Dedication To Gaggy who, in his own way, inspired .
Commissioning Editor: Ellen Green, Pauline Graham
Development Editor: Clive Hewat Project Manager: Christine Johnston Design Direction: Erik Bigland Illustration Manager: Gillian Richards Illustrator: Barking Dog Art
ELSEVIER CHURCHILL LMNGSTONE
© 2007, Elsevier Limited. All rights reserved. The right of Damian Roland to be identified as editor of this work has been asserted by him in accordance with the Copyright, Designs and Patents Act 1988. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, without the prior permission of the Publishers. Permissions may be sought directly from Elsevier's Health Sciences Rights Department, 1600 John F. Kennedy Boulevard, Suite 1800, Philadelphia, PA 19103-2899, USA: phone: (+1) 215 239 3804; fax: (+1) 215 239 3805; or, e-mail:
[email protected]. You may also complete your request on-line via the Elsevier homepage (http://www.elsevier.com), by selecting 'Support and contact' and then 'Copyright and Permission'. ISBN 978 0 443 10335 3 British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library Library of Congress Cataloging in Publication Data A catalog record for this book is available from the Library of Congress N ote Knowledge and best practice in this field are constantly changing. As new research and experience broaden our knowledge, changes in practice, treatment and drug therapy may become necessary or appropriate. Readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications. It is the responsibility of the practitioner, relying on their own experience and knowledge of the patient, to make diagnoses, to determine d osages and the best treatment for each individual patient, and to take all appropriate safety precautions. To the fullest extent of the law, neither the Publisher nor the Editor assumes any liability for any injury and/ or damage to persons or property arising out of or related to any use of the material contained in this book. The Publisher
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Contents Foreword vii Acknowledgements
IX
Introduction 1 How to get the most out of this book
2
Circuit A 17 Stations 1- 9 Questions 18 Stat ions 1- 9 Answers 27
Circuit B 45 Stations 1- 9 Questions 46 Stat ions 1- 9 Answers 55 Circuit C
75
Stati ons 1- 9 Questions 76 Stat ions 1- 9 Answers 85 Circu it D 101 Statio ns 1- 9 Questions 102 Stat ions 1-9 Answe rs 111 Circ:uit IE
123
Stations 1- 9 Questions 124 Stations 1-9 Answe rs 133 Circ:uit F 149 Stations 1-9 Questi o ns 150 Stat ions 1- 9 Answe rs 159 Circ:uit G 173 Stations 1- 9 Quest io ns 174 Stat ions 1- 9 Answers 184 Cin:uit H
199 Stations ·J- 9 Questions
Stations 1- 9 Answe rs
200 210
Appendix: Ch ild Deve lopment Sta ges Index 249
225
Foreword
Damian Roland and his colleagues have written a truly excellent book. As someone :who has tried to write books to guide previous generations of trainees through MRCPCH exams, I know that what the reader wants is a book written by someone who has sat the exam themselves, passed the exam and can provide real insights into the conduct of the exam. They want a book written in an easily readable style and ideally to show sympathy and humour as well as provide information. This book ticks all those boxes. The previous version of the MRCPCH exam, like many of the professional exams run by the Royal Colleges, gave rise to a fund of stories, no doubt many apocryphal, relating to both the exam cases and the examiners. The clinical exam was often viewed as something of a lottery, almost entirely dependent on which cases the candidate saw and whether the child (or indeed the parent) was in a good mood or not. There were allegations that examiners asked questions only on their favourite topics and that the examiners themselves could not elicit the physical signs that they were asking the candidate to elicit. Some examiners were thought to be particularly dyspeptic after a parsimonious lunch provided by the cash-strapped local NHS hospital running the exam. They longed for the halcyon days of examiners' dinners and fine wines! The new format of the MRCPCH examination has been developed to try and address many of these concerns. Fundamentally, a postgraduate professional exam should examine trainees in the things they need to know. Therefore, the keystones remain history-taking, examination of physical signs, clinical skills and, very importantly, communication, both with the child and their family. The circuit exam sets out to do this but brings the benefits of being robust, reproducible and, as far as possible, equitable to all the candidates attending on any one day. As someone who has examined both the old format and the new format, I believe that the new format is fairer to candidates if rather more boring for the examiners! I would strongly recommend that you read, learn and inwardly digest the material within this book. It is written from the heart and it is written with your interests in mind. I am sure it will assist you in your aspiration to be a fully trained and competent paediatrician because, while the advice is geared to helping you pass the lVIRCPCH exam, the advice is also about good practice in taking histories, examining children and communicating. Professor Terence Stephenson Nottingham, 2007
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DIAGNOSIS: WILLIAMS' SYNDROME
This question is another example of the need to be versatile. You may be askPrl to perform small, separate tasks. ln this y_uestion it is important to be able to interact wPll with a child and think on your feet. Don't be thrown by simple questions such as 'Look at this_ What do you think?' - a difficult skill to revise for but may be practised by asking friends to pretend to be syndromes and feed you snippets of their features until you recognise them. You may not be -able to spot the particular feature in the exam but you will start to learn certain patterns of abnormality. We learn quite quickly from Nicola that she has some intellectual impairment and soft dysmorphic features, so any number of general questions may be appropriate. Does Nicola attend a mainstream school? Does Nicola have a statement of special educational needs/special educational needs coordinator? Is Nicola under the care of a community paediatrician? Did Nicola spend time in SCBU /NICU? Is she growing well? We then see that Nicola has evidence of previous cardiac surgery with no residual murmurs. It is impossible to say what surgery she has had, although the absence of thoracotomy scars indicates that a shunting procedure was unnecessary. So ... dysmorphic features; intellectual impairment; cardiac problems. There are a few syndromes that may have this collection of difficulties:
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On entering the room the examiner says to you, 'Please examine the back of this 6-month-old boy and then proceed to examine where else you feel necessary'. CLINICAL SCENARIO
You are presented with a young infant who is lying on his back on an examining table. You introduce yourself to his mother and gain permission to examine the child's back. Quickly, but obviously, you scan the child before turning him over. There is no obvious dysmorphism, the child is not grossly hypotonic and the hips are flexed. He is only wearing a nappy and smiles as you approach. On turning him over there is an obvious large scar in the lumbosacral region. It is in the midline and looks at least a couple of months old. Which areas of the body could you justify examining now? You elect to examine the lower limbs. On close inspection he does not appear grossly wasted. There are no fasciculations and there is some intermittent kicking movement at the knee, although it doesn't appear as coordinatPrl as you would expect in a 6-month-old child. The hips are in a permanent flexed and abducted position. There is no hip extension. The knee is observed to extend but the foot is held in a position of dorsiflexion. You are unable to obtain ankle reflexes but feel happy you have obtained an adequate knee reflex. Babinski's reflex is unequivocal but the child shows no facial response to the test. As you begin to test sensation the examiner asks you to present your findings so far.
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mother if Sarah has a problem with her sugar levels should clinch the diagnosis of GSD. The underlying problem for glycogen storage disorders is the inability to break down glycogen into a useful substrate for energy. Although there are over 10 known types it is more useful to divide them into those which affect muscle or those affecting the liver or those affecting both.
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Down's syndrome is not true macroglossia- the tongue protrudes forwanl in a relatively small mouth
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COMMENTS ON STATION 3 DIAGNOSIS: SPINA BIFIDA
This child has spina bifida and must have a lesion below L2 and possibly commencing as low down as LS. This is a difficult case because it requires you to examine and think at the same time. Candidates come unstuck in neurological cases because they are so concerned about getting the examination correct they completely ignore the findings they have obtained. You must be able to perform a neurological exam without having to worry about what to do next. It must just come naturally. In the stress of the exam you will forget to do something or realise you have no idea which muscle was weak. You should not be examined by a paediatric neurologist, although you should make sure you are taught by one.
'This child has had a surgical repair of a lumbosacral lesion, presumably a meningocele or meningomyelocele. In light of this finding I elected to examine the lower limbs to define the level of the lesion. I will also need to examine the head, looking in particular for any evidence of hydrocephalus, as up to 90% of children with spina bifida may have an Arnold-Chiari·maljormation. On examination the child was not particularly wasted and the most obvious defect was the absence of hip extension, plantar flexion and ankle reflex. As I noted some spontaneous knee extension the lesion is likely to spare L3 and perhaps L4. I need to examine the anus to complete my lower limb examination.' There is a degree of difference in textbooks about specific myotomes and reflex roots which can make revision difficult. The suggested levels worked for the author but may differ slightly from other books. The important thing is not to be too precise about the level but be very consistent with your observations and findings. There are many peripheral issues in spina bifida which must be addressed, and may be part of a communication or history-taking station.
60
REFLEXES
Triceps: C7,8 Biceps: C5,6 Knee: L3A Ankle: S1,2
Remember Jendrassik's manoeuvre. Just before the reflex is elicited the child should be asked to perform an action to produce muscle tension: 'Screw up your face', 'Pull your locked hands away from each other', 'Squeeze your fists', etc. (see Fig. 2).
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