Sherlock’s Diseases of the Liver and Biliary System (13th Edition)

Description

A new, fully updated edition of the world’s most famous book on liver diseases—with updating of all areas and inclusion of new specific topics, by internationally renowned specialists

This brand new edition of the classic book on hepatology provides a concise, clearly presented and well-structured review across the whole spectrum of hepatobiliary diseases by some of the world’s leading hepatologists and hepatobiliary specialists. Where many other hepatology textbooks provide detailed accounts of basic science and clinical management, Sherlock’s Diseases of the Liver and Biliary System, 13th Edition takes a different approach. Concentrating on the clinical decisions to be taken and the relevant supporting data, it is written and edited to maintain Sheila Sherlock’s unique approach, in particular the clarity and layout of the text, and the explanatory figures and tables. The book is thus concise, highly accessible, and generously illustrated with over 700 attractive color figures. There is a pithy approach to each disease based both on evidence and on the authors’ experience, the hallmark of this book. Based on these elements, the 12^th edition was awarded first prize in the 2012 British Medical Association Book Awards in the Internal Medicine category.

Sherlock’s Diseases of the Liver and Biliary System begins by introducing the anatomy and function of the liver to readers, continuing then with in-depth coverage of liver biopsy techniques and interpretation, and fibrogenesis and its assessment. There are then chapters on all aspects of liver and biliary disease including acute liver failure, cirrhosis, portal hypertension, hepatic encephalopathy, ascites, hepatitis B and C, alcohol and the liver, non-alcoholic fatty liver disease, drug related liver reactions, cholestatic, autoimmune and genetic liver diseases, benign and malignant tumours and not least liver transplantation. There are also chapters on the liver in pregnancy, in the neonate, infancy and childhood, in systemic diseases and in infections. This new edition also features four new individual chapters focusing on coagulation, non-invasive assessment of fibrosis and cirrhosis; vascular diseases of the liver and portal vein thrombosis, and nutrition in liver disease. Digital downloads of the figures from this edition are offered on a companion website.

• Internationally recognized and loved, world-renowned hepatology book, first published in 1955
• Takes a one-of-a-kind, clinical approach maintaining Sheila Sherlock’s clarity and legacy of presentation
• Full colour throughout with 700 illustrative figures
• Wide faculty of international contributors

Sherlock’s Diseases of the Liver and Biliary System, 13th Edition is an ideal primer in hepatology for students and trainees in hepatology and gastroenterology, and a valuable resource for all specialist gastroenterologists and hepatologists, paediatricians, pathologists, radiologists, general physicians and specialist nurses.

Table of Contents

1. List of Contributors xi
2. Preface to the Thirteenth Edition xv
3. Preface to the First Edition xvi
4. Anatomy and Function 1 – Jay H. Lefkowitch
   1. Development of the liver and bile ducts 1
   2. Anatomy of the liver 1
   3. Functional liver anatomy: sectors and segments 3
   4. Anatomical abnormalities of the liver 3
   5. Anatomy of the biliary tract 4
   6. Surface marking 5
   7. Methods of examination 6
   8. Microanatomy of the liver 7
   9. Hepatic ultrastructure (electron microscopy) and organelle functions 9
   10. Functional heterogeneity of the liver 13
   11. Dynamics of the hepatic microenvironment in physiology and disease 14 Hepatocyte death and regeneration 15 References 16
5. 2 Liver Function in Health and Disease: Clinical Application of Liver Tests 20 – George Mells and Graeme Alexander
   1. Bilirubin metabolism 21
   2. Bile acids 23
   3. Lipid and lipoprotein metabolism 26
   4. Amino acid metabolism 28
   5. Plasma proteins 29
   6. Carbohydrate metabolism 31
   7. Markers of hepatocellular injury: the serum transaminases 32
   8. Markers of cholestasis: alkaline phosphatase (ALP) and gamma‐glutamyl transferase (GGT) 33
   9. Haematology in liver disease 34
   10. Effects of ageing on the liver 35
   11. References 36
6. 3 Biopsy of the Liver 39 – David Patch and Tu Vinh Luong
   1. Selection and preparation of the patient 39
   2. Techniques 40
   3. Risks and complications 43
   4. Sampling variability 45
   5. Naked‐eye appearances 46
   6. Preparation of the specimen 46
   7. Interpretation: a stepwise diagnostic approach 46
   8. Indications 48
   9. Special methods 49
   10. References 50
7. 4 Coagulation in Cirrhosis 53 – Nicolas M. Intagliata and Stephen H. Caldwell
   1. Introduction 53
   2. Normal coagulation pathways: a hepatologist’s perspective 54
   3. The coagulation system in cirrhosis 56
   4. Bleeding and thrombosis in cirrhosis 57
   5. Clinical laboratory tests of the coagulation system in cirrhosis 58
   6. Conclusion 60
   7. References 60
8. 5 Acute Liver Failure 62 – Shannan R. Tujios and William M. Lee
   1. Definition 62
   2. Epidemiology and aetiologies 63
   3. Clinical features 66
   4. Initial investigations 67
   5. Complications and management of acute liver failure 68
   6. Specific therapies 73
   7. Prognosis 75
   8. Liver transplantation 76
   9. Conclusion 78
   10. References 78
9. 6 Hepatic Fibrogenesis 82 – Meena B. Bansal and Scott L. Friedman
   1. Introduction 82
   2. Natural history of hepatic fibrosis 82
   3. Cellular and molecular features of hepatic fibrosis 83
   4. Clinical aspects of hepatic fibrosis 89
   5. Emerging antifibrotic targets and strategies 89
   6. References 90
10. 7 Non‐invasive Assessment of Fibrosis and Cirrhosis 93 – Avik Majumdar and Massimo Pinzani
    1. Introduction 93
    2. The use of invasive and non‐invasive tests 93
    3. Non‐invasive tests: specifics 95
    4. Conclusions 102
    5. References 103
11. 8 Hepatic Cirrhosis 107 – P. Aiden McCormick and Rajiv Jalan
    1. Definition 107
    2. Causes of cirrhosis 107
    3. Anatomical diagnosis 108
    4. Reversible cirrhosis 110
    5. Clinical cirrhosis: compensated versus
    6. decompensated 110
    7. Prognosis (Child–Pugh score, MELD, UKELD) 111
    8. Clinical and pathological associations 112
    9. Management 119
    10. Acute‐on‐chronic liver failure 120
    11. References 123
12. 9 Ascites 127 – Guadalupe Garcia‐Tsao
    1. Mechanisms of ascites formation 127
    2. Clinical features 130
    3. Differential diagnosis 132
    4. Spontaneous bacterial peritonitis 132
    5. Treatment of cirrhotic ascites 134
    6. Hyponatraemia 138
    7. Refractory ascites 139
    8. Hepatorenal syndrome 141
    9. Prognosis 144
    10. References 145
13. 10 Hepatic Encephalopathy in Patients with Cirrhosis 151 – Marsha Y. Morgan
    1. Clinical Features 151
    2. Classification 153
    3. Prevalence and consequences 154
    4. Diagnosis 154
    5. Diagnostic comorbidities, confounders, and alternatives 160
    6. Pathogenesis 161
    7. Management 167
    8. Prevention 174
    9. References 174
14. 11 Portal Hypertension in Cirrhosis 180 – Jaime Bosch and Annalisa Berzigotti
    1. Introduction 180
    2. Pathophysiology and rational basis of therapy 185
    3. Evaluation and diagnosis 188
    4. Natural history and prognosis 194
    5. Management 196
    6. Treatment of portal hypertension according to clinical scenarios 200
    7. References 205
15. 12 Vascular Disorders of the Liver and Extrahepatic Portal Hypertension 209 – Dominique‐Charles Valla
    1. Hepatic artery occlusion 209
    2. Aneurysms of the hepatic artery 210
    3. Hepatic arterioportal fistula 211
    4. Hepatic vascular malformations in hereditary haemorrhagic telangiectasia 212
    5. Congenital portosystemic shunts – Abernethy malformation 213
    6. Budd–Chiari syndrome – hepatic venous outflow tract obstruction 214
    7. Extrahepatic portal vein obstruction – portal vein thrombosis and portal cavernoma in the absence of cirrhosis 217
    8. Portal vein thrombosis in patients with cirrhosis 220
    9. Idiopathic non‐cirrhotic intrahepatic portal hypertension 221
    10. Hypoxic hepatitis 223
    11. Congestive cardiac hepatopathy 223
    12. Non‐obstructive sinusoidal dilation (NOSD) and peliosis 225
    13. References 226
16. 13 Jaundice and Cholestasis 231 – Peter L. M. Jansen
    1. Introduction 231
    2. Mechanics of bile formation 233
    3. Syndrome of cholestasis 238
    4. Causes of isolated hyperbilirubinaemia 239
    5. Causes of cholestatic and hepatocellular jaundice 242
    6. Consequences of cholestasis and their management 244
    7. Investigation of the jaundiced patient 247
    8. Decisions to be made in the jaundiced patient Management of cholestatic disorders 251
    9. References 251
17. 14 Gallstones and Benign Biliary Disease 256 – James S. Dooley, Kurinchi S. Gurusamy, and Brian R. Davidson
    1. Introduction 256
    2. Imaging the gallbladder and biliary tract 256
    3. Gallstones 259
    4. Symptoms and complications of gallstones 264
    5. Cholecystectomy 266
    6. Complicated acute gallbladder disease 268
    7. Percutaneous cholecystostomy 269
    8. Asymptomatic gallbladder stones 269
    9. Non‐surgical treatment of gallstones in the gallbladder 269 Common bile duct stones 270 Acute gallstone pancreatitis 272 Large common duct stones 272 Mirizzi syndrome 273
    10. Intrahepatic gallstones 274
    11. Haemobilia 274
    12. Functional gallbladder and sphincter of Oddi disorders 274
    13. Other gallbladder pathologies 276
    14. Relationships to malignant change 279
    15. Benign biliary strictures 279
    16. Anastomotic strictures following biliary surgery 282
    17. IgG4‐related sclerosing cholangitis 283
    18. Chronic pancreatitis 283
    19. References 284
18. 15 Malignant Biliary Diseases 294 – Rahul S. Koti and John Bridgewater
    1. Carcinoma of the gallbladder 294
    2. Carcinoma of the bile duct (cholangiocarcinoma) 296
    3. Other biliary malignancies 301 Metastases at the hilum 301
    4. Ampullary and periampullary carcinomas 301
    5. Conclusion 305
    6. References 305
19. 16 Fibropolycystic Liver Diseases and Congenital Biliary Abnormalities 308 – Nedim Hadžić and Mario Strazzabosco
    1. Overview 308
    2. Polycystic liver disease 309
    3. Fibropolycystic diseases 313
    4. Autosomal recessive polycystic kidney disease 314
    5. Congenital hepatic fibrosis 314
    6. Caroli disease 316
    7. Microhamartomas (von Meyenberg complexes) Choledochal cysts 318
    8. Solitary non‐parasitic liver cyst 320
    9. Congenital anomalies of the biliary tract 321
    10. References 324
20. 17 Primary Biliary Cholangitis 328 – David E. J. Jones
    1. Clinical features 328
    2. Diagnosis 329
    3. Epidemiology 333
    4. Aetiology and pathogenesis 334
    5. Management 335
    6. Prognosis 337
    7. References 338
21. 18 Sclerosing Cholangitis 341 – Tom Hemming Karlsen and Kirsten Muri Boberg
    1. Introduction 341
    2. Primary sclerosing cholangitis 341
    3. Secondary sclerosing cholangitis 350
    4. Sclerosing cholangitis in systemic inflammatory
    5. diseases 351
    6. References 351
22. 19 Autoimmune Hepatitis and Overlap Syndromes 355 – Ashnila Janmohamed and Gideon M. Hirschfield
    1. Introduction 355
    2. Disease overview 356
    3. Biological determinants of disease 357
    4. Disease presentation 359
    5. Laboratory features 361
    6. Imaging 363
    7. Liver biopsy and histological features 363
    8. Differential diagnosis 365
    9. Diagnostic dilemmas 366
    10. Making a diagnosis in practice 367
    11. Management strategies 368
    12. Pretreatment and on‐treatment considerations 371
    13. Treatment challenges and alternative agents 371
    14. Pregnancy and autoimmune hepatitis 372
    15. The elderly and autoimmune hepatitis 372
    16. Childhood‐onset autoimmune hepatitis 373
    17. Autoimmune hepatitis and liver transplantation 373
    18. Overlap syndromes 374
    19. Conclusion 377
    20. References 377
23. 20 Enterically Transmitted Viral Hepatitis: Hepatitis A and Hepatitis E 380 – Rinjal D. Brahmbhatt and Anna S. F. Lok
    1. General features of enterically transmitted viral hepatitis 380
    2. Hepatitis A virus 385
    3. Hepatitis E virus 389
    4. References 391
24. 21 Hepatitis B 395 – Anna S. F. Lok
    1. Introduction 395
    2. Hepatitis B virus 395
    3. Immune response and mechanisms of hepatic injury 398
    4. Epidemiology 399
    5. Prevention 400
    6. Diagnosis 402
    7. Clinical manifestations 404
    8. Natural history 405
    9. Treatment 408
    10. HBV and HCV coinfection 414
    11. HBV and HDV coinfection 414
    12. HBV and HIV coinfection 414
    13. References 415
25. 22 Hepatitis D 421 – Patrizia Farci and Grazia Anna Niro
    1. History 421
    2. Hepatitis D virus 421
    3. Epidemiology 422
    4. Pathogenesis 425
    5. Modes of infection and clinical course 425
    6. Diagnosis 428
    7. Treatment 429
    8. Prevention 432
    9. References 432
26. 23 Hepatitis C 436 – Geoffrey Dusheiko
    1. Introduction 436
    2. Epidemiology 436
    3. Virology 438
    4. Pathology and pathogenesis 439
    5. Diagnostic tests for hepatitis C 440
    6. Acute hepatitis C 441
    7. Chronic hepatitis C 441
    8. References 459
27. 24 Drug‐Induced Liver Injury 468 – Frank W. DiPaola and Robert J. Fontana
    1. Introduction 468
    2. Epidemiology 468
    3. Complications of DILI 469
    4. Classification of hepatotoxicity 469
    5. Drug metabolism and pharmacokinetics 469
    6. Hepatic drug metabolism 471
    7. Molecular mechanisms in drug‐induced liver injury 472
    8. Non‐genetic risk factors for DILI 473
    9. Diagnosis of DILI 474
    10. Medical management 478
    11. Pharmacogenetic risk factors 478
    12. Potential immunological mechanisms in idiosyncratic DILI 479
    13. Liver injury from specific drugs 479
    14. References 486
28. 25 Alcohol and the Liver 494 – Stephen Stewart and Ewan Forrest
    1. Introduction 494
    2. Alcohol metabolism 494
    3. Pathogenesis 496
    4. Susceptibility 497
    5. Histological features 498
    6. Clinical features 501
    7. Clinical syndromes 503
    8. Prognosis 504
    9. Treatment 506
    10. Conclusions 507
    11. References 508
29. 26 Iron Overload States 511 – Paul Adams and Heinz Zoller
    1. Normal iron physiology 511
    2. Iron overload and liver damage 515
    3. Genetic haemochromatosis 516
    4. Other iron storage diseases 521
    5. References 523
30. 27 Wilson Disease 526 – Eve A. Roberts and Karl Heinz Weiss
    1. Molecular genetics: pathogenesis 527
    2. Pathology 528
    3. Clinical picture 529
    4. Laboratory tests 532
    5. Genetic strategies 533
    6. Diagnostic difficulties 533
    7. Treatment 533
    8. Prognosis 536
    9. Non-Wilsonian copper-related cirrhosis 536
    10. References 536
31. 28 Non‐Alcoholic Fatty Liver Disease 540 – Timothy Hardy and Christopher P. Day
    1. Introduction 540
    2. Further definitions, terminology, and diagnosis 541
    3. Liver biopsy, classification of NAFLD, and non‐ invasive markers of NASH and fibrosis 541
    4. Clinical features 543
    5. Laboratory testing 544
    6. Epidemiology 545
    7. Ethnic variation in NAFLD 545
    8. Pathogenesis of NASH 545
    9. Natural history of NAFLD 550
    10. NAFLD and hepatocellular carcinoma (HCC) 551
    11. Therapy for non‐alcoholic fatty liver disease 552
    12. Other forms of NAFLD 554
    13. References 555
32. 29 Nutrition and Chronic Liver Disease 561 – Manuela Merli
    1. Introduction 561
    2. Epidemiology and general characteristics 562
    3. Causes of malnutrition 562
    4. Consequences of malnutrition 564
    5. Diagnosis and assessment 565
    6. Treatment and management 568
    7. References 570
33. 30 Pregnancy and the Liver 572 – Rachel H. Westbrook and Catherine Williamson
    1. Introduction 572
    2. Normal physiology in pregnancy 572
    3. Pregnancy‐related liver diseases 573
    4. Pre‐existing liver diseases and pregnancy 578
    5. Liver transplantation and pregnancy 580
    6. Liver disease coincidentally
    7. arising with pregnancy 581
    8. Conclusion 582
    9. References 582
34. 31 The Liver in the Neonate, in Infancy, and Childhood 588 – Susan M. Siew and Deirdre A. Kelly
    1. Investigation of liver disease in children 588
    2. Neonatal jaundice 589
    3. Neonatal unconjugated hyperbilirubinaemia 589
    4. Neonatal liver disease (conjugated hyperbilirubinaemia) 591
    5. Neonatal hepatitis 594
    6. Inherited disease in the neonate 596
    7. Genetic cholestatic syndromes 598
    8. Structural abnormalities: biliary atresia and choledochal cyst 600
    9. Acute liver failure in infancy 602
    10. Liver disease in older children 605
    11. Metabolic disease in older children 607
    12. Cirrhosis and portal hypertension 613
    13. Liver transplantation 613
    14. Tumours of the liver 614
    15. References 615
35. 32 The Liver in Systemic Diseases 622 – James S. Dooley and Christopher McNamara
    1. Collagen‐vascular and autoimmune disorders 622
    2. Hepatic granulomas 624
    3. Sarcoidosis 626
    4. The liver in endocrine disorders 628
    5. Amyloidosis 629
    6. Porphyrias 632
    7. The liver in haemolytic anaemias 634
    8. The liver in myelo‐ and lymphoproliferative disease 638
    9. Bone marrow transplantation 639
    10. Lymphoma 640
    11. Extramedullary haemopoiesis 642
    12. Rare haematological disorders that may involve the liver 643
    13. Lipid storage diseases 643
    14. Non‐metastatic complications of malignancy 646
    15. References 646
36. 33 The Liver in Infections 652 – Sanjay Bhagani and Ian Cropley
    1. Introduction 652
    2. Jaundice of infections 652
    3. Pyogenic liver abscess 652
    4. Hepatic amoebiasis 655
    5. Tuberculosis of the liver 657
    6. Hepatic actinomycosis 659
    7. Syphilis of the liver 659
    8. Perihepatitis 660
    9. Leptospirosis 660
    10. Relapsing fever 663
    11. Lyme disease 663
    12. Rickettsial infections 663
    13. Fungal infections 664
    14. Schistosomiasis (bilharzia) 665
    15. Malaria 667
    16. Kala‐azar (visceral leishmaniasis) 668
    17. Echinococcosis (hydatid disease) 668
    18. Ascariasis 673
    19. Strongyloides stercoralis 674
    20. Trichinosis 674
    21. Toxocara canis (visceral larva migrans) 674
    22. Liver flukes 675
    23. References 676
37. 34 Imaging of the Liver and Diagnostic Approach of Space‐Occupying Lesions 682 – Neil H. Davies and Dominic Yu
    1. Ultrasound 682
    2. Computed tomography 683
    3. Magnetic resonance imaging 685
    4. Radioisotope scanning 688
    5. Positron emission tomography 691
    6. MR spectroscopy 691
    7. Conclusions and choice of imaging technique 691
    8. References 691
38. 35 Benign Liver Tumours 693 – Ian R. Wanless
    1. Diagnosis of focal liver lesions 693
    2. Hepatocellular lesions 693
    3. Biliary and cystic lesions 699
    4. Mesenchymal tumours 700
    5. References 701
39. 36 Primary Malignant Neoplasms of the Liver 705 – Adam Doyle and Morris Sherman
    1. Hepatocellular carcinoma 705
    2. Intrahepatic cholangiocarcinoma 718
    3. Other malignant neoplasms of the liver 721
    4. Other sarcomas 722
    5. References 722
40. 37 Hepatic Transplantation 730 – Lindsay Y. King and Carl L. Berg
    1. Selection of patients 730
    2. Candidates 732
    3. Absolute and relative contraindications 737
    4. General preparation of the patient 738
    5. Donor selection and operation 738
    6. The recipient operation 739
    7. Immunosuppression 742
    8. Postoperative course 743
    9. Post‐transplantation complications 744
    10. Conclusion 751
    11. References 752
41. 38 Hepatic Transplantation and HBV, HCV, and HIV Infections 758 – Norah A. Terrault
    1. Introduction 758
    2. Hepatitis B and liver transplantation 759
    3. Hepatitis C and liver transplantation 763
    4. HIV and liver transplantation 770
    5. References 773
    6. Index 781

Author Biography

James S. Dooley, The Royal Free Hospital, London, UK.

Anna S. Lok, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA.

Guadalupe Garcia-Tsao, Yale University School of Medicine, VA-CT Healthcare System, CT, USA.

Massimo Pinzani, UCL Institute for Liver and Digestive Health, London, UK.