Clinical Paediatric Dietetics Second Edition Edited by
Vanessa Shaw and
Margaret Lawson
Clinical Paediatric Dietetics
Clinical Paediatric Dietetics Second Edition Edited by
Vanessa Shaw and
Margaret Lawson
© 1994, 2001 by Blackwell Science Ltd Editorial Offices: Osney Mead, Oxford OX2 0EL 25 John Street, London WC1N 2BS 23 Ainslie Place, Edinburgh EH3 6AJ 350 Main Street, Malden MA 02148 5018, USA 54 University Street, Carlton Victoria 3053, Australia 10, rue Casimir Delavigne 75006 Paris, France Other Editorial Offices: Blackwell Wissenschafts-Verlag GmbH Kurfürstendamm 57 10707 Berlin, Germany Blackwell Science KK MG Kodenmacho Building 7–10 Kodenmacho Nihombashi Chuo-ku, Tokyo 104, Japan Iowa State University Press A Blackwell Science Company 2121 S. State Avenue Ames, Iowa 50014-8300, USA The right of the Authors to be identified as the Authors of this Work has been asserted in accordance with the Copyright, Designs and Patents Act 1988. All rights reserved. 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, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher. First edition published 1994 Reprinted 1995 Second edition published 2001 Set in 10 on 11 pt Ehrhardt MT by Best-set Typesetters Ltd., Hong Kong Printed and bound in Great Britain by Antony Rowe Ltd, Chippenham The Blackwell Science logo is a trade mark of Blackwell Science Ltd, registered at the United Kingdom Trade Marks Registry
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[email protected]) Australia Blackwell Science Pty Ltd 54 University Street Carlton, Victoria 3053 (Orders: Tel: 03 9347 0300 Fax: 03 9347 5001) A catalogue record for this title is available from the British Library ISBN 0-632-05241-4 Library of Congress Cataloging-in-Publication Data Clinical paediatric dietetics / edited by Vanessa Shaw and Margaret Lawson. – 2nd ed. p.; cm. Includes bibliographical references and index. ISBN 0-632-05241-4 (hb) 1. Diet therapy for children. I. Shaw, Vanessa. II. Lawson, Margaret, MSc. [DNLM: 1. Diet Therapy – Child. WS 366 C6405 2001] RJ53.D53 C58 2001 615.8⬘54⬘083 – dc21 00-052960 For further information on Blackwell Science, visit our website: www.blackwell-science.com
Contents
Contributors Foreword Preface Acknowledgements SECTION 1 Introduction 1 Principles of Paediatric Dietetics Vanessa Shaw and Margaret Lawson 2 Provision of Nutrition in a Hospital Setting Ruth Watling SECTION 2 Enteral and Parenteral Nutrition 3 Enteral Feeding Tracey Johnson 4 Parenteral Nutrition Janice Glynn SECTION 3 Preterm and Low Birthweight Nutrition 5 Preterm Infants Caroline King SECTION 4 Diseases of Organ Systems 6 The Gastrointestinal Tract Sarah Macdonald 7 Anatomical Abnormalities of the Gastrointestinal Tract Vanessa Shaw 8 The Liver and Pancreas Stephanie France (Jackson) 9 Diabetes Mellitus Alison Johnston 10 Cystic Fibrosis Anita MacDonald
vii ix xi xii 1 3 19
29 31 43
53 55
67 69 97 110 126 137
11 The Kidney Janet Coleman 12 The Cardiothoracic System Marion Noble 13 The Immune System – Food Allergy and Intolerance Christine Carter – Immunodeficiency Syndromes Dona Hileti-Telfer – HIV and AIDS Jayne Butler 14 Ketogenic Diet for Epilepsy Margaret Lawson SECTION 5 Inborn Errors of Metabolism 15 Disorders of Amino Acid Metabolism, Organic Acidaemias and Urea Cycle Defects Marjorie Dixon – Phenylketonuria Anita MacDonald – Homocystinuria Fiona White 16 Disorders of Carbohydrate Metabolism Marjorie Dixon 17 Disorders of Fatty Acid Oxidation Marjorie Dixon
158 182 193 193 214 218 222
233 235 235 267 295 316
SECTION 6 Lipids 18 Lipid Disorders Patricia Rutherford
327 329
SECTION 7 Peroxisomal Disorders 19 Refsum’s Disease June Brown
337 339
vi 20 X-linked Adrenoleukodystrophy Anita MacDonald SECTION 8 Childhood Cancers 21 Nutritional Support: Leukaemias, Lymphomas and Solid Tumours Evelyn Ward SECTION 9 Eating Disorders and Obesity 22 Eating Disorders Dasha Nicholls 23 Obesity Mary Deane SECTION 10 Other Conditions Requiring Nutritional Support and Advice 24 Epidermylosis Bullosa
Contents 345
349 351
361 363 371
381 383
Lesley Haynes 25 Burns Helen McCarthy and Dearbhla Hunt 26 Nutrition for Children with Feeding Difficulties Karen Jeffereys 27 Failure to Thrive Zofia Smith 28 Children from Ethnic Minorities and those following Cultural Diets Sue Wolfe
396 403 423 431
APPENDICES Appendix I Manufacturers of Dietetic Products Appendix II Dietetic Products
447 449 451
Index
455
Contributors
June Brown Former Senior Dietitian Chelsea and Westminster Hospital, London SW10 9NH
Dona Hileti-Telfer Senior Dietitian Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH
Jayne Butler Senior Paediatric Dietitian St Mary’s Hospital, Praed Street, London W2 1NY
Dearbhla Hunt Senior Dietitian The Children’s Hospital, Temple Street, Dublin, Eire
Christine Carter Specialist Dietitian Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH
Stephanie France (née Jackson) Senior Paediatric Dietitian Kings College Hospital, Denmark Hill, London SE5 9RS
Janet Coleman Chief Paediatric Renal Dietitian City Hospital, Hucknall Road, Nottingham NG5 1PB
Karen Jeffereys Chief Dietitian Services for People with Learning Disabilities, P O Box 107, Southsea, Hants PO4 8NG
Mary Deane Senior Dietitian Royal Hospital for Sick Children, Sciennes Road, Edinburgh EH9 1LF
Tracey Johnson Senior Dietitian The Birmingham Children’s Hospital NHS Trust, Steelhouse Lane, Birmingham B4 6NH
Marjorie Dixon Specialist Dietitian Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH
Alison Johnston Senior Dietitian Royal Hospital for Sick Children, Yorkhill, Glasgow G3 8SJ
Janice Glynn Senior Dietitian Royal Liverpool Children’s NHS Trust, Eaton Road, Liverpool L2 2AP
Caroline King Chief Paediatric Dietitian Queen Charlotte’s Hospital, Goldhawk Road, London W6 0XG
Lesley Haynes Specialist Dietitian Institute of Child Health, 30 Guilford Street, London WC1N 1EH
Margaret Lawson Nestle Senior Research Fellow Institute of Child Health, 30 Guilford Street, London WC1N 1EH
viii
Contributors
Helen McCarthy Senior Dietitian Manchester Children’s Hospitals, Charlestown Road, Blackley, Manchester M9 7AA
Vanessa Shaw Chief Dietitian Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH
Anita MacDonald Head of Dietetic Services The Birmingham Children’s Hospital NHS Trust, Steelhouse Lane, Birmingham B4 6NH
Zofia Smith Senior Dietitian St Mary’s Hospital, Greenhill Road, Leeds LS12 3QU
Sarah Macdonald Specialist Dietitian Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH
Evelyn Ward Senior Paediatric Dietitian St James University Hospital NHS Trust, Beckett Street, Leeds LS9 7TF
Dasha Nicholls Clinical Lecturer in Neurosciences and Mental Health Institute of Child Health, 30 Guilford Street, London WC1N 1EH
Ruth Watling Chief Dietitian Royal Liverpool Children’s NHS Trust, Eaton Road, Liverpool L2 2AP
Marion Noble Former Specialist Dietitian Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH
Fiona White Chief Dietitian Manchester Children’s Hospitals, Hospital Road, Manchester M27 4HA
Patricia Rutherford Chief Dietitian Royal Liverpool Children’s NHS Trust, Eaton Road, Liverpool L2 2AP
Sue Wolfe Chief Paediatric Dietitian St James University Hospital NHS Trust, Beckett Street, Leeds LS9 7TF
Foreword
The first edition of Clinical Paediatric Dietetics was published in 1994, contributions being made by many senior dietitians. It is now a great pleasure to write a Foreword to this second edition to which no fewer than 28 experienced colleagues have contributed. Over the years the role of dietetics in the treatment of sick infants and children has become both increasingly important and in many instances far more complex. The Paediatric Group of the British Dietetic Association is to be congratulated for the way it has moved dietetics forwards, with great benefit to their young patients. As before, the chapters cover a wide variety of subjects ranging from the principles of paediatric dietetics to the treatment of diabetes mellitus, allergic disorders, inherited metabolic disorders, burns and eating disorders to name but a few. As previously, the needs of sick children from ethnic minorities have been included. The practical aspects of dietary management are given a high profile, excellent tables and clear diagrams provide essential information and each chapter has a suitable list of references. All the chapters are full of commonsense and the volume
provides lists of commercial products and the addresses of manufacturers. Once again, Vanessa Shaw and Margaret Lawson have undertaken a huge task; they have contributed chapters themselves and undertaken the editing. Obtaining high standard material from so many colleagues is certainly not easy, and I congratulate Vanessa and Margaret most warmly. This multiauthor book will, I am sure, go through numerous editions as the years pass and each edition will help so many sick infants and children. Looking back to 1965 when the first edition of Diets for Sick Children appeared, one is conscious of the huge developments which have taken place in dietetics and the ever increasing importance of the dietitians. Dame Barbara Clayton DBE, MD, PhD, HonDSc, FRCP, FRCPE, HonFRCPI, HonFRCPCH, FMedSci, HonFIBiol, FRCPath Honorary Research Professor in Metabolism, University of Southampton Honorary President, British Dietetic Association
Preface
The aim of this manual is to provide a very practical approach to the nutritional management of a wide range of paediatric nutritional disorders that may benefit from nutritional support or be ameliorated or resolved by dietary manipulation. The text will be of particular relevance to professional dietitians, dietetic students and their tutors, paediatricians, paediatric nurses and members of the community health team involved with children requiring therapeutic diets. The growing importance of nutritional support in many paediatric conditions is recognised and reflected in new text for this edition. The authors are largely drawn from experienced paediatric dietitians around the United Kingdom, with additional contributions from other specialist dietitians and a psychiatrist. The text does not attempt to discuss normal nutrition in healthy children (though references for this topic are addressed), but concentrates on the nutritional requirements of sick infants and children in a clinical setting. Normal dietary constituents are used alongside special dietetic products to provide a prescription that will control progression and symptoms of disease whilst maintaining the growth potential of the child.
There has been an expansion of the range of disorders and treatments described: additional information has been included on nutritional assessment, inborn errors of metabolism, food allergy and intolerance, immunodeficiency syndromes and gastroenterology. Arranged under headings of disorders of organ systems rather than type of diet, and with much information presented in tabular form, the manual is easy to use. Dietary restrictions due either to customs, religious beliefs or environmental conditions which may affect the nutritional adequacy of the diet of the growing child are also discussed. Appendices list the many and varied special products described in the text, together with details of their manufacturers. The appendices are not exhaustive, but include the products most commonly used in the UK. The information has been updated for this edition and the most recent data has been used in the preparation of the manual, but no guarantee can be given of the validity or availability at the time of going to press. Vanessa Shaw Margaret Lawson January 2001
Acknowledgements
We would like to thank a number of the contributors to the first edition, who were unable to contribute to this second edition, but whose work has formed the basis for the following chapters: Chapter 2, Provision of Nutrition in a Hospital Setting: Christine Clothier, Former Chief Dietitian, Royal Liverpool Children’s NHS Trust
Chapter 14, Ketogenic Diet for Epilepsy: Jane Eaton, Former Chief Community Dietitian, Dorset Health Care NHS Trust
Chapter 3, Enteral Feeding: Debra Woodward, Former Senior Dietitian, The Children’s Hospital, Birmingham
Chapter 18, Lipid Disorders: Anne Maclean, Former Senior Paediatric Dietitian, St George’s Hospital, London
Chapter 4, Parenteral Nutrition: Alison Macleod, Former Senior Dietitian, Royal Liverpool Children’s Hospital NHS Trust
Chapter 22, Eating Disorders: Bernadette Wren, Former Principal Clinical Psychologist, and Bryan Lask, Former Consultant Psychiatrist, Great Ormond Street Hospital for Children NHS Trust
Chapter 6, The Gastrointestinal Tract: Sheena Laing, Chief Dietitian, Royal Hospital for Sick Children, Edinburgh Chapter 8, The Liver and Pancreas: Jane Ely, Former Senior Paediatric Dietitian, King’s College Hospital, London
Chapter 25, Burns: Judith Martin, Former Chief Dietitian, Whittington Hospital, London.
SECTION 1
Introduction
CHAPTER 1
Principles of Paediatric Dietetics
Assessment and monitoring of nutritional status should be included in any dietary regimen, audit procedure or research project where a modified diet plays a role. There are a number of methods of assessing specific aspects of nutritional status, but no one measurement will give an overall picture of status for all nutrients. There are a number of assessment techniques, some of which should be used routinely in all centres whilst others are still in a developmental stage or suitable only for research. Figure 1.1 outlines the techniques that can be used for nutritional assessment.
diet history taken from the usual carers (or from the child if appropriate), a quantitative food diary over a number of days, a weighed food intake over a number of days or a food frequency questionnaire. The assessment of milk intake for breast-fed infants is difficult and only very general estimations can be made. Infants can be test-weighed before and after a breast-feed and the amount of milk consumed can be calculated. This requires the use of very accurate scales (±1–2 g) and should be done for all feeds over a 24 hour period as the volume consumed varies throughout the day. Test-weighing should be avoided if at all possible as it is disturbing for the infant, engenders anxiety in the mother and is likely to compromise breast-feeding. Studies have shown that the volume of breast milk consumed is approximately 770 ml at 5 weeks and 870 ml at 11 weeks [1]. In general an intake of 850 ml is assumed for all infants who are fully breast-fed and over the age of 6 weeks. Estimation of food intake is particularly difficult in infants who are taking solids, as it is not possible to assess accurately the amount of food wasted through spitting, drooling etc. Conversion of food intake into nutrient values for young children may involve the use of manufacturers’ data if the child is taking proprietary infant foods and/or infant formula. The composition of breast milk varies and food table values may be inaccurate by up to 20% because of individual variation. Assessment of the adequacy of an individual calculated nutrient intake for sick and for healthy infants and children is discussed in the section on dietary reference values later in this chapter.
Nutritional intake
Anthropometry
For children over the age of two years food intake is assessed in the same way as for adults: using a recall
Measurement of weight and height or length is critical as the basis for calculating dietary requirements as
This manual provides a practical approach to the dietary management of a range of paediatric disorders. The therapies describe dietetic manipulations and the nutritional requirements of the infant and child in a clinical setting. It does not attempt to address the nutrition of the healthy child in any detail (see Further Reading at end of this chapter), but illustrates how normal dietary constituents are used alongside special dietetic products to allow the continued growth of the child whilst controlling the progression and symptoms of disease. Dietary restrictions, due either to custom, religious beliefs or environmental conditions are also discussed. DIETARY PRINCIPLES The following principles are relevant to the treatment of all infants and children and provide the basis for many of the therapies described later in the text. Assessment of nutritional status
4
Clinical Paediatric Dietetics First line or basic assessment
Nutrient intake
Clinical assessment
Anthropometry
䉲 Second line tests, as indicated by results from basic assessment
Biochemistry
Haematology 䉲
Research tools or unsuitable for paediatrics
Body composition studies • Immunology • Functional tests • Isotope studies Fig. 1.1 Nutritional assessement methods
well as monitoring the effects of dietary intervention. Other anthropometric measurements are summarised in Table 1.1. Measurement of weight is an easy and routine procedure using an electronic digital scale or a beam balance. Ideally infants should be weighed nude and children wearing just a clean nappy or pants, but if this is not possible it is important to record whether the infant is weighed wearing a clean nappy, and the amount and type of clothing worn by older children. For weighing infants up to 10 kg, scales should be accurate to 10 g; for children up to 20 kg, accuracy should be +/- 20 g and over 20 kg it should be +/- 50–200 g. A higher degree of accuracy is required for the assessment of sick children than for routine measurements in the community. Frequent weight monitoring is important for the sick infant or child and hospitalised infants should be weighed each day or every second day; children over the age of two in hospital should be weighed at least weekly. Healthy infants should be weighed every 2 weeks until the age of 6 months, every month between the ages of 6 months and 24 months and every 2–3 months after the age of 2 years. Height or length measurement requires a stadiometer or length-board. Details of suitable equipment, which may be fixed or portable, are available from the Child Growth Foundation (see end of chapter). Measurement of length using a tape measure is too inaccurate to be of use for longitudinal monitoring of growth, although an approximate length may be useful as a single measure (e.g. for cal-
culating body mass index). Under the age of about 2 years supine length is measured; standing height is usually measured over this age or whenever the child can stand straight and unsupported. When the method of measurement changes there is likely to be a difference, and measurements should be made by both methods on one occasion when switching from supine length to standing height. Measurement of length is difficult and requires careful positioning of the infant, ensuring that the back, legs and head are straight, the heels are against the footboard, the shoulders are touching the baseboard and the crown of the head is touching the headboard. Two people are required to measure length – one to hold the child in position and one to record the measurement. Positioning of the child is also important when measuring standing height and care should be taken to ensure that the back and legs are straight, the heels, buttocks, shoulder blades and back of head are touching the measurement board and that the child is looking straight ahead. A body mass index (BMI) measurement can be calculated from the weight and height measurements (BMI = weight(kg)/height(m)2) and provides an indication of fatness or thinness. In adults body fatness is largely unrelated to age and high BMI measurements are related to health risks. In children the amount and distribution of body fat is dependent on age, and does not appear to be related to health. Age-related centile charts for BMI have been developed [2] and indicate how heavy the child is relevant to his height and age.
Principles of Paediatric Dietetics Table 1.1 Anthropometric measurements Comments
Measurement
Easy to do Useful 1 person Best indicator of overall nutritional well-being
Height
Easy to do Useful
