Prader-willi Syndrome As A Model For Obesity: International Symposium, Zurich, October 18-19, 2002

Prader-Willi Syndrome as a Model for Obesity This symposium was held in memoriam for Andrea Prader. Drawing of Andrea Prader by his friend and colleague, Emile Gautier, formerly Chairman of the Department of Paediatrics, University of Lausanne, Switzerland. International Symposium, Zurich, October 18–19, 2002 Prader-Willi Syndrome as a Model for Obesity Editors Urs Eiholzer Zurich Dagmar l'Allemand Zurich William B. Zipf Columbus, Ohio 38 figures and 25 tables, 2003 Basel · Freiburg · Paris · London · New York · Bangalore · Bangkok · Singapore · Tokyo · Sydney Urs Eiholzer William B. Zipf Foundation Growth Puberty Adolescence Zurich, Switzerland Clinical Professor Pediatrics Department of Pediatrics The Ohio State University Columbus, Ohio, USA Dagmar l'Allemand Foundation Growth Puberty Adolescence Zurich, Switzerland This book was sponsored by Pharmacia Endocrine Care with an unrestricted educational grant and by the Foundation Growth Puberty Adolescence, Zurich. Library of Congress Cataloging-in-Publication Data Prader-Willi syndrome as a model for obesity : international symposium, Zurich, October 18–19, 2002 / editors, Urs Eiholzer, Dagmar l’Allemand, William B. Zipf. p. ; cm. Includes bibliographical references and indexes. ISBN 3–8055–7574–2 (hard cover : alk. paper) 1. Prader-Willi Syndrome–Congresses. 2. Somatotropin–Therapeutic use–Congresses. 3. Obesity in children–Congresses. I. Eiholzer, Urs, 1951– II. l’ Allemand, Dagmar. III. Zipf, William B. (William Byron) [DNLM: 1. Prader-Willi Syndrome–Congresses. 2. Growth Hormone–therapeutic use– Child–Congresses. 3. Homeostasis–physiology–Congresses. 4. Hormone Replacement Therapy–Child–Congresses. 5. Obesity–Child–Congresses. QS 675 P8959 2003] RJ520.P7P734 2003 618.92⬘85884–dc21 2003047425 Bibliographic Indices. This publication is listed in bibliographic services. Drug Dosage. The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. © Copyright 2003 by S. Karger AG, P.O. Box, CH–4009 Basel (Switzerland) www.karger.com Printed in Switzerland on acid-free paper by Reinhardt Druck, Basel ISBN 3–8055–7574–2 Contents Introduction 1 Prader-Willi Syndrome as a Model for Obesity Eiholzer, U. (Zurich) Central Nervous System and Body Weight Homeostasis 7 Obesity due to Mutations in the Anorexigenic Melanocortin Pathway: A Paradigm for Obesity in Prader-Willi Syndrome? Krude, H.; Grüters, A. (Berlin) 15 Signals that Control Central Appetite Regulation Woods, S.C.; Clegg, D.J. (Cincinnati, Ohio) 31 Hypothalamic Neuropeptides and Regulation of Fat Mass in Prader-Willi Syndrome Goldstone, A.P. (London/Amsterdam); Unmehopa, U.A. (Amsterdam); Thomas, E.L.; Brynes, A.E.; Bell, J.D.; Frost, G.; Ghatei, M.A. (London); Holland, A. (Cambridge); Bloom, S.R. (London); Swaab, D.F. (Amsterdam) 44 Discussion Energy Balance in Prader-Willi Syndrome Compared to Simple Obesity 49 Assessment of Body Composition in Children with Prader-Wi