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