Sherlock's diseases of the liver and biliary system

Content: List of Contributors xiPreface to the Thirteenth Edition xvPreface to the First Edition xvi1. Anatomy and Function 1Jay H. LefkowitchDevelopment of the liver and bile ducts 1Anatomy of the liver 1Functional liver anatomy: sectors and segments 3Anatomical abnormalities of the liver 3Anatomy of the biliary tract 4Surface marking 5Methods of examination 6Microanatomy of the liver 7Hepatic ultrastructure (electron microscopy) and organelle functions 9Functional heterogeneity of the liver 13Dynamics of the hepatic microenvironment in physiology and disease 14 Hepatocyte death and regeneration 15 References 162 Liver Function in Health and Disease: Clinical Application of Liver Tests 20George Mells and Graeme AlexanderBilirubin metabolism 21Bile acids 23Lipid and lipoprotein metabolism 26Amino acid metabolism 28Plasma proteins 29Carbohydrate metabolism 31Markers of hepatocellular injury: the serum transaminases 32Markers of cholestasis: alkaline phosphatase (ALP) and gamma?glutamyl transferase (GGT) 33Haematology in liver disease 34Effects of ageing on the liver 35References 363 Biopsy of the Liver 39David Patch and Tu Vinh Luong Selection and preparation of the patient 39Techniques 40Risks and complications 43Sampling variability  45Naked?eye appearances 46Preparation of the specimen 46Interpretation: a stepwise diagnostic approach 46Indications 48Special methods 49References 504 Coagulation in Cirrhosis 53Nicolas M. Intagliata and Stephen H. Caldwell    Introduction  53Normal coagulation pathways: a hepatologist's perspective 54The coagulation system in cirrhosis 56Bleeding and thrombosis in cirrhosis 57Clinical laboratory tests of the coagulation system in cirrhosis 58Conclusion 60References 605 Acute Liver Failure 62Shannan R. Tujios and William M. Lee Definition 62Epidemiology and aetiologies  63Clinical features 66Initial investigations 67Complications and management of acute liver failure 68Specific therapies 73Prognosis 75Liver transplantation 76Conclusion 78References 786 Hepatic Fibrogenesis 82Meena B. Bansal and Scott L. Friedman Introduction 82Natural history of hepatic fibrosis 82Cellular and molecular features of hepatic fibrosis 83Clinical aspects of hepatic fibrosis 89Emerging antifibrotic targets and strategies 89References 907 Non?invasive Assessment of Fibrosis and Cirrhosis 93Avik Majumdar and Massimo PinzaniIntroduction 93The use of invasive and non?invasive tests 93Non?invasive tests: specifics 95Conclusions 102References 1038 Hepatic Cirrhosis 107P. Aiden McCormick and Rajiv JalanDefinition 107Causes of cirrhosis 107Anatomical diagnosis 108Reversible cirrhosis 110Clinical cirrhosis: compensated versusdecompensated 110Prognosis (Child-Pugh score, MELD, UKELD) 111Clinical and pathological associations 112Management 119Acute?on?chronic liver failure 120References 1239 Ascites 127Guadalupe Garcia?TsaoMechanisms of ascites formation 127Clinical features 130Differential diagnosis 132Spontaneous bacterial peritonitis 132Treatment of cirrhotic ascites 134Hyponatraemia 138Refractory ascites 139Hepatorenal syndrome 141Prognosis 144References 14510 Hepatic Encephalopathy in Patients with Cirrhosis 151Marsha Y. MorganClinical Features 151Classification 153Prevalence and consequences 154Diagnosis 154Diagnostic comorbidities, confounders, and alternatives 160Pathogenesis 161Management 167Prevention 174References 17411 Portal Hypertension in Cirrhosis 180Jaime Bosch and Annalisa Berzigotti Introduction 180Pathophysiology and rational basis of therapy 185Evaluation and diagnosis  188Natural history and prognosis 194Management 196Treatment of portal hypertension according to clinical scenarios 200References 20512 Vascular Disorders of the Liver and Extrahepatic Portal Hypertension 209Dominique?Charles VallaHepatic artery occlusion 209Aneurysms of the hepatic artery 210Hepatic arterioportal fistula 211Hepatic vascular malformations in hereditary haemorrhagic telangiectasia 212Congenital portosystemic shunts - Abernethy malformation  213Budd-Chiari syndrome - hepatic venous outflow tract obstruction 214Extrahepatic portal vein obstruction - portal vein thrombosis and portal cavernoma in the absence of cirrhosis 217Portal vein thrombosis in patients with cirrhosis 220Idiopathic non?cirrhotic intrahepatic portal hypertension 221Hypoxic hepatitis 223Congestive cardiac hepatopathy 223Non?obstructive sinusoidal dilation (NOSD) and peliosis 225References 22613 Jaundice and Cholestasis 231Peter L. M. JansenIntroduction  231Mechanics of bile formation 233Syndrome of cholestasis 238Causes of isolated hyperbilirubinaemia 239Causes of cholestatic and hepatocellular jaundice 242Consequences of cholestasis and their  management 244Investigation of the jaundiced patient 247Decisions to be made in the jaundiced patient Management of cholestatic disorders 251References 25114 Gallstones and Benign Biliary Disease 256James S. Dooley, Kurinchi S. Gurusamy, and Brian R. DavidsonIntroduction  256Imaging the gallbladder and biliary tract 256Gallstones 259Symptoms and complications of gallstones 264Cholecystectomy 266Complicated acute gallbladder disease 268Percutaneous cholecystostomy 269Asymptomatic gallbladder stones 269Non?surgical treatment of gallstones in the gallbladder 269  Common bile duct stones 270 Acute gallstone pancreatitis 272 Large common duct stones 272 Mirizzi syndrome 273Intrahepatic gallstones 274Haemobilia 274Functional gallbladder and sphincter of Oddi disorders 274Other gallbladder pathologies 276Relationships to malignant change 279Benign biliary strictures 279Anastomotic strictures following biliary surgery 282IgG4?related sclerosing cholangitis 283Chronic pancreatitis 283References 28415 Malignant Biliary Diseases 294Rahul S. Koti and John Bridgewater Carcinoma of the gallbladder  294Carcinoma of the bile duct (cholangiocarcinoma) 296Other biliary malignancies 301 Metastases at the hilum 301Ampullary and periampullary carcinomas  301Conclusion 305References 30516 Fibropolycystic Liver Diseases and Congenital Biliary Abnormalities 308Nedim Had i? and Mario StrazzaboscoOverview 308Polycystic liver disease 309Fibropolycystic diseases 313Autosomal recessive polycystic kidney disease 314Congenital hepatic fibrosis 314Caroli disease 316Microhamartomas (von Meyenberg complexes) Choledochal cysts 318Solitary non?parasitic liver cyst 320Congenital anomalies of the biliary tract 321References 32417 Primary Biliary Cholangitis 328David E. J. JonesClinical features 328Diagnosis 329Epidemiology 333Aetiology and pathogenesis 334Management 335Prognosis 337References 33818 Sclerosing Cholangitis 341Tom Hemming Karlsen and Kirsten Muri BobergIntroduction  341Primary sclerosing cholangitis 341Secondary sclerosing cholangitis 350Sclerosing cholangitis in systemic inflammatorydiseases 351References 35119 Autoimmune Hepatitis and Overlap Syndromes 355Ashnila Janmohamed and Gideon M. HirschfieldIntroduction  355Disease overview  356Biological determinants of disease 357Disease presentation 359Laboratory features 361Imaging 363Liver biopsy and histological features 363Differential diagnosis 365Diagnostic dilemmas 366Making a diagnosis in practice 367Management strategies 368Pretreatment and on?treatment considerations 371Treatment challenges and alternative agents 371Pregnancy and autoimmune hepatitis 372The elderly and autoimmune hepatitis 372Childhood?onset autoimmune hepatitis 373Autoimmune hepatitis and liver transplantation 373Overlap syndromes 374Conclusion 377References 37720 Enterically Transmitted Viral Hepatitis: Hepatitis A and Hepatitis E 380Rinjal D. Brahmbhatt and Anna S. F. LokGeneral features of enterically transmitted viral hepatitis 380Hepatitis A virus 385Hepatitis E virus 389References 39121 Hepatitis B 395Anna S. F. Lok Introduction 395Hepatitis B virus 395Immune response and mechanisms of hepatic injury 398Epidemiology 399Prevention 400Diagnosis 402Clinical manifestations 404Natural history 405Treatment 408HBV and HCV coinfection 414HBV and HDV coinfection 414HBV and HIV coinfection 414References 41522 Hepatitis D 421Patrizia Farci and Grazia Anna Niro History 421Hepatitis D virus 421Epidemiology 422Pathogenesis 425Modes of infection and clinical course 425Diagnosis 428Treatment 429Prevention 432References 43223 Hepatitis C 436Geoffrey Dusheiko Introduction 436Epidemiology 436Virology 438Pathology and pathogenesis 439Diagnostic tests for hepatitis C 440Acute hepatitis C 441Chronic hepatitis C 441References 45924 Drug?Induced Liver Injury 468Frank W. DiPaola and Robert J. Fontana Introduction 468Epidemiology 468Complications of DILI 469Classification of hepatotoxicity 469Drug metabolism and pharmacokinetics 469Hepatic drug metabolism 471Molecular mechanisms in drug?induced liver injury 472Non?genetic risk factors for DILI 473Diagnosis of DILI 474Medical management 478Pharmacogenetic risk factors 478Potential immunological mechanisms in idiosyncratic DILI 479Liver injury from specific drugs 479References 48625 Alcohol and the Liver 494Stephen Stewart and Ewan Forrest Introduction 494Alcohol metabolism 494Pathogenesis 496Susceptibility 497Histological features 498Clinical features 501Clinical syndromes 503Prognosis 504Treatment 506Conclusions 507References 50826 Iron Overload States 511Paul Adams and Heinz Zoller Normal iron physiology 511Iron overload and liver damage  515Genetic haemochromatosis 516Other iron storage diseases 521References 52327 Wilson Disease 526Eve A. Roberts and Karl Heinz WeissMolecular genetics: pathogenesis 527Pathology 528Clinical picture 529Laboratory tests 532Genetic strategies 533Diagnostic difficulties 533Treatment 533Prognosis 536Non-Wilsonian copper-related cirrhosis 536References 53628 Non?Alcoholic Fatty Liver Disease 540Timothy Hardy and Christopher P. Day Introduction 540Further definitions, terminology, and diagnosis 541Liver biopsy, classification of NAFLD, and non? invasive markers of NASH and fibrosis 541 Clinical features 543Laboratory testing 544Epidemiology 545Ethnic variation in NAFLD 545Pathogenesis of NASH 545Natural history of NAFLD 550NAFLD and hepatocellular carcinoma (HCC) 551Therapy for non?alcoholic fatty liver disease 552Other forms of NAFLD 554References 55529 Nutrition and Chronic Liver Disease 561Manuela MerliIntroduction 561Epidemiology and general characteristics 562Causes of malnutrition 562Consequences of malnutrition 564Diagnosis and assessment 565Treatment and management 568References 57030 Pregnancy and the Liver 572Rachel H. Westbrook and Catherine WilliamsonIntroduction 572Normal physiology in pregnancy 572Pregnancy?related liver diseases 573Pre?existing liver diseases and pregnancy 578Liver transplantation and pregnancy 580Liver disease coincidentallyarising with pregnancy 581Conclusion 582References 58231 The Liver in the Neonate, in Infancy, and Childhood 588Susan M. Siew and Deirdre A. KellyInvestigation of liver disease in children 588Neonatal jaundice 589Neonatal unconjugated hyperbilirubinaemia 589Neonatal liver disease (conjugated hyperbilirubinaemia) 591Neonatal hepatitis 594Inherited disease in the neonate 596Genetic cholestatic syndromes 598Structural abnormalities: biliary atresia and choledochal cyst 600Acute liver failure in infancy 602Liver disease in older children 605Metabolic disease in older children 607Cirrhosis and portal hypertension 613Liver transplantation 613Tumours of the liver 614References 61532 The Liver in Systemic Diseases 622James S. Dooley and Christopher McNamara Collagen?vascular and autoimmune disorders 622Hepatic granulomas 624Sarcoidosis 626The liver in endocrine disorders 628Amyloidosis 629Porphyrias 632The liver in haemolytic anaemias 634The liver in myelo? and lymphoproliferative disease 638Bone marrow transplantation 639Lymphoma 640Extramedullary haemopoiesis 642Rare haematological disorders that may involve the liver 643Lipid storage diseases  643Non?metastatic complications of malignancy 646References 64633 The Liver in Infections 652Sanjay Bhagani and Ian Cropley Introduction 652Jaundice of infections 652Pyogenic liver abscess 652Hepatic amoebiasis 655Tuberculosis of the liver 657Hepatic actinomycosis 659Syphilis of the liver  659Perihepatitis 660Leptospirosis 660Relapsing fever 663Lyme disease 663Rickettsial infections 663Fungal infections 664Schistosomiasis (bilharzia) 665Malaria 667Kala?azar (visceral leishmaniasis) 668Echinococcosis (hydatid disease) 668Ascariasis 673Strongyloides stercoralis 674Trichinosis 674Toxocara canis (visceral larva migrans) 674Liver flukes  675References 67634 Imaging of the Liver and Diagnostic Approach of Space?Occupying Lesions 682Neil H. Davies and Dominic YuUltrasound 682Computed tomography 683Magnetic resonance imaging 685Radioisotope scanning 688Positron emission tomography 691MR spectroscopy 691Conclusions and choice of imaging technique 691References 69135 Benign Liver Tumours 693Ian R. WanlessDiagnosis of focal liver lesions 693Hepatocellular lesions 693Biliary and cystic lesions 699Mesenchymal tumours 700References 70136 Primary Malignant Neoplasms of the Liver 705Adam Doyle and Morris Sherman Hepatocellular carcinoma 705Intrahepatic cholangiocarcinoma 718Other malignant neoplasms of the liver 721Other sarcomas 722References 72237 Hepatic Transplantation 730Lindsay Y. King and Carl L. BergSelection of patients 730Candidates 732Absolute and relative contraindications 737General preparation of the patient 738Donor selection and operation 738The recipient operation 739Immunosuppression 742Postoperative course 743Post?transplantation complications 744Conclusion 751References 75238 Hepatic Transplantation and HBV, HCV, and HIV Infections 758Norah A. TerraultIntroduction 758Hepatitis B and liver transplantation 759Hepatitis C and liver transplantation 763HIV and liver transplantation 770References 773Index 781