Sherlock's diseases of the liver and biliary system

835 Pages·2018·89.98 MB·english

by  Dooley

SHERLOCK’S DISEASES OF THE LIVER AND BILIARY SYSTEM
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About the Companion Website
This book is accompanied by a companion website:
www.wiley.com/go/sherlock/liver13e
The website includes:
● PowerPoints of all figures from the book for downloading
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Sherlock’s Diseases of the Liver and Biliary System
Thirteenth Edition
Edited By
James S. Dooley
University College London Institute for Liver and Digestive Health and
Sheila Sherlock Liver Centre
Royal Free London NHS Foundation Trust
London
UK
Anna S. F. Lok
Division of Gastroenterology and Hepatology
Michigan Medicine, University of Michigan
Ann Arbor, MI
USA
Guadalupe Garcia‐Tsao
Section of Digestive Diseases
Yale University School of Medicine
New Haven, CT;
Veterans Affairs Connecticut Healthcare System
West Haven, CT
USA
Massimo Pinzani
University College London Institute for Liver and Digestive Health and
Sheila Sherlock Liver Centre
Royal Free London NHS Foundation Trust
London
UK
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iii
This edition first published 2018.
© 2018 John Wiley & Sons Ltd
Edition History
Blackwell Publishing Ltd (1e, 1955; 2e,1958; 3e,1963; 4e,1968; 5e,1975; 6e,1981; 7e,1985; 8e,1989; 9e,1993; 10e,1997; 11e, 2002; 12e, 2011)
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The right of James S. Dooley, Anna S. F. Lok, Guadalupe Garcia‐Tsao, Massimo Pinzani to be identified as the authors of the editorial material in
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Library of Congress Cataloging‐in‐Publication Data
Names: Dooley, James (James S.), editor. | Lok, Anna S. F., editor. | Garcia-Tsao, Guadalupe, editor. | Pinzani, Massimo, editor.
Title: Sherlock’s diseases of the liver and biliary system / edited by James S. Dooley, Anna S.F. Lok, Guadalupe Garcia-Tsao, Massimo Pinzani.
Other titles: Diseases of the liver and biliary system
Description: 13th edition. | Hoboken, NJ : Wiley, 2018. | Preceded by Sherlock’s diseases of the liver and biliary system / edited by
James S. Dooley … [et al.]. 12th ed. 2011. | Includes bibliographical references and index. |
Identifiers: LCCN 2017057075 (print) | LCCN 2017059204 (ebook) | ISBN 9781119237563 (pdf) | ISBN 9781119237648 (epub) |
ISBN 9781119237549 (cloth)
Subjects: | MESH: Liver Diseases | Biliary Tract Diseases
Classification: LCC RC845 (ebook) | LCC RC845 (print) | NLM WI 700 | DDC 616.3/6–dc23
LC record available at https://lccn.loc.gov/2017057075
Cover image: © Nerthuz/Shutterstock
Cover design by Wiley
Set in 10/12pt Warnock by SPi Global, Pondicherry, India
1 2018
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v
Contents
List of Contributors 3 Biopsy of the Liver
xi 39
Preface to the Thirteenth Edition David Patch and Tu Vinh Luong
xv
Preface to the First Edition xvi Selection and preparation of the patient 39
Techniques 40
1 Anatomy and Function 1 Risks and complications 43
Jay H. Lefkowitch Sampling variability 45
Development of the liver and bile ducts 1 Naked‐eye appearances 46
Anatomy of the liver 1 Preparation of the specimen 46
Functional liver anatomy: sectors and  Interpretation: a stepwise diagnostic
segments 3 approach 46
Anatomical abnormalities of the liver 3 Indications 48
Anatomy of the biliary tract 4 Special methods 49
Surface marking 5 References 50
Methods of examination 6
4 Coagulation in Cirrhosis
Microanatomy of the liver 7 53
Hepatic ultrastructure (electron microscopy) Nicolas M. Intagliata and Stephen H. Caldwell
and organelle functions 9 Introduction 53
Functional heterogeneity of the liver 13 Normal coagulation pathways: a hepatologist’s
Dynamics of the hepatic microenvironment perspective 54
in physiology and disease 14 The coagulation system in cirrhosis 56
Hepatocyte death and regeneration 15 Bleeding and thrombosis in cirrhosis 57
References 16 Clinical laboratory tests of the coagulation
system in cirrhosis 58
2 Liver Function in Health and Disease: Clinical Conclusion 60
Application of Liver Tests 20 References 60
George Mells and Graeme Alexander
Bilirubin metabolism 21 5 Acute Liver Failure 62
Bile acids 23 Shannan R. Tujios and William M. Lee
Lipid and lipoprotein metabolism 26 Definition 62
Amino acid metabolism 28 Epidemiology and aetiologies 63
Plasma proteins 29 Clinical features 66
Carbohydrate metabolism 31 Initial investigations 67
Markers of hepatocellular injury: the serum Complications and management of acute liver
transaminases 32 failure 68
Markers of cholestasis: alkaline phosphatase (ALP) Specific therapies 73
and gamma‐glutamyl transferase (GGT) 33 Prognosis 75
Haematology in liver disease 34 Liver transplantation 76
Effects of ageing on the liver 35 Conclusion 78
References 36 References 78
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vi Contents
6 Hepatic Fibrogenesis 82 Diagnostic comorbidities, confounders,
Meena B. Bansal and Scott L. Friedman and alternatives 160
Introduction 82 Pathogenesis 161
Natural history of hepatic fibrosis 82 Management 167
Cellular and molecular features of hepatic Prevention 174
fibrosis 83 References 174
Clinical aspects of hepatic fibrosis 89
Emerging antifibrotic targets and strategies 89 11 Portal Hypertension in Cirrhosis
180
References 90 Jaime Bosch and Annalisa Berzigotti
Introduction 180
7 Non‐invasive Assessment of Fibrosis
Pathophysiology and rational basis
and Cirrhosis
93 of therapy 185
Avik Majumdar and Massimo Pinzani
Evaluation and diagnosis 188
Introduction 93 Natural history and prognosis 194
The use of invasive and non‐invasive tests 93 Management 196
Non‐invasive tests: specifics 95 Treatment of portal hypertension according
Conclusions 102 to clinical scenarios 200
References 103 References 205
8 Hepatic Cirrhosis
107
12 Vascular Disorders of the Liver and Extrahepatic
P. Aiden McCormick and Rajiv Jalan
Portal Hypertension
209
Definition 107
Dominique‐Charles Valla
Causes of cirrhosis 107
Hepatic artery occlusion 209
Anatomical diagnosis 108
Aneurysms of the hepatic artery 210
Reversible cirrhosis 110
Hepatic arterioportal fistula 211
Clinical cirrhosis: compensated versus
Hepatic vascular malformations in hereditary
decompensated 110
haemorrhagic telangiectasia 212
Prognosis (Child–Pugh score, MELD, UKELD) 111
Congenital portosystemic shunts – Abernethy
Clinical and pathological associations 112
malformation 213
Management 119
Budd–Chiari syndrome – hepatic venous outflow
Acute‐on‐chronic liver failure 120
tract obstruction 214
References 123
Extrahepatic portal vein obstruction – portal vein
9 Ascites thrombosis and portal cavernoma in the
127
Guadalupe Garcia‐Tsao absence of cirrhosis 217
Portal vein thrombosis in patients
Mechanisms of ascites formation 127
with cirrhosis 220
Clinical features 130
Idiopathic non‐cirrhotic intrahepatic portal
Differential diagnosis 132
hypertension 221
Spontaneous bacterial peritonitis 132
Hypoxic hepatitis 223
Treatment of cirrhotic ascites 134
Congestive cardiac hepatopathy 223
Hyponatraemia 138
Non‐obstructive sinusoidal dilation (NOSD)
Refractory ascites 139
and peliosis 225
Hepatorenal syndrome 141
References 226
Prognosis 144
References 145
13 Jaundice and Cholestasis
231
10 Hepatic Encephalopathy in Patients Peter L. M. Jansen
with Cirrhosis 151 Introduction 231
Marsha Y. Morgan Mechanics of bile formation 233
Clinical Features 151 Syndrome of cholestasis 238
Classification 153 Causes of isolated hyperbilirubinaemia 239
Prevalence and consequences 154 Causes of cholestatic and hepatocellular
Diagnosis 154 jaundice 242
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Contents vii
Consequences of cholestasis and their Autosomal recessive polycystic kidney disease 314
management 244 Congenital hepatic fibrosis 314
Investigation of the jaundiced patient 247 Caroli disease 316
Decisions to be made in the jaundiced patient 250 Microhamartomas (von Meyenberg complexes) 318
Management of cholestatic disorders 251 Choledochal cysts 318
References 251 Solitary non‐parasitic liver cyst 320
Congenital anomalies of the biliary tract 321
14 Gallstones and Benign Biliary Disease 256 References 324
James S. Dooley, Kurinchi S. Gurusamy, and
Brian R. Davidson 17 Primary Biliary Cholangitis 328
Introduction 256 David E. J. Jones
Imaging the gallbladder and biliary tract 256 Clinical features 328
Gallstones 259 Diagnosis 329
Symptoms and complications of gallstones 264 Epidemiology 333
Cholecystectomy 266 Aetiology and pathogenesis 334
Complicated acute gallbladder disease 268 Management 335
Percutaneous cholecystostomy 269 Prognosis 337
Asymptomatic gallbladder stones 269 References 338
Non‐surgical treatment of gallstones
in the gallbladder 269 18 Sclerosing Cholangitis 341
Common bile duct stones 270 Tom Hemming Karlsen and Kirsten Muri Boberg
Acute gallstone pancreatitis 272 Introduction 341
Large common duct stones 272 Primary sclerosing cholangitis 341
Mirizzi syndrome 273 Secondary sclerosing cholangitis 350
Intrahepatic gallstones 274 Sclerosing cholangitis in systemic inflammatory
Haemobilia 274 diseases 351
Functional gallbladder and sphincter of Oddi References 351
disorders 274
Other gallbladder pathologies 276 19 Autoimmune Hepatitis and Overlap
Relationships to malignant change 279 Syndromes
355
Benign biliary strictures 279 Ashnila Janmohamed and Gideon M. Hirschfield
Anastomotic strictures following biliary surgery 282
Introduction 355
IgG4‐related sclerosing cholangitis 283
Disease overview 356
Chronic pancreatitis 283
Biological determinants of disease 357
References 284
Disease presentation 359
Laboratory features 361
15 Malignant Biliary Diseases
294 Imaging 363
Rahul S. Koti and John Bridgewater
Liver biopsy and histological features 363
Carcinoma of the gallbladder 294 Differential diagnosis 365
Carcinoma of the bile duct Diagnostic dilemmas 366
(cholangiocarcinoma) 296 Making a diagnosis in practice 367
Other biliary malignancies 301 Management strategies 368
Metastases at the hilum 301 Pretreatment and on‐treatment considerations 371
Ampullary and periampullary carcinomas 301 Treatment challenges and alternative
Conclusion 305 agents 371
References 305 Pregnancy and autoimmune hepatitis 372
The elderly and autoimmune hepatitis 372
16 Fibropolycystic Liver Diseases and Congenital
Childhood‐onset autoimmune hepatitis 373
Biliary Abnormalities
308 Autoimmune hepatitis and liver
Nedim Hadžić and Mario Strazzabosco transplantation 373
Overview 308 Overlap syndromes 374
Polycystic liver disease 309 Conclusion 377
Fibropolycystic diseases 313 References 377
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viii Contents
20 Enterically Transmitted Viral Hepatitis: Hepatitis Drug metabolism and pharmacokinetics 469
A and Hepatitis E 380 Hepatic drug metabolism 471
Rinjal D. Brahmbhatt and Anna S. F. Lok Molecular mechanisms in drug‐induced
General features of enterically transmitted viral liver injury 472
hepatitis 380 Non‐genetic risk factors for DILI 473
Hepatitis A virus 385 Diagnosis of DILI 474
Hepatitis E virus 389 Medical management 478
References 391 Pharmacogenetic risk factors 478
Potential immunological mechanisms
21 Hepatitis B 395 in idiosyncratic DILI 479
Anna S. F. Lok Liver injury from specific drugs 479
Introduction 395 References 486
Hepatitis B virus 395
25 Alcohol and the Liver
Immune response and mechanisms of hepatic 494
Stephen Stewart and Ewan Forrest
injury 398
Epidemiology 399 Introduction 494
Prevention 400 Alcohol metabolism 494
Diagnosis 402 Pathogenesis 496
Clinical manifestations 404 Susceptibility 497
Natural history 405 Histological features 498
Treatment 408 Clinical features 501
HBV and HCV coinfection 414 Clinical syndromes 503
HBV and HDV coinfection 414 Prognosis 504
HBV and HIV coinfection 414 Treatment 506
References 415 Conclusions 507
References 508
22 Hepatitis D
421
Patrizia Farci and Grazia Anna Niro 26 Iron Overload States 511
Paul Adams and Heinz Zoller
History 421
Hepatitis D virus 421 Normal iron physiology 511
Epidemiology 422 Iron overload and liver damage 515
Pathogenesis 425 Genetic haemochromatosis 516
Modes of infection and clinical course 425 Other iron storage diseases 521
Diagnosis 428 References 523
Treatment 429
27 Wilson Disease
Prevention 432 526
References 432 Eve A. Roberts and Karl Heinz Weiss
Molecular genetics: pathogenesis 527
23 Hepatitis C 436 Pathology 528
Geoffrey Dusheiko Clinical picture 529
Introduction 436 Laboratory tests 532
Epidemiology 436 Genetic strategies 533
Virology 438 Diagnostic difficulties 533
Pathology and pathogenesis 439 Treatment 533
Diagnostic tests for hepatitis C 440 Prognosis 536
Acute hepatitis C 441 Non-Wilsonian copper-related cirrhosis 536
Chronic hepatitis C 441 References 536
References 459
28 Non‐Alcoholic Fatty Liver Disease
540
24 Drug‐Induced Liver Injury 468 Timothy Hardy and Christopher P. Day
Frank W. DiPaola and Robert J. Fontana Introduction 540
Introduction 468 Further definitions, terminology, and 
Epidemiology 468 diagnosis 541
Complications of DILI 469 Liver biopsy, classification of NAFLD, and non‐
Classification of hepatotoxicity 469 invasive markers of NASH and fibrosis 541
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Contents ix
Clinical features 543 32 The Liver in Systemic Diseases 622
Laboratory testing 544 James S. Dooley and Christopher McNamara
Epidemiology 545 Collagen‐vascular and autoimmune
Ethnic variation in NAFLD 545 disorders 622
Pathogenesis of NASH 545 Hepatic granulomas 624
Natural history of NAFLD 550 Sarcoidosis 626
NAFLD and hepatocellular carcinoma The liver in endocrine disorders 628
(HCC) 551 Amyloidosis 629
Therapy for non‐alcoholic fatty liver disease 552 Porphyrias 632
Other forms of NAFLD 554 The liver in haemolytic anaemias 634
References 555 The liver in myelo‐ and lymphoproliferative
disease 638
29 Nutrition and Chronic Liver Disease Bone marrow transplantation 639
561
Manuela Merli Lymphoma 640
Extramedullary haemopoiesis 642
Introduction 561
Rare haematological disorders that may involve
Epidemiology and general characteristics 562
the liver 643
Causes of malnutrition 562
Lipid storage diseases 643
Consequences of malnutrition 564
Non‐metastatic complications of malignancy 646
Diagnosis and assessment 565
References 646
Treatment and management 568
References 570
33 The Liver in Infections
652
Sanjay Bhagani and Ian Cropley
30 Pregnancy and the Liver
572
Introduction 652
Rachel H. Westbrook and Catherine Williamson
Jaundice of infections 652
Introduction 572
Pyogenic liver abscess 652
Normal physiology in pregnancy 572
Hepatic amoebiasis 655
Pregnancy‐related liver diseases 573
Tuberculosis of the liver 657
Pre‐existing liver diseases and pregnancy 578
Hepatic actinomycosis 659
Liver transplantation and pregnancy 580
Syphilis of the liver 659
Liver disease coincidentally
Perihepatitis 660
arising with pregnancy 581
Leptospirosis 660
Conclusion 582
Relapsing fever 663
References 582
Lyme disease 663
Rickettsial infections 663
31 The Liver in the Neonate, in Infancy, Fungal infections 664
and Childhood 588 Schistosomiasis (bilharzia) 665
Susan M. Siew and Deirdre A. Kelly Malaria 667
Investigation of liver disease in children 588 Kala‐azar (visceral leishmaniasis) 668
Neonatal jaundice 589 Echinococcosis (hydatid disease) 668
Neonatal unconjugated hyperbilirubinaemia 589 Ascariasis 673
Neonatal liver disease (conjugated Strongyloides stercoralis 674
hyperbilirubinaemia) 591 Trichinosis 674
Neonatal hepatitis 594 Toxocara canis (visceral larva migrans) 674
Inherited disease in the neonate 596 Liver flukes 675
Genetic cholestatic syndromes 598 References 676
Structural abnormalities: biliary atresia
34 Imaging of the Liver and Diagnostic Approach of
and choledochal cyst 600
Space‐Occupying Lesions
Acute liver failure in infancy 602 682
Liver disease in older children 605 Neil H. Davies and Dominic Yu
Metabolic disease in older children 607 Ultrasound 682
Cirrhosis and portal hypertension 613 Computed tomography 683
Liver transplantation 613 Magnetic resonance imaging 685
Tumours of the liver 614 Radioisotope scanning 688
References 615 Positron emission tomography 691
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MR spectroscopy 691 Absolute and relative contraindications 737
Conclusions and choice of imaging technique 691 General preparation of the patient 738
References 691 Donor selection and operation 738
The recipient operation 739
35 Benign Liver Tumours 693 Immunosuppression 742
Ian R. Wanless Postoperative course 743
Diagnosis of focal liver lesions 693 Post‐transplantation complications 744
Hepatocellular lesions 693 Conclusion 751
Biliary and cystic lesions 699 References 752
Mesenchymal tumours 700
38 Hepatic Transplantation and HBV, HCV, and HIV
References 701
Infections
758
36 Primary Malignant Neoplasms of the Liver 705 Norah A. Terrault
Adam Doyle and Morris Sherman Introduction 758
Hepatocellular carcinoma 705 Hepatitis B and liver transplantation 759
Intrahepatic cholangiocarcinoma 718 Hepatitis C and liver transplantation 763
Other malignant neoplasms of the liver 721 HIV and liver transplantation 770
Other sarcomas 722 References 773
References 722
Index 781
37 Hepatic Transplantation
730
Lindsay Y. King and Carl L. Berg
Selection of patients 730
Candidates 732
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