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SHERLOCK’S DISEASES OF THE LIVER AND BILIARY SYSTEM Companion Website This book has a companion website www.wiley.com/go/sherlock/liver with: • All 700 fi gures and captions in the book as Powerpoints for downloading Sherlock’s Diseases of the Liver and Biliary System EDITED BY JAMES S. DOOLEY Centre for Hepatology University College London Medical School and Royal Free Sheila Sherlock Liver Centre Royal Free Hospital London UK ANNA S. F. LOK Division of Gastroenterology University of Michigan Health System Ann Arbor USA ANDREW K. BURROUGHS Royal Free Sheila Sherlock Liver Centre Royal Free Hospital; University College London London UK E. JENNY HEATHCOTE Division of Gastroenterology University Health Network University of Toronto Toronto Ontario Canada 12TH EDITION A John Wiley & Sons, Ltd., Publication This edition fi rst published 2011, © 1963, 1968, 1975, 1981, 1985, 1989, 1993, 1997, 2002, 2011 by Blackwell Publishing Ltd Blackwell Publishing was acquired by John Wiley & Sons in February 2007. Blackwell’s publishing program has been merged with Wiley’s global Scientifi c, Technical and Medical business to form Wiley-Blackwell. First published 1955 Second edition 1958 Third edition 1963 Fourth edition 1968 Fifth edition 1975 Sixth edition 1981 Seventh edition 1985 Eighth edition 1989 Ninth edition 1993 Tenth edition 1997 Eleventh edition 2002 Registered offi ce: John Wiley & Sons Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK Editorial offi ces: 9600 Garsington Road, Oxford, OX4 2DQ, UK The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK 111 River Street, Hoboken, NJ 07030-5774, USA For details of our global editorial offi ces, for customer services and for information about how to apply for permission to reuse the copyright material in this book please see our website at www.wiley.com/wiley-blackwell The right of the author to be identifi ed as the author of this work has been asserted in accordance with the Copyright, Designs and Patents Act 1988. All rights reserved. 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No warranty may be created or extended by any promotional statements for this work. Neither the publisher nor the author shall be liable for any damages arising herefrom. Library of Congress Cataloging-in-Publication Data Sherlock’s diseases of the liver and biliary system / edited by James S. Dooley ... [et al.]. – 12th ed. p. ; cm. Diseases of the liver and biliary system Rev. ed. of: Diseases of the liver and biliary system / Sheila Sherlock. 11th ed. 2002. Includes bibliographical references and index. ISBN 978-1-4051-3489-7 (hardcover : alk. paper) 1. Liver–Diseases. 2. Biliary tract–Diseases. I. Dooley, James (James S.) II. Sherlock, Sheila, Dame. Diseases of the liver and biliary system. III. Title: Diseases of the liver and biliary system. [DNLM: 1. Liver Diseases. 2. Biliary Tract Diseases. WI 700] RC845.S52 2011 616.3’6–dc22 2010039149 A catalogue record for this book is available from the British Library. This book is published in the following electronic formats: ePDF 9781444341263; Wiley Online Library 9781444341294; ePub 9781444341270; Mobi 9781444341287 Set in 9.5/12 pt Palatino by Toppan Best-set Premedia Limited 01 2011 Contents List of Contributors, xi 3 Biopsy of the Liver, 36 David Patch & Amar Paul Dhillon Preface to the Twelfth Edition, xv Selection and preparation of the patient, 36 Techniques, 37 Preface to the First Edition, xvi Risks and complications, 40 Sampling variability, 41 Naked-eye appearances, 43 1 Anatomy and Function, 1 Preparation of the specimen, 43 Jay H. Lefkowitch Interpretation, 43 Development of the liver and bile ducts, 1 Indications, 43 Anatomy of the liver, 1 Special methods, 45 Functional liver anatomy: sectors and segments, References, 46 3 Anatomical abnormalities of the liver, 4 4 Haematological Disorders Anatomy of the biliary tract, 5 of the Liver, 48 Surface marking, 6 Pramod K. Mistry & Dhanpat Jain Methods of examination, 6 Microanatomy of the liver, 7 The liver and blood coagulation, 50 Hepatic ultrastructure (electron microscopy) and Haemolytic jaundice, 53 organelle functions, 11 The liver in haemolytic anaemias, 54 Functional heterogeneity of the liver, 15 The liver in myelo- and lymphoproliferative disease, 57 Dynamics of the hepatic microenvironment in Leukaemia, 57 physiology and disease, 16 Bone marrow transplantation, 57 Hepatocyte death and regeneration, 17 Lymphoma, 58 References, 18 Lipid storage diseases, 62 References, 66 2 Assessment of Liver Function, 20 Sandeep Mukherjee & John L. Gollan 5 Acute Liver Failure, 70 Shannan R. Tujios & William M. Lee Selection of biochemical tests, 20 Bile pigments, 21 Defi nition, 70 Serum enzyme tests, 22 Epidemiology and aetiologies, 71 Quantitative assessment of hepatic function, Clinical features, 74 25 Initial investigations, 75 Lipid and lipoprotein metabolism, 25 Complications and management of acute liver failure, 77 Bile acids, 27 Specifi c therapies, 84 Amino acid metabolism, 30 Prognosis, 86 Plasma proteins, 30 Liver transplantation, 86 Carbohydrate metabolism, 33 Liver support systems, 88 Effects of ageing on the liver, 33 Conclusion, 88 References, 34 References, 89 v vi Contents 6 Hepatic Fibrogenesis, 94 Bleeding oesophageal varices, 179 Meena B. Bansal & Scott L. Friedman Management of acute variceal bleeding, 181 The hepatic veins, 189 Introduction, 94 Budd–Chiari (hepatic venous obstruction) syndrome, Natural history of hepatic fi brosis, 94 191 Cellular and molecular features of hepatic fi brosis, 95 Circulatory failure, 197 Clinical aspects of hepatic fi brosis, 100 References, 202 Emerging antifi brotic targets and strategies, 101 References, 101 10 Ascites, 210 Guadalupe Garcia-Tsao 7 Hepatic Cirrhosis, 103 P. Aiden McCormick Mechanisms of ascites formation, 210 Clinical features, 213 Defi nition, 103 Differential diagnosis, 215 Causes of cirrhosis, 103 Spontaneous bacterial peritonitis, 216 Anatomical diagnosis, 104 Treatment of cirrhotic ascites, 218 Reversible cirrhosis, 106 Hyponatraemia, 222 Clinical cirrhosis: compensated versus decompensated, Refractory ascites, 223 106 Hepatorenal syndrome, 224 Vasodilatation and hyperdynamic circulation, 108 Prognosis, 228 Prognosis (Child–Pugh score, MELD, UKELD), 110 References, 228 Clinical and pathological associations, 111 Management, 117 11 Jaundice and Cholestasis, 234 References, 118 Elwyn Elias 8 Hepatic Encephalopathy in Patients Introduction, 234 with Cirrhosis, 121 Classifi cation of jaundice, 234 Physiology and pathophysiology, 235 Marsha Y. Morgan Syndrome of cholestasis, 240 Classifi cation, 121 Investigation of the jaundiced patient, 245 Diagnosis, 124 Differential diagnosis, 247 Differential diagnosis, 130 Treatment, 249 Hepatic encephalopathy and liver transplantation, 131 Familial non-haemolytic hyperbilirubinaemias, 250 Prognosis, 131 References, 254 Pathogenesis, 131 Management of hepatic encephalopathy, 139 12 Gallstones and Benign Biliary Prevention, 146 Diseases, 257 References,