective of the amount of free
phenytoin. Doubling or re-loading the patient should only be considered in an emergency.

3.13 E: Administer activated charcoal

The most appropriate management in this instance would be to administer activated charcoal, to
limit further absorption of paracetamol. Inducing emesis with ipecacuanha is now generally
contraindicated. Measurement of plasma paracetamol levels is useful only after 4 hours of ingestion. A
gastric lavage may be beneficial, but when you are given these five choices it would not be the
first choice of treatment. N-Acetylcysteine would not be the first line of treatment either.

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 CLINICAL PHARMACOLOGY AND TOXICOLOGY ANSWERS

EMQ Answers

3.14 Interactions

1. A: Drug/ drug
Penicillins interact with gentamicin if they come into contact, and the gentamicin is degraded.

2. D: Drug/plastic
Insulin is absorbed onto plastic and, over time, the amount in the bag is decreased. For this reason, insulin
is usually given via a syringe driver.

3. E: Drug/acid

Omeprazole tablets are made of tiny spheres of coated drug. The drug is absorbed in the
duodenum but is degraded by acid. The spheres' coats are thus acid-resistant. If they are crushed,
the drug will be degraded by the stomach acid, making it less effective. The crushed tablets are
sometimes given mixed with sodium bicarbonate to try to protect the drug.

3.15 Neonatal pharmacology

1. G: Volume of distribution

Neonates have a far greater relative extracellular fluid volume than infants (who have a far greater relative
fat content), so neonates require relatively more water-soluble drug to achieve the same
plasma concentration.

2. H: Permeability of the blood-brain barrier

Co-trimoxazole can displace bilirubin from its albumin-binding sites in any individual. However,
this event can quickly become life-threatening in a neonate. The blood-brain barrier is very
permeable, and allows the increased bilirubin across into the brain. If this happens, the often fatal
encephalopathy of kernicterus will occur.

3. E: Renal function

Benzylpenicillin is cleared renally. Neonates have a reduced renal clearance and they do not require
such frequent dosing, despite requiring the same doses (if not higher) on a mg/kg basis.
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37

4. Dermatology

Multiple True-False Questions

4.1 The normal procedure for a skin biopsy involves

A oral sedation
B uninvolved skin

C taking an elliptical piece of skin and suturing the wound

D a special technique for epidermolysis bullosa
E a scar being left in 50% of cases

4.2 The following are true of skin infections:

A they are usually caused by Staphylococcus epidermidis

B nodules may persist for 3 months after successful treatment of
scabies

C tinea infections are usually due to Microsporum canis

D tinea corporis will show improvement if steroid creams are applied E scalp abscesses
due to fungal infection require incision and
drainage

4.3 The following conditions are more common in children with

atopic eczema:

A juvenile plantar dermatosis

B lamellar ichthyosis
C cataracts

Dpityriasis alba
 E keratosis pilaris

4.4 In infected atopic eczema, the following is true:

A steroid creams/ointments should be stopped in eczema herpeticum
B wet wraps are an effective treatment for infected eczema
C eczema herpeticum is treated with intravenous aciclovir
D failure to respond to a course of oral antibiotics is the result of
methicillin-resistant Staphylococcus aureus (MRSA) infection
E topical antibiotic is the treatment of choice

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4.5

4.6
DERMATOLOGY
QUESTIONS

Blisters are found in the following conditions:

A toxic epidermal necrolysis

B erythema multiforme
C erythropoietic protoporphyria
D Stevens-Johnson syndrome
E granuloma annulare

Nail changes are a recognised feature of:

A epidermolysis bullosa
 B hypothyroidism
C pityriasis rubra pilaris
D alopecia areata
E atopic eczema

Best of Five Questions

4.7

A 2-year-old boy presents to the casualty department with a 3- day history of a high
temperature and widespread erythematous skin, with peeling skin on the fingers. Which of the
following treatments is MOST appropriate?
A Morphine
B Flucloxacillin

C Aciclovir

4.8
D Methotrexate

E Immunoglobulins

Since she was 6 months of age, a 2-year-old girl has been affected by an itchy red rash of the limb flexures,
which comes and goes. Which would be the MOST suitable initial treatment?
A Diet free from cow's milk

B Betnovate cream applied twice daily

C Use of regular emollient therapy
D Pimecrolimus cream (1%) applied to the face
E Tacrolimus ointment (0.03%)

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 4.9
DERMATOLOGY
QUESTIONS

An 8-year-old girl has severe generalised atopic eczema and has attended clinic monthly for the
last 6 months. She has missed nearly 4 weeks of school in the past 2 months. Which treatment
would be MOST appropriate?
A Twice-daily application of emollient wet wraps
B Twice-daily application of clobetasone (Eumovate) cream
C Oral acitretin

D Oral ciclosporin
E Twice-daily application of clobetasol (Dermovate) cream
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39

4.10 A 9-year-old girl has had widespread plaque psoriasis for the past
3 years, affecting her limbs, trunk, and face. Many treatments have been tried without success. She
has missed several weeks of school and has regularly attended the dermatology clinic. Which of the
following therapies is the MOST appropriate?
A Psoralen ultraviolet A (PUVA)
B Vitamin D analogues
C Betnovate 1 in 4 cream
D Methotrexate
E Penicillin V

4.11 A 6-year-old girl complains of a red scaly rash on the limbs. On

examination, you find numerous plaques affecting the arms and legs. Which of the following is
MOST likely?

A Onset of rash was in the first 2 years of life

B Rubbing the skin provokes white scaling C Oral lesions with a white reticulate
surface
D Flexural sites are affected
 E Severe destructive arthritis that symmetrically affects large joints

4.12 An 18-month-old boy is referred by his GP with a 5-day history of

not eating and drinking, being unwell, and having multiple mouth and lip ulcers, and white lesions on the
tongue. This is his first attendance at the surgery because of illness. On examination, he
has a pulse rate of 120 beats/min, a blood pressure of 85/55 mmHg, and his capillary refill
time is less than 2 seconds. Which of the following is MOST important for his care?

A Oral nystatin
B Regular mouth care, including antiseptic mouthwashes
C Intravenous aciclovir
D Intravenous fluids

E Oral paracetamol

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DERMATOLOGY
QUESTIONS

4.13 A 13-month-old boy has an itchy red rash and coryzal symptoms.
Which one of the following is MOST likely?

A The pattern of rash is helpful in making the diagnosis

B The rash will have resolved within a week

C An urticarial rash rarely occurs with viral infections

D The season may give a clue to the diagnosis
E A causal agent is identified in 75% of cases

Extended Matching Questions

4.14 Theme: Skin rashes and viral infections

A Rubella
B Scarlet fever

C Non-specific viral exanthema

D Roseola infantum

E Erythema infectiosum
F Gianotti-Crosti syndrome
 G Herpes simplex virus
H Coxsackie infection

For each child described below with a viral infection and skin lesions, select the most likely condition from the
list above. Each option may be used once, more than once, or not at all.

1. An 18-month-old girl presents with a 2-week history of

erythematous papules that started on the upper legs and buttocks, and then spread to the arms
and cheeks.
2. A 2-year-old girl presents with a 3-day history of a high temperature
and malaise, with vesicles in the mouth and on her thumb.

3. A 9-month-old boy presents with a convulsion. He has had a high temperature for 4
days, suboccipital lymph nodes, and has a pink macular rash that started on the trunk and
spread to the extremities.

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QUESTIONS
41

4.15 Theme: Vesiculopustular lesions

A Impetigo
B Transient neonatal pustulosis
C Scabies

D Erythema toxicum neonatorum

E Urticaria pigmentosa
F Epidermolysis bullosa
G Infantile acropustulosis
H Incontinentia pigmenti
For each infant described below with a vesiculopustular rash, select the condition that is most likely
from the above list. Each option may be used once, more than once, or not at all.

1. A 1-week-old girl is noticed to have vesicular lesions which spare her face. Examination shows that
she is apyrexial, and the lesions follow Blaschko's lines and are in a linear distribution on the limbs and
arranged circumferentially on the trunk.
2. An 8-month-old girl presents with a 6-month history of crops of itchy small
vesicopustules on the hands and feet recurring at monthly intervals.
3. A West Indian baby girl is noticed to have vesicopustules at birth,
some of which have burst, leaving a pigmented macule.

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DERMATOLOGY
ANSWERS

MT-F Answers

4.1 Skin biopsy: D

In epidermolysis bullosa, a shave biopsy is taken after rubbing unaffected skin. For a skin biopsy,
a 3- or 4-mm punch biopsy carried out under local anaesthesia is the normal procedure. Elliptical pieces
 of skin are taken often as part of cot death investigations and when an excision biopsy is needed.
Usually, affected skin is chosen, but unaffected skin is required for some conditions, eg direct
immunofluorescence in linear IgA disease (chronic bullous dermatosis of childhood) and for
epidermolysis bullosa. Although a scar may be left by a punch biopsy (eg in a child who has
previously had hypertrophic scars or keloids), in the majority of cases the biopsy site heals well,
leaving no long-term scar.

4.2
Skin infections: B D

Skin infections in an immunocompetent child are usually caused by Staphylococcus aureus or group
A B-haemolytic Streptococcus. Scabetic nodules are the result of an immune response to the mite,
and often persist for 2-3 months after successful treatment. If new lesions appear, then treatment
has not been successful. The majority of tinea infections are due to Trichophyton species, with
human-to-human spread, rather than Microsporum from animals. Tinea corporis does show temporary
improvement with topical steroid creams, as the redness and inflammation. improve. Pustules
are often prominent and the edge of the lesion is less scaly. The appearance is given the term
'tinea incognito'. Scalp abscesses due to fungal infection are called kerions. These should not
be incised and drained, as a permanent scar will be left. The treatment of choice is antifungal
therapy started without delay.

4.3
Conditions associated with atopic eczema: ACDE

All the conditions except for lamellar ichthyosis are more common in children with atopic eczema.
Atopic eczema is, however, associated with ichthyosis vulgaris, which is the most common form of
ichthyosis, with an incidence of 1 in 250; it is characterised by fine, light, scaly skin, in contrast to the
large, dark plate-like scales of lamellar ichthyosis. Juvenile plantar dermatosis affects the sole of
the foot, with red, shiny skin, often with painful fissures. Anterior subcapsular cataracts affect 4-12% of
children with atopic eczema. Posterior subcapsular cataracts are due to use of either oral corticosteroids
or topical steroid creams or ointments applied to the eyelids or around the eye. In pityriasis alba there
are dry, slightly scaly

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ANSWERS
43

hypopigmented areas, common on the face and upper trunk. Keratosis pilaris is keratin plugging
of hair follicles, with varying degrees of surrounding erythema that tends to be prominent on the
 upper arms and legs.

4.4 Infected atopic eczema: A C

Steroid creams or ointments should be discontinued in eczema herpeticum, as they are thought
to encourage viral spread. Most cases are due to widespread infection of the skin with herpes simplex
virus, and need intravenous aciclovir. Wet wraps should be discontinued if there is overt skin infection.
There are many reasons why children do not respond to a course of oral antibiotics, eg too short a
course given (10 days is treatment duration of choice), flucloxacillin only given, and group A
ẞ-haemolytic Streptococcus is present in addition to Staphylococcus aureus, erythromycin
resistance, nasal Staphylococcus carriage. MRSA colonisation of eczema is increasing amongst
children with chronic eczema - remember that whether to treat is a clinical decision, as most children with
atopic eczema have positive skin swabs for S. aureus. Infection is usually widespread, and needs
oral antibiotics rather than topical ones. Use of topical antibiotics is for localised infections only, and those
such as fusidic acid (Fucidin) should not be used for more than 5 days; otherwise bacterial resistance
occurs.

4.5 Conditions associated with blisters: A B D

In both toxic epidermal necrolysis and Stevens-Johnson syndrome, there is erythematous skin
with bullae. Erythema multiforme characteristically has symmetrical red papules with target lesions, some
of which may blister. In erythropoeitic protoporphyria there is photosensitivity and small pitted
scars on the cheeks, which should not be confused with eczema; blistering is not a typical feature. In
granuloma annulare, lesions are ring-shaped, with skin-coloured papules but not blisters.

4.6 Nail changes: All true

Epidermolysis bullosa is the name given to a group of genetically determined disorders in which the
skin and mucosae are excessively susceptible to separation from the underlying tissues and
blistering following trauma. Nails may be affected in both junctional and dystrophic
epidermolysis bullosa. In hypothyroidism there is decreased nail growth, ridging and
brittleness. Pityriasis rubra pilaris is an erythematous eruption of unknown aetiology characterised
by palmoplantar keratoderma (thickening of skin on palms and soles). There are different types, with nail

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DERMATOLOGY
ANSWERS

features that include thickened curved nails and terminal hyperaemia (half- and-half nail). Nail
changes in alopecia areata are usually in the form of nail pitting. Such changes tend to be greater in those
with more severe disease. In atopic eczema there may be nail pitting, Beau's lines, and
onycholysis (separation of the nail plate from the nail bed).

Best of Five Answers

4.7 B: Flucloxacillin

This is staphylococcal scalded-skin syndrome. Systemic antibiotics are needed. Although

uncomfortable, pain relief with paracetamol / ibuprofen is usually sufficient. Morphine is required in
Stevens-Johnson syndrome or toxic epidermal necrolysis. Aciclovir would be given for herpes
simplex virus (HSV) infection when lesions at different stages would be expected in addition to
erythema. Peeling is not usually found in HSV infection. Methotrexate would be indicated for
acute erythrodermic psoriasis. Infection must always be excluded before making this diagnosis, and a
skin biopsy is recommended. Immunoglobulins would be given for Kawasaki disease; for this
diagnosis, 5 days of fever is required, and skin peeling is a late sign, typically in the second week.

4.8
C: Use of regular emollient therapy

This is atopic eczema. It is the initial treatment that is being considered, and this should include use of
emollients such as bath oils, soap substitutes and moisturisers. A diet free of cow's milk may be
considered for severe atopic eczema, especially in those younger than 1 year of age.
Betamethasone (Betnovate) cream is a potent topical steroid that is not used routinely in the treatment of
childhood atopic eczema. Both topical pimecrolimus and tacrolimus are used for treatment of
moderate to severe eczema that is not responding to conventional treatment.

4.9 D: Oral ciclosporin

This is chronic atopic eczema that is evidently not responding to treatment, as the girl is
coming to clinic monthly and is taking a lot of time off school. A short course of oral ciclosporin
is one of the systemic therapies used for atopic eczema not responding to topical therapies.
Emollient wet wraps may help as part of the treatment regimen, but other therapy is needed, and wet
wraps are often not well tolerated by older children. Clobetasone (Eumovate) cream is a
moderately potent topical steroid cream which would already have been used in this case. Acitretin
is used in the

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ANSWERS
45
treatment of psoriasis and ichthyotic disorders. Clobetasol (Dermovate) cream is a potent
topical steroid cream. Potent steroid creams should be used cautiously in children because of
possible local skin side-effects and, as the eczema is generalised, there should be concern about the
child developing Cushing syndrome.

4.10 D: Methotrexate

This is chronic plaque psoriasis which is relapsing often, and which is not under control. Many topical
therapies will have been used over a 3-year period. Systemic therapy is warranted, and methotrexate is
one of the systemic drugs used for psoriasis. PUVA involves the use of phytotoxic psoralens in
combination with ultraviolet A light, and tends not to be used in children (particularly in those younger
than teenagers) because its use increases the risk of skin cancer. Vitamin D analogues (eg
calcipotriol [Dovonex]) are used for mild to moderate psoriasis when less than 40% of the skin area is
affected. Penicillin V is used in the treatment of guttate psoriasis, which may be triggered by
streptococcal infection, and in other forms of psoriasis only if there is evidence of streptococcal
infection.

4.11 B: Rubbing the skin provokes white scaling

This is chronic plaque psoriasis. If erythematous plaques are present and are due to psoriasis,
then rubbing produces the typical white scale. Less than 2% of psoriasis presents in the first 2 years
of life and 10% of cases will have an age of onset less than 10 years. Girls tend to be affected between
the ages of 5 and 9 years and boys between 15 and 19 years. Lesions in the mouth affecting the buccal
mucosa with a white reticulate surface are typical of lichen planus. Psoriasis tends to affect the
extensor surfaces. Psoriatic arthritis is occasionally severely destructive. It tends to be an asymmetric
arthritis.

4.12 E: Oral paracetamol

This is primary herpes simplex gingivostomatitis. Pain relief is needed, and paracetamol is
appropriate. The tongue is often white, and this is not usually due to Candida. Mouth care is difficult
to perform in children, and most mouthwashes sting, causing further misery to the child. Aciclovir stops
further viral replication, but does not help when there are several lesions present. Some children
treated with aciclovir develop poorer antibody responses. This child is highly unlikely to be
immunocompromised or to have skin disease, as it is his first attendance at the GP surgery.
Intravenous fluids may be needed in very severe cases, but children often start to drink after adequate
analgesia has been given.

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 DERMATOLOGY
ANSWERS

4.13 D: The season may give a clue to the diagnosis

Some infections are more common in certain seasons, eg enteroviruses are more common in summer
and autumn. The pattern of rash may be helpful in making a diagnosis (as in hand, foot and mouth
disease), but is often non-specific in the majority of cases of viral rash. Rashes associated with
viral infections can last for 2-3 weeks, and sometimes longer. Urticarial rashes occur quite often with viral
infections. Most viral rashes are not investigated, and would tend to be only if atypical or if the child had
been in contact with a pregnant mother or immunocompromised child or adult.

EMQ Answers

4.14 Skin rashes and viral infections

1. F: Gianotti-Crosti syndrome
Gianotti-Crosti syndrome (also known as papular acrodermatitis) typically occurs in children of 1-6 years
of age. Symmetrical lichenoid, skin-coloured or coppery-red papules develop and last 3-6 weeks. Lesions
are on the legs and buttocks, and then spread to the arms and face. It is important to recognise
this condition because of the duration of the rash. Many viruses have been linked to Gianotti-Crosti syndrome.
In scarlet fever, the rash starts
in the axillae and groins and consists of fine, red papules. The
erythroderma fades over 2-3 days, followed by desquamation 7-10 days later.

2. G: Herpes simplex virus

In primary HSV infection, children are febrile, miserable, and may have several mouth
lesions. HSV lesions on the digits are common, because children suck their fingers (and toes).
In hand, foot and mouth disease caused by coxsackie infection, the fever is low-grade.

3. D: Roseola infantum

Children with roseola infantum (synonyms - exanthema subitum, sixth disease) are typically aged between
6 and 36 months and have 3-5 days of high fever, with enlarged suboccipital lymph nodes. The
rose-pink rash tends to last for 2 days, starting on the trunk and then spreading to the extremities and
the face. Once the rash comes out, the temperature becomes normal. Human herpesvirus types 6
and 7 have been found in this condition. In rubella there is lymphadenopathy - especially cervical,
postauricular and suboccipital - but fever is low-grade and it is not associated with convulsions. Fever
occurs for 1-2 days in erythema

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ANSWERS
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infectiosum (slapped-cheek disease) caused by parvovirus infection and there is a generalised

maculopapular exanthem, which is often 'lace-like', on the limbs after physical activity.

4.15 Vesiculopustular lesions

1. H: Incontinentia pigmenti

Incontinentia pigmenti is an X-linked dominant condition and is usually lethal in boys. Lesions typically
develop in the first week of life, and the first stage is a vesicular one. Had widespread impetigo
been present, the baby would be expected to have systemic symptoms, including pyrexia. It is rather
early for a scabies rash to develop, and this rash does not follow Blaschko's lines. Lesions are papules as
well as vesicles. Erythema toxicum neonatorum does not follow Blaschko's lines. Epidermolysis bullosa
often presents at birth with absence of skin.

2. G: Infantile acropustulosis
Infantile acropustulosis usually presents in the first few months of life and is characterised by
recurrent crops of vesicopustules on the hands and feet.

3. B: Transient neonatal pustulosis

Transient neonatal pustulosis is characterised by pustules present at birth, some of which may
have ruptured. They leave a pigmented macule, which may persist for up to 3 months. It is more
common in black skin. Urticaria pigmentosa commonly presents in the first year of life but can be
present at birth. These are skin-coloured or red-brown macules which urticate
when rubbed.
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5. Emergency Medicine

Multiple True-False Questions

5.1 The basic life support algorithm includes the following:

5.2
5.3
A two rescue breaths are administered before chest compressions are
commenced

B agonal breaths may be present in the child with cardiac arrest

C the femoral pulse is recommended to check for circulation in
children

D compressions should be at a rate of 80 per minute

E emergency services should be called after 1 minute of
cardiopulmonary resuscitation (CPR)

In the case of asystole,

A the initial dose of adrenaline is 0.1 mL/kg of 1 in 1000 adrenaline B repeat doses of
adrenaline are 0.1 mL/kg of 1 in 1000 adrenaline C in a neonate, adrenaline should be given
down the endotracheal
tube

D in hospital, the ratio of compressions to ventilation for a 7-year-old

is 30:2

E as soon as the child's trachea is intubated, continuous chest

compressions can be commenced

Pulseless electrical activity

A is a shockable rhythm
B has a rhythm on the monitor that is indistinguishable from
ventricular tachycardia
C is a rhythm that may be seen in a patient with hypovolaemia
D requires that a shock of 4 J/kg be given
E may be seen in a child with a pulmonary embolus

5.4 The following is true in cases of choking:

A for the choking child, chest thrusts should be used instead of

abdominal thrusts

B back blows and chest thrusts should be at a rate of 1 per second

C a blind finger sweep may be performed before back blows
D the Heimlich procedure may be useful in an infant
E an effective cough should be encouraged
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5.5
EMERGENCY MEDICINE QUESTIONS

Which of the following statements is true of hypothermia?

A below 30°C, repeated shocks may be needed to treat ventricular
fibrillation

B clotting is typically deranged in hypothermia

C during rewarming, fluid resuscitation should be avoided.
49

D analgesia is recommended when rewarming is done in the presence

of frostbite

Emyocardial dysfunction may only become apparent on rewarming

5.6 With respect to electrocution injury,

5.7

A electrocution occurring outside the home is usually associated with

other injuries
B dysrhythmias are a recognised late complication
C fluid and blood have the most resistance to electricity
D compartment syndrome is a recognised complication
 E lightning injury is difficult to diagnose if not witnessed

When brainstem testing is performed,

A the recorded time of death follows the second set of tests

B the procedure is contraindicated to determine brainstem death in

premature neonates
C two doctors with at least 5 years of post-qualification experience
are required
D oculovestibular reflexes are no longer included
E death is pronounced after the second set of tests

Best of Five Questions

5.8 Which one of the following is the MOST correct concerning

myoglobinuria?
A It is a rare complication of hypoxia
B Diuretics are relatively contraindicated
C Keeping the urine acid keeps the toxin in its ionised form
D Alkalinising the urine reduces the amount of resorption in the renal
tubule

E The use of sodium bicarbonate should be avoided

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EMERGENCY MEDICINE QUESTIONS

5.9 Which one of the following statements is correct regarding

airway resistance?

A It is directly proportional to the radius squared

B It is directly proportional to the radius to the power of 4
 C It is inversely proportional to the radius to the power of 4
D It is inversely proportional to the radius squared
E It is inversely proportional to the radius

5.10 In burns, which one of the following indications does not merit
referral to a burns unit in a child?

A An 8% partial-thickness burn to leg BA 6% full-thickness burn to

arm CA 3% full-thickness burn to hands

D A high-voltage burn to arm

E A circumferential burn of one arm

5.11 Which one of the following interventions is not a recognised

neuroprotective treatment following head injury?

A Draining cerebrospinal fluid from a ventricular drain

B Raising the head 20-30 degrees and keeping it in the midline C Aiming for high serum sodium
levels (greater than 140 mmol/L) D Hyperventilation aiming for a PCO2 between 3
and 3.5 kPa E Tight control of glucose using insulin for high glucose levels

5.12 Which one of the following statements is correct concerning

inhaled nitric oxide?

A It relaxes vascular and smooth muscle by stimulating production of

cyclic adenosine monophosphate (CAMP)
B It has marked effects on both pulmonary and peripheral vasculature C It suppresses
endogenous release of nitric oxide from the respiratory
mucosa

D It rarely improves oxygenation in neonates with severe neonatal

pulmonary hypertension
E It rarely induces tolerance to its effects

5.13 With regard to the epidemiology of sepsis in children, which one

of the following statements is correct?

A Adolescents have the highest incidence of sepsis

B The hospital mortality rate for children with severe sepsis is 30% C The mortality of severe sepsis in
adults is three times that in children.
D Pre-existing morbidity is rare in those presenting with sepsis E Cardiac failure is a rare cause
of death in those with severe sepsis
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Extended Matching Questions

5.14 Theme: Airway obstruction

A Croup
B Epiglottitis
C Diphtheria
D Anaphylaxis
E Mediastinal tumour
F Inhaled foreign body
G Bacterial tracheitis

H Angioneurotic oedema
I Retropharyngeal abscess
J Asthma

K Tracheobronchomalacia

For each of the case histories below, select from the above causes of airways obstruction the one that
best matches the clinical scenario. Each option may be used once, more than once, or not at all.

1. A 3-year-old child presents to the emergency department with a

sudden onset of stridor, and a pyrexia of 40 °C. He is quiet and withdrawn. The mother informs you that
her son has not been vaccinated.
2. A 15-year-old boy presents with difficulty breathing and a severe
wheeze. A chest X-ray demonstrates hyperinflation and a widened mediastinum.
3. A 2-month-old girl with tetralogy of Fallot is noted to have a
prolonged expiratory phase and is breathless. She has marked recession when she
starts to cry. Her recession improves after nasal continuous positive airways pressure is
commenced.
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thyroid hormones 6.9, 6.12, 6.15 tick-borne infections 13.15,

21.3 toxic shock syndrome 8.3 Toxocara canis 18.10
tracheobronchomalacia 5.14 transient neonatal pustulosis 4.15
translocation carriers 9.15 traumatic injuries 5.8, 5.11
travellers, returning with disease
13.15

tricuspid atresia 1.7

tricuspid regurgitation 1.11, 1.15 trisomy 13 15.15
trisomy 18 15.15
trochlear nerve palsy 18.14
tropical diseases 13.15
tuberculosis 8.9, 20.13
tumour necrosis factor 12.8

Turkish ethnicity 9.11

Turner syndrome 6.11, 9.14, 9.15 typhoid 13.15

ulcerative colitis 8.15, 21.8 umbilical hernias 19.6

urate 14.15

urinary incontinence 2.7 utilitarianism 7.8 uveitis

18.1

vaccination 11.11, 12.14 valproate 17.14

INDEX
 varicella (chickenpox) 12.12, 13.1 veno-occlusive disease
11.10 venous hum 1.12

ventricular fibrillation 5.15 ventricular septal defects 1.1, 1.6 very-long-chain fatty acids (VLCFAs)
14.10, 14.15 vesicoureteric reflux 16.4

vesiculopustular lesions 4.15

see also blisters vigabatrin 17.14, 18.15
vision

acuity testing 18.13

eye conditions affecting 15.13, 18.3,
18.5, 18.6, 18.10
visual field defects 18.7, 18.15

visually directed behaviours 2.5

vitamins 8.2, 14.2
vomiting 8.14
von Willebrand disease (WWD) 10.3

white blood cells 12.2, 12.15

cytopenia 10.10, 12.9, 21.14 leukaemia 6.10, 10.8, 21.14 Williams syndrome 9.14 Wilms' tumour 10.15 Wilson's
disease 11.3, 11.15 Wiskott-Aldrich syndrome 10.14

Zellweger syndrome 14.15 zoonoses 13.5, 18.10, 21.3

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