Management of Prader-Willi Syndrome

Management of Prader-Willi Syndrome brings together the contributions of professionals with considerable expertise in diagnosis and management of PWS. Clinical, social, family, and community issues are explored and management strategies identified. The te

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La Monstrua vestida

La Monstrua desnuda

These paintings by the Spanish baroque court painter Juan Carreño de Miranda (1616-1685) depict Doña Eugenia Martinez Vallejo at approximately 6 years of age (c. 1680), after she was brought to the court of Charles II of Spain and given the moniker “La Monstrua.” It is said that upon viewing these paintings in the Museo del Prado, Madrid, Dr. Andrea Prader immediately recognized features consistent with Prader-Willi syndrome. Although we may never know if this child did in fact have PWS, the paintings and contemporaneous 17th century descriptions of her condition strongly support this supposition. Indeed, these paintings are often regarded as the earliest illustrations of PWS. La Monstrua has continued to be an intriguing figure, as evidenced by an outdoor sculpture in Avilés created in 1997 by the Spanish artist Amado González Hevia “Favila” (b. 1954; www.amadofavila.com) based on these baroque portraits.

Management of Prader-Willi Syndrome Third Edition Merlin G. Butler, MD, PhD Chief, Section of Medical Genetics and Molecular Medicine, William R. Brown/Missouri Chair of Medical Genetics and Molecular Medicine; Professor of Pediatrics, University of Missouri-Kansas City School of Medicine, Children’s Mercy Hospitals and Clinics, Kansas City, Missouri

Phillip D.K. Lee, MD Chief Scientific Officer, Immunodiagnostic Systems Ltd., Boldon, Tyne and Wear, United Kingdom

Barbara Y. Whitman, PhD Professor of Pediatrics, Saint Louis University School of Medicine, Cardinal Glennon Children’s Hospital, St. Louis, Missouri

Editors

With 125 Illustrations, 11 in Full Color

Merlin G. Butler, MD, PhD Chief, Section of Medical Genetics and Molecular Medicine William R. Brown/Missouri Chair of Medical Genetics and Molecular Medicine and Professor of Pediatrics University of Missouri-Kansas City School of Medicine Children’s Mercy Hospitals and Clinics Kansas City, MO 64108 USA

Phillip D.K. Lee, MD Chief Scientific Officer Immunodiagnostic Systems Ltd. Boldon, Tyne and Wear NE 35 9PD UK Barbara Y. Whitman, PhD Professor of Pediatrics Saint Louis University School of Medicine Cardinal Glennon Children’s Hospital St. Louis, MO 63104 USA

Supported by an unrestricted grant from Pharmacia Corporation, a division of Pfizer, Inc. Library of Congress Control Number: 2005930806 ISBN 10: 0-387-25397-1 ISBN 13: 978-0387-25397-8 Printed on acid-free paper. © 2006, 1995 by the Prader-Willi Syndrome Association (USA) All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, Inc., 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they