Table Of Content

Clinical Hepatology Henryk Dancygier Clinical Hepatology Principles and Practice of Hepatobiliary Diseases Volume 2 With Contributions by H.-D. Allescher · U. Beuers · H. Blum · R. S. Brown, Jr · E. Cay · H. Dancygier S. Dasarathy · C. F. Dietrich · M. Doss · A. Dragan · S. M. Erturk · S. A. Fink L. S. Friedman · G. Gerken · D. Häussinger · P. Hilgard · M. A. Kern · A. Koch J. H. Lefkowitch · U. Leuschner · T. Longerich · A. J. McCullough · U. Merle J. Mössner · S. Mueller · A. Niedenthal · C. Niederau · B. Riemann · J. N. Rogart P. Ros · C. Sarrazin · P. Schirmacher · O. Schober · H. K. Seitz · S. H. Sigal F. Stenschke · U. Stölzel · C. P. Strassburg · W. Stremmel · S. Susser · C. Trautwein M. Tröltzsch · I. S. Weisberg · M. Wiedman · C. Wittekind · H. Witzigmann P. S. Yachimski · S. Zeuzem Foreword by Scott L. Friedman Henryk Dancygier, MD Professor of Medicine Chair, Department of Medicine II Klinikum Offenbach, Goethe University Frankfurt/Main Starkenburgring 66 63069 Offenbach, Germany email: [email protected] Adjunct Professor of Medicine Department of Medicine, Division of Liver Diseases Mount Sinai School of Medicine New York, NY, USA email: [email protected] ISBN: 978-3-642-04509-7 e-ISBN: 978-3-642-04519-6 DOI: 10.1007/978-3-642-04519-6 Springer Heidelberg Dordrecht London New York Library of Congress Control Number: 2009929701 © Springer-Verlag Berlin Heidelberg 2010 This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifi cally the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfi lm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current version, and permission for use must always be obtained from Springer. Violations are liable to prosecution under the German Copyright Law. The use of general descriptive names, registered names, trademarks, etc. in this publication does not imply, even in the absence of a specifi c statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product liability: The publishers cannot guarantee the accuracy of any information about dosage and application contained in this book. In every individual case the user must check such information by consulting the relevant literature. Cover design: eStudioCalamar, Figueres/Berlin Printed on acid-free paper Springer is part & Springer Science + Business Media (www.springer.com) Dedication Dedicated to Dr. Herbert Falk (1924–2008). A humanist of great generosity, supporter of scientifi c advancement and the always visible, but never dominant magnanimous patron of global medical education. v Foreword Modern hepatology would be unrecognizable to those clinicians and pathologists, who founded the fi eld only a generation ago. From a discipline that was largely obser- vational – with few diagnostic tests and even fewer therapies – has emerged an excit- ing area that is among the most rapidly changing in all of medicine, and which now offers remarkable new tools and treatments. This textbook edited by Professor Dancygier beautifully captures this dynamism of hepatology, and in doing so provides a remarkably complete opus. The work is beautifully laid out exactly as a clinician would think, weaving in the science underlying clinical hepatology with precision and clarity. Standardized and gorgeous drawings, comprehensive tables, and a very consis- tent style are among its most valuable assets – quite simply, this book is fun to read! While thoroughly modern, this work still proudly draws upon the roots of our specialty. A strong emphasis on pathology, patterns of injury, clinical presentations of disease, and approaches to clinical problems harken back to hepatology’s earliest treatises. The book is particularly reminiscent of early editions of Dame Sheila Sherlock’s classic single-author textbook, the fi fth edition of which I read cover-to- cover while spending 3 months at the Royal Free Hospital as a medical student in 1977; a signed copy sits proudly on my shelf to this day, and I suspect that many will come to value Professor Dancygier’s book for many of its similar virtues. The highly personal stamp of Professor Dancygier infuses this book with cohesion, and conveys the wonders of clinical hepatology. Underscoring another enduring feature of our specialty is the book’s transatlantic fl avor, with authors from throughout Germany and the USA. This connection between our two countries is also personal – Professor Dancygier’s invitation for me to speak at a conference in Munich in 1989 was my fi rst international meeting, and I am proud that our professional association and friendship continue to this day as a result of that fi rst meeting 20 years ago. I am delighted to be associated with Clinical Hepatology. Thus, it is a great personal privilege to introduce this unique and valuable textbook, which is sure to appeal to practitioners of today and ignite a spark of enthusiasm among the hepatologists of tomorrow. Scott L. Friedman, M.D. President, American Association for the Study of Liver Diseases Fishberg Professor and Chief, Division of Liver Diseases The Mount Sinai School of Medicine vii Preface Clinical hepatology is thriving. Hepatology has evolved from a pure diagnostic art to a science in which new treatment options are emerging at a quick pace. At this excit- ing time, based on the gratifying success of the German Edition of 2003, Springer Publishers have asked me to prepare a US-American/International edition. Gravely miscalculating the amount of labor, I gladly accepted this challenge. The present book is not merely an updated version of the German edition but a completely new work with new US-American authors, a transatlantic endeavor. In common with its German predecessor, however, it not only aims to provide know- ledge in hepatology but also to promote an understanding of liver diseases, and to create joy in dealing with clinical hepatological problems. It is intended for every- body caring for adult patients with hepatobiliary problems, particularly gastroente- rologists/hepatologists, internists and clinical pathologists. My aim was not to provide an encyclopedic behemoth. Instead, I intended to create a comprehensive, up-to-date (references until early 2009 are included), practical and readable book that outlines the current standards of diagnosis and treatment in hepatology and its associated biliary disorders. By elaborating on concepts in hepatology, disease mechanisms, common clinical problems and rare diseases alike it tries to serve the needs not only of the novice in hepatology, but also of the experienced practicing clinician. Ultimately, the success of the book will be determined by its ability to provide answers to clinically relevant questions and to guide clinical decisions. The competent clinical hepatologist, like hardly any other clinician, has to inte- grate histopathological, biochemical, immunological, instrumental and clinical skills. The organization of the text follows this principle. It is divided into 3 main parts with 30 sections covering Basic Principles, Clinical Methods, and Hepatobiliary Diseases. Starting from basic concepts the fi eld of clinical hepatology gradually unfolds. Unlike other Hepatology Textbooks I preferred not to include a “stand alone” chapter on liver pathology. Instead hepatopathology has been integrated throughout the entire text as it represents an integral part of clinical hepatology. The fi rst fi ve sections inte- grate structure and function of the liver and basically provide a general pathology of the liver. The clinical chapters are stringently organized and uniformly structured to enable rapid retrieval of the desired information. In order to enhance readability I have accepted some redundancy, especially in chapters dealing with hepatocellular transporters. I am indebted to my coauthors, all renowned experts in hepatology. Without their help the creation of a textbook of this volume would not have been possible. My ix x Preface special thanks go to a young gastroenterologist from Yale, Jason Rogart. He not only served as a proof reader for the contributions by authors whose native language is not English, but also as an author of several chapters and as an editorial assistant. My thanks go also to Ms. Annette Hinze, Meike Stoeck, and Mr. Claus-Dieter Bachem from Springer Publishers who skillfully supported the development of the Textbook. Last but not least I thank my wife Hellena for her endurance and unwavering support. After immerging into the project and resurfacing after fi nishing the last chapter she was still there. Offenbach, May 2009 Henryk Dancygier Volume 1 A. Basic Principles Part I Structure and Function of the Liver Part II Pathophysiology and Morphology of Liver Injury B. Clinical Methods Part III Evaluation of the Patient with Hepatobiliary Disease Volume 2 C. Hepatobiliary Diseases Part IV Diseases of the Liver Part V Diseases of the Gallbladder and Extrahepatic Bile Ducts Contents of Volume 1 A. Basic Principles Part I Structure and Function of the Liver . . . . . . . . . . . . . . . . . . 3 Section I Embryology, Anatomy, and Histology. . . . . . . . . . . . . . . . . . . . . . 5 1 Embryonic Development. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 Henryk Dancygier 2 Gross Anatomy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11 Henryk Dancygier 3 Microscopic Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Henryk Dancygier Section II Fundamentals of Hepatic Physiology and Biochemistry. . . . . . 53 4 Hepatic Circulation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55 Henryk Dancygier 5 Hepatocellular Transport . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61 Henryk Dancygier 6 Hepatic Metabolism. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75 Henryk Dancygier, Uta Merle, Wolfgang Stremmel, and Claus Niederau 7 Formation and Secretion of Bile and Bilirubin Metabolism . . . . . . . . 103 Ulrich Leuschner 8 Hepatic Biotransformation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 Henryk Dancygier 9 Functional Heterogeneity and Metabolic Zonation. . . . . . . . . . . . . . . . 131 Henryk Dancygier 10 Liver Cell Hydration and Cell Function . . . . . . . . . . . . . . . . . . . . . . . . 137 Dieter Häussinger 11 The Liver as an Immune Organ. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141 Henryk Dancygier xiii