Fibrocystic Diseases of the Liver
In recent years there have been huge advances in the understanding of the genetic and molecular basis of the fibrocystic diseases. This volume provides a thorough review of fibrocyctic diseases that affect the liver. It contains in-depth discussions of th
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Karen F. Murray, MD Anne M. Larson, MD
CLINICAL GASTROENTEROLOGY
Series Editor George Y. Wu University of Connecticut Health Center, Farmington, CT, USA
For further volumes: http://www.springer.com/series/7672
Fibrocystic Diseases of the Liver Edited by
KAREN F. MURRAY, MD Division of Pediatric Gastroenterology and Hepatology, Seattle Children’s, University of Washington School of Medicine, Seattle, WA
ANNE M. L ARSON, MD, FACP, AGAF University Hospital – St. Paul, Dallas, TX
Editors Karen F. Murray Division of Pediatric Gastroenterology and Hepatology Seattle Children’s University of Washington School of Medicine 4800 Sand Point Way NE. Seattle, WA 98105 USA [email protected]
Anne M. Larson Medical Director of Liver Transplant Director of Clinical Hepatology University Hospital – St. Paul 5939 Harry Hines Blvd HQ8.803 Dallas, TX 75390-9258 USA [email protected]
ISBN 978-1-60327-523-1 e-ISBN 978-1-60327-524-8 DOI 10.1007/978-1-60327-524-8 Springer New York Dordrecht Heidelberg London Library of Congress Control Number: 2010920543 C Springer Science+Business Media, LLC 2010
All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Humana Press, c/o Springer Science+Business Media, LLC, 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 are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Humana Press is part of Springer Science+Business Media (www.springer.com)
To my husband Bernie and children Michael and Katie, for their love, understanding, and ongoing encouragement and support; to my parents for their love and encouragement; and to Dr. Jay Perman for rousing my interest in pediatric gastroenterology, guidance in academic pediatrics, and for his friendship. Karen F. Murray, MD
Preface It has been recognized for centuries that cystic pathology of the liver and kidneys, and occasionally other organs, can occur in the same individual or within families and may have grave consequences. We have learned over time that the inheritance pattern seen in many of these conditions honors Mendelian genetics and that some conditions have non-cystic syndromic associations. Our ability to catego
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