Sherlock's Diseases of the Liver and Biliary System

Name: Sherlock's Diseases of the Liver and Biliary System
Brand: Wiley-Blackwell
Price: 162.99 EUR
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James S. Dooley Anna S. Lok Guadalupe Garcia-Tsao Massimo Pinzani(Herausgeber*in)

Wiley-Blackwell (Verlag)

13. Auflage

Erschienen am 8. Juni 2018

832 Seiten

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Beschreibung

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 12th 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.

Weitere Details

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Inhalt

Intro
Title Page
Copyright Page
Contents
List of Contributors
Preface to the Thirteenth Edition
Prefaceto the First Edition
Chapter 1 Anatomy and Function
Development of the liver and bile ducts
Anatomy of the liver
Functional liver anatomy: sectors and segments
Anatomical abnormalities of the liver
Anatomy of the biliary tract (Fig. 1.6)
Surface marking (Fig. 1.7, Fig. 1.8)
Methods of examination
Microanatomy of the liver
Hepatic ultrastructure (electron microscopy) and organelle functions
Hepatocytes (Fig. 1.14, Fig. 1.15, Fig. 1.16, Fig. 1.17)
Sinusoidal cells
Bile duct epithelial cells
Functional heterogeneity of the liver (Fig. 1.20)
Dynamics of the hepatic microenvironment in physiology and disease (Fig. 1.21)
Hepatocyte death and regeneration (Fig. 1.22)
Cell death
Regeneration
References
Chapter 2 Liver Function in Health and Disease: Clinical Application of Liver Tests
Bilirubin metabolism (see Chapter 13)
Laboratory measurement of bilirubin
Bile acids
Bile acid metabolism
Bile acids in health and disease
Measurement of serum bile acids
Changes in serum bile acids
Lipid and lipoprotein metabolism
Lipids
Lipoproteins
Changes in liver disease
Amino acid metabolism
Plasma proteins
Carbohydrate metabolism
Markers of hepatocellular injury: the serum transaminases
Elevated transaminases
Markers of cholestasis: alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT)
Elevated ALP and/or GGT
Haematology in liver disease
Anaemia
Effects of ageing on the liver
References
Chapter 3 Biopsy of the Liver
Selection and preparation of the patient
Informed consent
Techniques
After care
Number of passes
Risks and complications
Pleurisy and perihepatitis
Haemorrhage (Fig. 3.5)
Intrahepatic haematomas
Haemobilia
Arteriovenous fistula
Biliary peritonitis
Puncture of other organs
Infection
Carcinoid crisis
Sampling variability
Naked-eye appearances
Preparation of the specimen
Interpretation: a stepwise diagnostic approach
Indications (Table 3.4) [5]
Special methods [34]
References
Chapter 4 Coagulation in Cirrhosis
Introduction
Normal coagulation pathways: a hepatologist's perspective
The coagulation system in cirrhosis
Bleeding and thrombosis in cirrhosis
Bleeding
Prophylaxis and therapy for bleeding in cirrhosis
Thrombosis
Medical prophylaxis and therapy for thrombosis in cirrhosis
Clinical laboratory tests of the coagulation system in cirrhosis
Conclusion
References
Chapter 5 Acute Liver Failure
Definition
Epidemiology and aetiologies (Fig. 5.1, Table 5.1)
Viral hepatitis
Paracetamol (acetaminophen)
Other aetiologies
Clinical features
Distinction from chronic liver disease
Initial investigations
Laboratory (Table 5.3)
Abdominal imaging and liver biopsy
Complications and management of acute liver failure
Hepatic encephalopathy
Cerebral oedema and intracranial hypertension
Coagulopathy
Metabolic derangements
Infection
Renal
Haemodynamic changes
Gastrointestinal bleeding
Pulmonary complications
Acute pancreatitis
Specific therapies
Paracetamol (acetaminophen) hepatotoxicity
Mushroom poisoning
Hepatitis B
Herpes simplex virus
Autoimmune hepatitis
Pregnancy
Wilson disease
Prognosis
Liver transplantation (Chapter 37)
Indications
Contraindications
Intraoperative and postoperative care
Outcomes
Other modes of liver support
Auxiliary liver transplantation
Conclusion
References
Chapter 6 Hepatic Fibrogenesis
Introduction
Natural history of hepatic fibrosis
Cellular and molecular features of hepatic fibrosis (Fig. 6.2)
Cellular anatomy of sinusoids
Extracellular matrix composition in normal liver and hepatic scar tissue: functional and biologic roles
Cellular sources of extracellular matrix in normal and fibrotic liver
Stellate cell activation: a central feature of hepatic fibrosis
Stellate cell activation
Local interactions influencing fibrogenesis
Matrix production (fibrogenesis) and degradation (fibrinolysis) (Fig. 6.5)
Clinical aspects of hepatic fibrosis
Emerging antifibrotic targets and strategies
References
Chapter 7 Non-invasive Assessment of Fibrosis and Cirrhosis
Introduction
The use of invasive and non-invasive tests
Liver biopsy
Hepatic venous pressure gradient measurement
Non-invasive testing
Non-invasive tests: specifics
Serum markers of fibrosis
Liver stiffness measurement
Spleen stiffness measurement
Molecular imaging
Conclusions
References
Chapter 8 Hepatic Cirrhosis
Definition
Causes of cirrhosis
Cirrhosis and cofactors (Fig. 8.2)
Anatomical diagnosis
Reversible cirrhosis
Clinical cirrhosis: compensated versus decompensated
Compensated cirrhosis
Decompensated cirrhosis
Prognosis (Child-Pugh score, MELD, UKELD)
Clinical and pathological associations
Vasodilation and hyperdynamic circulation
Cardiopulmonary conditions
Gastrointestinal
Renal
Foetor hepaticus
Skin changes
Endocrine changes
Muscle cramps
Drug metabolism
Laboratory findings
Infections
Management
General
Specific
Precipitating factors
Nutrition
Surgical procedures [51]
Acute-on-chronic liver failure
Diagnostic criteria for ACLF
Prognosis and course of ACLF
Pathophysiological basis of ACLF
The organs in ACLF
The PIRO concept and approach to treatment
Declaration of interest
References
Chapter 9 Ascites
Mechanisms of ascites formation
Sinusoidal hypertension
Sodium retention
Vasodilation theory (Fig. 9.3)
Circulation of ascites
Summary
Clinical features
Symptoms
Examination
Associated conditions
Ascitic fluid
Radiological features
Differential diagnosis
Spontaneous bacterial peritonitis (Table 9.3)
Prognosis
Treatment
Prophylaxis
Treatment of cirrhotic ascites
Sodium restriction/diet
Diuretics
Complications
Follow-up advice
Therapeutic (large-volume) abdominal paracentesis
Summary (Table 9.6)
Hyponatraemia
Mechanism
Treatment
Summary (Table 9.7)
Refractory ascites
Treatment (Table 9.8)
Therapeutic paracentesis
Transjugular intrahepatic portosystemic shunt (TIPS)
Peritoneovenous (LeVeen) shunt
Hepatorenal syndrome
Diagnostic criteria (Table 9.9)
Mechanism
Clinical features
Differential diagnosis (Table 9.10)
Prevention
Treatment
Summary
Prognosis
References
Chapter 10 Hepatic Encephalopathy in Patients with Cirrhosis
Clinical Features [1-3]
Classification [2,4]
Episodic/recurrent hepatic encephalopathy
Persistent hepatic encephalopathy
Minimal hepatic encephalopathy
Prevalence and consequences
Diagnosis [30]
Neurological examination including mental state assessment [31]
Psychometric performance [30,34]
Electroencephalography [30,37]
Critical Flicker Fusion Frequency
Inhibitory Control Test
Scan test
Stroop test
Cerebral magnetic resonance [50,51]
Blood ammonia
Cerebrospinal fluid
Neuropathology [1,56]
Choice of diagnostic variables [2,4,30]
Diagnostic comorbidities, confounders, and alternatives
Pathogenesis
Key concepts and contributors
A unified hypothesis (Fig. 10.12)
Management [4]
General considerations
Specific measures
Potential future therapeutic options
Prevention [4]
References
Chapter 11 Portal Hypertension in Cirrhosis
Introduction
The syndrome: definition, classification, and aetiology
Relevance
Anatomy of the portal system and formation of portosystemic collaterals
Pathology of portal hypertension
Pathophysiology and rational basis of therapy
Increased hepatic vascular resistance
Formation of collaterals and varices
Increased splanchnic blood flow and hyperkinetic circulation
Evaluation and diagnosis
Measurement of portal pressure
Non-invasive assessment and techniques
Endoscopy
Natural history and prognosis
Management
Therapeutic tools
Treatment of portal hypertension according to clinical scenarios
Compensated patients without varices
Compensated patients with varices
Acute bleeding episode
Treatment of patients who fail standard therapy ('rescue therapy')
Acute bleeding from gastric varices
Prevention of recurrent variceal bleeding (treatment of patients who survived an episode of variceal bleeding)
Secondary prophylaxis in special situations
References
Chapter 12 Vascular Disorders of the Liver and Extrahepatic Portal Hypertension
Hepatic artery occlusion
Causes
Pathophysiology
Diagnosis
Management
Aneurysms of the hepatic artery
Aetiology
Manifestations
Diagnosis
Treatment
Hepatic arterioportal fistula
Aetiology
Manifestations
Diagnosis
Management
Hepatic vascular malformations in hereditary haemorrhagic telangiectasia
Aetiology
Pathophysiology
Manifestations
Diagnosis
Treatment
Congenital portosystemic shunts - Abernethy malformation
Causes
Pathophysiology
Manifestations
Diagnosis
Treatment
Budd-Chiari syndrome - hepatic venous outflow tract obstruction
Pathophysiology
Aetiology
Manifestations
Diagnosis
Treatment
Outcome and prognosis
Extrahepatic portal vein obstruction - portal vein thrombosis and portal cavernoma in the absence of cirrhosis
Aetiology
Pathophysiology
Manifestations
Diagnosis
Treatment
Outcome and prognosis
Portal vein thrombosis in patients with cirrhosis
Aetiology
Pathophysiology
Manifestations
Diagnosis
Treatment
Idiopathic non-cirrhotic intrahepatic portal hypertension
Aetiology and pathogenesis
Manifestations
Diagnosis - liver biopsy
Treatment
Outcome and prognosis
Hypoxic hepatitis
Aetiology
Pathophysiology
Manifestations
Diagnosis
Treatment
Outcome and prognosis
Congestive cardiac hepatopathy
Aetiology
Pathophysiology
Manifestations
Diagnosis
Treatment and prognosis
Non-obstructive sinusoidal dilation (NOSD) and peliosis
Causes
Pathophysiology
Manifestations
Diagnosis
Treatment and prognosis
References
Chapter 13 Jaundice and Cholestasis
Introduction
Jaundice
Cholestasis
Classification of jaundice
Mechanics of bile formation
Secretion of bile
Sinusoidal uptake
Bile salt synthesis
Canalicular secretion
Bile flow
Bile ductules
Enterohepatic circulation
Syndrome of cholestasis
Causes of isolated hyperbilirubinaemia
Bilirubin overproduction
Disorders of bilirubin conjugation
Disorders of hepatic uptake
Disorders of hepatic secretion
Causes of cholestatic and hepatocellular jaundice
Inherited causes of cholestasis
Bile duct and hepatocellular diseases
Extrahepatic cholestasis
Consequences of cholestasis and their management
Vitamin deficiency (Table 13.2)
Pruritus (Table 13.3)
Fatigue
Xanthomas
Hepatic osteodystrophy
Hepatocellular failure
Extrahepatic effects
Biochemistry
Investigation of the jaundiced patient
History
Physical examination (Fig. 13.6)
Laboratory investigation
Summary
Decisions to be made in the jaundiced patient
Management of cholestatic disorders
References
Chapter 14 Gallstones and Benign Biliary Disease
Introduction
Imaging the gallbladder and biliary tract
Gallbladder imaging
Bile duct imaging
Gallstones
Introduction
Gallstone formation
Gallbladder function
Biliary sludge
Epidemiology of gallstones [16,26]
Factors in gallstone formation
Pigment gallstones [57]
Symptoms and complications of gallstones
Diagnosis of gallstones
Treatment of gallstones
Symptomatic gallstones
Cholecystectomy
The risks
Surgical technique
Complicated acute gallbladder disease
Empyema of the gallbladder (gallbladder abscess)
Emphysematous cholecystitis
Acute acalculous cholecystitis
Percutaneous cholecystostomy
Asymptomatic gallbladder stones
Non-surgical treatment of gallstones in the gallbladder
Summary of gallstone management
Common bile duct stones
Background
Presentation of common bile duct stones
Assessing the severity of acute cholangitis
Management
Method of biliary decompression
Common duct stones without cholangitis
Acute gallstone pancreatitis
Large common duct stones
Mirizzi syndrome
Intrahepatic gallstones
Haemobilia
Functional gallbladder and sphincter of Oddi disorders
Acalculous biliary pain and functional gallbladder disorder
Post-cholecystectomy syndromes
Sphincter of Oddi and suspected functional biliary sphincter disorders
Other gallbladder pathologies
Gallbladder polyps
Identifying 'high-risk' polyps
Adenomyomatosis
Cholesterolosis of the gallbladder
Xanthogranulomatous cholecystitis
Porcelain gallbladder
Typhoid, HIV, and vasculitis-related cholecystitis
Congenital gallbladder anomalies (see Chapter 16)
Other complications of acute cholecystitis
Relationships to malignant change
Association between gallstones and cancer of the gallbladder
Association between gallstones, cholecystectomy and others cancers
Benign biliary strictures
Epidemiology and pathogenesis (Table 14.6)
Symptoms and complications
Diagnosis
Treatment
Intraductal papillary neoplasms
Summary
Anastomotic strictures following biliary surgery
Clinical features
Investigations
Treatment
IgG4-related sclerosing cholangitis
Chronic pancreatitis
Management
References
Chapter 15 Malignant Biliary Diseases
Carcinoma of the gallbladder
Pathology
Clinical features
Imaging
Treatment
Prognosis
Other gallbladder tumours
Carcinoma of the bile duct (cholangiocarcinoma)
Associations
Pathology
Molecular changes
Clinical presentation
Imaging
Endoscopic techniques
Staging and preparation for surgery
Treatment
Prognosis
Other biliary malignancies
Metastases at the hilum
Ampullary and periampullary carcinomas
Genetics and environmental predisposition
Spread of the tumour
Clinical features
Investigations
Differential diagnosis
Diagnosis and staging
Treatment
Prognosis
Conclusion
References
Chapter 16 Fibropolycystic Liver Diseases and Congenital Biliary Abnormalities
Overview
Polycystic liver disease
Molecular genetics
Pathogenesis
Pathology
Clinical features
Differential diagnosis
Diagnostic criteria
Complications
Prognosis and treatment
Fibropolycystic diseases
Autosomal recessive polycystic kidney disease
Clinical features
Hepatic pathology
Imaging
Treatment
Congenital hepatic fibrosis
Clinical features
Investigations
Treatment and prognosis
Caroli disease [58]
Clinical features
Imaging
Treatment and prognosis
Microhamartomas (von Meyenberg complexes)
Choledochal cysts
Pathogenesis
Classification
Clinical features
Imaging
Treatment
Solitary non-parasitic liver cyst
Other cysts
Carcinoma secondary to cystic disease
Congenital anomalies of the biliary tract
Absence of the gallbladder [86]
Double gallbladder
Accessory bile ducts
Left-sided gallbladder
Rokitansky-Aschoff sinuses of the gallbladder
Folded gallbladder
Diverticula of the gallbladder and ducts
Intrahepatic gallbladder
Congenital adhesions to the gallbladder
Floating gallbladder and torsion of the gallbladder
Anomalies of the cystic duct and cystic artery
References
Chapter 17 Primary Biliary Cholangitis
Clinical features
Presentation (Table 17.1)
Diagnosis
Biochemical tests
Autoantibodies
Liver biopsy
Differential diagnosis
Natural history of disease
Associated diseases
Epidemiology
Aetiology and pathogenesis
Aetiology
Pathogenesis
Management
Treatment aimed at improving survival
Symptomatic treatment
Prognosis
References
Chapter 18 Sclerosing Cholangitis
Introduction
Primary sclerosing cholangitis
Epidemiology
Pathogenesis
Clinical features of PSC
Diagnosis
Special populations
Prognosis
Medical treatment
Role of endoscopy
Dysplasia and cancer
Liver transplantation
Secondary sclerosing cholangitis
Ischaemia- and trauma-associated sclerosing cholangitis
Infectious cholangitis
Opportunistic infections
Drug-related sclerosing cholangitis
Sclerosing cholangitis in systemic inflammatory diseases
References
Chapter 19 Autoimmune Hepatitis and Overlap Syndromes
Introduction
Historical perspective
Disease overview
Clinical manifestations
Serology
Epidemiology
Natural history
Biological determinants of disease
Immunobiology
Genetics and AIH
HLA loci and AIH
Non-HLA loci and AIH
Environmental and drug triggers
Viral triggers
Disease presentation
General features
Patterns of presentation
Symptoms and signs
Associated autoimmune diseases
Laboratory features
Liver biochemistry and immunoglobulins
Serology
Antinuclear antibodies
Smooth muscle antibodies
Microsomal antibodies
Soluble liver and pancreas antigen
Mitochondrial antibodies
Imaging
Liver biopsy and histological features
Histological features
Simplified grading
Biliary lesions
Fibrosis and necroinflammatory activity
Surrogate markers of fibrosis
Differential diagnosis
Drug injury
Viral hepatitis
Metabolic
Diagnostic dilemmas
Autoantibody-negative patients
Viral hepatitis
Coeliac disease
Making a diagnosis in practice
International Autoimmune Hepatitis Group (IAIHG)
2008 simplified scoring system
Management strategies
Who and how to treat AIH?
Treatment approaches
Corticosteroid dose
Treatment response
Maintenance of remission
Pretreatment and on-treatment considerations
Treatment challenges and alternative agents
Pregnancy and autoimmune hepatitis
The elderly and autoimmune hepatitis
Childhood-onset autoimmune hepatitis
Autoimmune hepatitis and liver transplantation
Outcomes and disease recurrence
De novo AIH (alloimmune hepatitis)
Overlap syndromes
Definitions
AIH-PBC 'overlap'
Primary sclerosing cholangitis-autoimmune hepatitis 'overlap' syndrome
Autoimmune sclerosing cholangitis (ASC)
IgG4-associated AIH
Conclusion
References
Chapter 20 Enterically Transmitted Viral Hepatitis: Hepatitis A and Hepatitis E
General features of enterically transmitted viral hepatitis
Pathology
Clinical types
Investigations
Differential diagnosis
Prognosis
Prevention
Treatment of the acute attack
Hepatitis A virus
Epidemiology
Clinical course
Prognosis
Prevention
Immune serum globulin (ISG) prophylaxis
Hepatitis A vaccines
Hepatitis E virus
Clinical features
Diagnostic tests
Prevention
Treatment of chronic HEV
Acknowledgement
References
Chapter 21 Hepatitis B
Introduction
Hepatitis B?virus
Replication cycle
HBV?serotypes and genotypes
HBV?variants
Immune response and mechanisms of hepatic injury
Epidemiology
Modes of transmission
Prevention
Hepatitis B?immune globulin
HBV?vaccines
Diagnosis
Serological diagnosis
Liver biopsy
Non-invasive assessment of liver fibrosis
Clinical manifestations
Extrahepatic manifestations
Natural history
Acute HBV?infection
Chronic HBV?infection
Clinical outcomes
Treatment
Goals of treatment
Safety and efficacy of approved treatments
Indications for treatment (Table 21.11)
What should be the initial therapy?
Dose regimen
Management of patients with treatment failure
HBV?and HCV?coinfection (also see Chapter 23)
HBV?and HDV?coinfection
HBV?and HIV?coinfection
References
Chapter 22 Hepatitis D
History
Hepatitis D virus (Table 22.1)
HDV genotypes
Epidemiology
Transmission
Geographic distribution
Changing epidemiology
Pathogenesis
Modes of infection and clinical course
Acute hepatitis D
Chronic hepatitis D
Hepatic histology
Detection of HDAg in the liver
Course and prognosis
HDV and carcinogenesis
Diagnosis
Acute hepatitis D
Chronic hepatitis D
Detection of HDV RNA
Treatment
Acute hepatitis D
Chronic hepatitis D
Perspectives
Prevention
References
Chapter 23 Hepatitis C
Introduction
Epidemiology
Virology
Pathology and pathogenesis
Diagnostic tests for hepatitis C
Anti-HCV
Confirmatory HCV testing
Genotyping
Acute hepatitis C
Pathology
Management
Chronic hepatitis C
Diagnosis
Natural history
Extrahepatic manifestations
Pathology
Management
Prevention and elimination
References
Chapter 24 Drug-Induced Liver Injury
Introduction
Epidemiology
Complications of?DILI
Classification of hepatotoxicity
Drug metabolism and pharmacokinetics
Hepatic drug metabolism
Phase 1
Phase 2
Phase 3
The cytochrome P450 system
Molecular mechanisms in drug-induced liver injury
Non-genetic risk factors for?DILI
Age
Gender
Daily dose
Alcohol use
Other risk factors
Diagnosis of?DILI
Laboratory profile at?DILI?onset
Other laboratory tests
Liver histology
Assessing causality
The adjudication process
Medical management
Pharmacogenetic risk factors
Potential immunological mechanisms in idiosyncratic?DILI
Liver injury from specific drugs
Non-steroidal anti-inflammatory drugs (NSAIDs)
Antibacterials
Macrolides
Quinolones
Tetracyclines
Antituberculosis agents
Drugs used for treatment of inflammatory bowel disease
Chemotherapeutic agents
Cardiovascular drugs
Central nervous system drugs
Hypolipidaemics
Herbal and dietary supplement (HDS) products
References
Chapter 25 Alcohol and the Liver
Introduction
Alcohol metabolism
Absorption and distribution
Alcohol to acetaldehyde
Acetaldehyde to acetate
Pathogenesis
Pathogenesis of steatosis
Oxidative stress and lipid peroxidation
Acetaldehyde
Endotoxin and cytokines
Immunological liver damage
Alcohol and fibrosis
Alcohol and cancer
Susceptibility
Environmental factors
Genetic factors
Histological features
Fatty liver
Alcoholic hepatitis
Cirrhosis
Clinical features
History, examination, and early recognition
Laboratory investigations
Imaging
Liver biopsy
Clinical syndromes
Fatty liver
Acute alcoholic hepatitis
Cirrhosis
Extrahepatic features
Prognosis
Treatment
Harmful drinking and dependence
Acute alcohol-related hepatitis (Table 25.6)
Cirrhosis
Transplantation
Conclusions
References
Chapter 26 Iron Overload States
Normal iron physiology
Absorption
Regulation
Circulating iron
Storage of iron
Iron overload and liver damage
Genetic haemochromatosis
Molecular genetics
Heterozygotes
Pathology
Relation to alcoholism
Clinical features
Special investigations
Differential diagnosis
Prognosis
Treatment
Transplantation
Screening for early haemochromatosis in relatives
Other iron storage diseases (see Tables 26.1 and 26.2)
Non-HFE-related inherited iron overload
Dysmetabolic syndrome
Erythropoietic siderosis
Neonatal haemochromatosis/Gestational alloimmune liver disease (see also Chapter 31)
African iron overload (Bantu siderosis)
Porphyria cutanea tarda (see Chapter 32)
References
Chapter 27 Wilson Disease
Molecular genetics: pathogenesis
Pathology
Liver
Electron microscopy
Other organs
Clinical picture
Hepatic forms
Neuropsychiatric forms
Renal changes
Other changes
Laboratory tests
Liver biopsy
Imaging
Genetic strategies
Diagnostic difficulties
Detection of symptom-free affected individuals
Treatment (Table 27.1)
Prognosis
Non-Wilsonian copper-related cirrhosis
References
Chapter 28 Non-Alcoholic Fatty Liver Disease
Introduction
Further definitions, terminology, and diagnosis
Liver biopsy, classification of NAFLD, and non-invasive markers of NASH and fibrosis
Clinical features
Laboratory testing
Epidemiology
Ethnic variation in NAFLD
Pathogenesis of NASH
Mechanisms of steatosis
Regulation of lipid synthesis
Steatosis in humans
Lipid composition in NAFLD
Lipid peroxidation and mitochondrial dysfunction in NASH
Endoplastic reticulum stress
Hepatocyte injury and cell death in NASH
Fibrosis and disease progression
Role of adipose tissue in NAFLD pathogenesis: adipokines
Modifiers of NAFLD/NASH
Genetics
The microbiome
Natural history of NAFLD (Fig 28.9)
Fibrosis is the key histological determinant of prognosis
Paired-biopsy studies
NAFLD and hepatocellular carcinoma (HCC)
Therapy for non-alcoholic fatty liver disease
Exercise, calorie reduction, and weight loss
Dietary composition
Pharmacological intervention
Agents with potential benefit.
Agents with no or unclear benefit
Emerging therapies
Bariatric surgery
Liver transplantation
Other forms of NAFLD
References
Chapter 29 Nutrition and Chronic Liver Disease
Introduction
Epidemiology and general characteristics
Causes of malnutrition
Consequences of malnutrition
Diagnosis and assessment
Treatment and management
References
Chapter 30 Pregnancy and the Liver
Introduction
Normal physiology in pregnancy
Pregnancy-related liver diseases
Hyperemesis gravidarum
Intrahepatic cholestasis of pregnancy
Pre-eclampsia/eclampsia/HELLP syndrome
Acute fatty liver of pregnancy
Hepatic rupture, infarction, and haematoma
Pre-existing liver diseases and pregnancy
Cirrhosis and portal hypertension
(Chronic) hepatitis B virus infection and pregnancy
Chronic hepatitis C infection and pregnancy
Autoimmune hepatitis and pregnancy
Cholestatic liver disease and pregnancy
Liver transplantation and pregnancy
Liver disease coincidentally arising with pregnancy
Acute viral infections and pregnancy
Pregnancy and thrombosis
Conclusion
References
Chapter 31 The Liver in the Neonate, in Infancy, and Childhood
Investigation of liver disease in children
Biochemical tests
Bile acid metabolism
Radiology
Liver size
Circulatory factors and hepatic necrosis
Neonatal jaundice
Neonatal unconjugated hyperbilirubinaemia (Tables 31.2, 31.3)
Physiological jaundice
Breast-milk jaundice
Haemolytic disease of the newborn
Congenital haemolytic disorders
Crigler-Najjar syndrome type I and II (see also Chapter 13)
Perinatal complications
Upper gastrointestinal obstruction
Neonatal liver disease (conjugated hyperbilirubinaemia)
Clinical features and diagnosis (Table 31.4) [15]
Investigations
Neonatal hepatitis
Intrauterine infection
Endocrine disorders
Chromosomal disorders
Idiopathic neonatal hepatitis
Neonatal hepatitis in preterm infants
Prolonged parenteral nutrition
Inherited disease in the neonate
a1-Antitrypsin deficiency
Cystic fibrosis
Alagille syndrome (arteriohepatic dysplasia) [36]
Genetic cholestatic syndromes
Progressive familial intrahepatic cholestasis type 1
Progressive familial intrahepatic cholestasis type 2
Benign recurrent intrahepatic cholestasis
Progressive familial intrahepatic cholestasis type 3
Progressive familial intrahepatic cholestasis type 4
Treatment and outcome
Abnormal bile acid synthesis
Dubin-Johnson syndrome (see also Chapter 13)
Symptomatic treatment of cholestatic syndromes
Other causes of cholestatic jaundice
Structural abnormalities: biliary atresia and choledochal cyst
Biliary atresia
Choledochal cyst (see also Chapter 16)
Spontaneous perforation of the bile ducts
Inspissated bile syndrome and cholelithiasis
Acute liver failure in infancy
Galactosaemia
Gestational alloimmune liver disease (GALD)
Disorders of mitochondrial energy metabolism
Tyrosinaemia type I
Liver disease in older children
Acute viral hepatitis
Chronic liver disease
Autoimmune hepatitis [115]
Non-alcoholic fatty liver disease (NAFLD) (see also Chapter 28)
Congenital hepatic fibrosis (see Chapter 16)
Drug-induced liver disease
Metabolic disease in older children
Wilson disease (see Chapter 27)
Cystic fibrosis liver disease
Glycogen storage disease [129,130]
Hereditary fructose intolerance (HFI)
Fructose-1,6-bisphosphatase deficiency
Glutaric aciduria type II
Lysosomal storage disorders (see also Chapter 32)
Familial hypercholesterolaemia
Cirrhosis and portal hypertension
Liver transplantation
Indications
Surgical approach, immunosuppressive regimens, and outcome
Tumours of the liver (see also Chapters 35 and 36)
Diagnosis
Hamartomas
Mesenchymal hamartoma
Malignant mesenchymoma (undifferentiated sarcoma)
Adenomas
Hepatoblastoma
Hepatocellular carcinoma
Infantile haemangioendothelioma
References
Chapter 32 The Liver in Systemic Diseases
Collagen-vascular and autoimmune disorders
Systemic lupus erythematosus
Anticardiolipin syndrome
Rheumatoid arthritis and Felty syndrome
Sjögren syndrome
Scleroderma
Polyarteritis nodosa and other vasculitides
Coeliac disease
Hepatic granulomas
Pathogenesis
Clinical syndrome of hepatic granulomas
Specific causes and syndromes
Sarcoidosis
Hepatic histology
Clinical features
Treatment
Granulomatous drug reactions (see Chapter 24)
Non-specific reticuloendothelial proliferations: 'reactive hepatitis'
The liver in endocrine disorders
Diabetes mellitus
Thyroid
Adrenal
Liver and growth hormone
Amyloidosis
Clinical features
Hepatic involvement
Diagnostic approach
Prognosis
Treatment
Porphyrias
Hepatic porphyrias
Erythropoietic porphyrias
Secondary coproporphyrias
The liver in haemolytic anaemias
Bilirubin handling (see also Chapter 13)
Pathological changes
Clinical features
Differential diagnosis
Hereditary spherocytosis [86]
Enzyme defects
Sickle cell disease [88,89]
Thalassaemia
Paroxysmal nocturnal haemoglobinuria [98]
Acquired haemolytic anaemia
Haemolytic disease of the newborn
The liver in myelo- and lymphoproliferative disease [102]
Hepatic histology
Bone marrow transplantation
Lymphoma
Diagnosis of hepatic involvement
Jaundice in lymphoma (Table 32.8)
Primary hepatic lymphoma
Plasma cell myeloma
Extramedullary haemopoiesis
Microscopic features
Rare haematological disorders that may involve the liver
Lipid storage diseases
Lysosomal acid lipase deficiency (LAL-D) [131,132]
Gaucher disease [134]
Niemann-Pick diseases [143]
Non-metastatic complications of malignancy
References
Chapter 33 The Liver in Infections
Introduction
Jaundice of infections
Bacteraemia and septic shock
Pyogenic liver abscess
Aetiology
Infecting agents
Pathology
Clinical features
Localization of the abscess
Treatment
Prognosis
Hepatic amoebiasis
Epidemiology
Clinical features
Diagnostic tests
Biochemical tests
Radiological features
Diagnostic criteria
Complications
Treatment
Tuberculosis of the liver
Clinical features
Biochemical tests
Diagnosis
The effect on the liver of tuberculosis elsewhere
Other mycobacteria
Hepatic actinomycosis
Clinical features
Diagnosis
Treatment
Syphilis of the liver
Congenital
Secondary
Tertiary
Treatment
Perihepatitis
Leptospirosis
Weil disease
Other types of leptospirosis
Relapsing fever
Clinical features [78]
Diagnosis
Treatment
Lyme disease
Rickettsial infections
Q fever
Rocky mountain spotted fever
Infection with Bartonella henselae
Fungal infections
Opportunistic fungal infections
Pathogenic/endemic fungal infections
Schistosomiasis (bilharzia)
Pathogenesis
Clinical features
Diagnostic tests
Portal hypertension
Biochemical changes
Disease association
Treatment
Disease control
Bleeding oesophageal varices
Malaria [113]
Pathology
Clinical features
Kala-azar (visceral leishmaniasis)
Echinococcosis (hydatid disease)
Biology (Fig. 33.14)
Development of the hepatic cyst (Fig. 33.15)
Endemic regions
Clinical features
Complications
Diagnosis
Prognosis
Prevention
Rupture into the peritoneal cavity
Echinococcus multilocularis (alveolar echinococcosis)
Ascariasis
Strongyloides stercoralis
Trichinosis
Toxocara canis (visceral larva migrans)
Liver flukes
Clonorchiasis and opisthorchiasis
Fascioliasis
Recurrent pyogenic cholangitis
References
Chapter 34 Imaging of the Liver and Diagnostic Approach of Space-Occupying Lesions
Ultrasound
Computed tomography
Magnetic resonance imaging
Radioisotope scanning
Positron emission tomography
MR spectroscopy
Conclusions and choice of imaging technique
References
Chapter 35 Benign Liver Tumours
Diagnosis of focal liver lesions
Hepatocellular lesions
Hepatocellular adenoma
Dysplastic nodule
Focal nodular hyperplasia
Arterialized regenerative nodule
Nodular regenerative hyperplasia
Focal fatty change and focal fatty sparing
Biliary and cystic lesions
Bile duct adenoma
Mucinous cystic neoplasm (formerly biliary cystadenoma)
Intraductal papillary neoplasm (formerly biliary papillomatosis)
Biliary adenofibroma
Bile duct hamartoma (von Meyenburg complex)
Simple cyst and polycystic disease
Ciliated hepatic foregut cyst
Cystic dilation of peribiliary glands
Tubular adenoma of bile duct or duodenum
Mesenchymal tumours
Cavernous haemangioma
Peliosis hepatis
Angiomyolipoma
Epithelioid haemangioendothelioma
Mesenchymal hamartoma
Other mesenchymal tumours
Regional parenchymal extinction
Inflammatory pseudotumour
References
Chapter 36 Primary Malignant Neoplasms of the Liver
Hepatocellular carcinoma
Epidemiology
Pathology
Pathogenesis
Assessment of HCC risk
Clinical presentation
Surveillance
Diagnosis
Treatment
Intrahepatic cholangiocarcinoma
Epidemiology
Pathogenesis
Clinical presentation and diagnosis
Management
Other malignant neoplasms of the liver
Fibrolamellar hepatocellular carcinoma
Cholangio-hepatocellular carcinoma
Cholangiolar carcinoma
Cholangiolocellular carcinoma
Angiosarcoma
Malignant epithelioid haemangioendothelioma
Other sarcomas
References
Chapter 37 Hepatic Transplantation
Selection of patients (Table 37.1)
Candidates (Table 37.2)
Cirrhosis
Autoimmune chronic hepatitis
Chronic viral hepatitis (Chapter 38)
Alcoholic liver disease
Cholestatic liver disease
Primary metabolic disease
Acute liver failure (Chapter 5)
Malignant disease (Chapter 36)
Budd-Chiari syndrome (Chapter 12)
Absolute and relative contraindications (Table 37.4)
Absolute
Relative (higher risk)
General preparation of the patient
Donor selection and operation
The recipient operation (Fig. 37.3)
Segmental (split) liver transplantation
Living donor liver transplantation
Auxiliary liver transplantation
Domino liver transplantation
Hepatocyte transplantation
Xenotransplantation
Liver transplantation in paediatrics
Immunosuppression
Tolerance
Postoperative course
Post-transplantation complications (Table 37.9)
Technical complications
Vascular complications
Biliary tract complications
Renal failure
Pulmonary complications
Non-specific cholestasis
Rejection
Infections
De novo autoimmune hepatitis
Malignancies
Lymphoproliferative disorders
Drug-related toxicity and interactions
Disease recurrence
Central nervous system toxicity and complications
Bone disease
Metabolic syndrome
Pregnancy after liver transplantation
Immunizations pre and post liver transplant
Conclusion
Acknowledgement
References
Chapter 38 Hepatic Transplantation and HBV, HCV, and HIV Infections
Introduction
Hepatitis B and liver transplantation
Natural history and factors affecting disease recurrence
Treatment of patients with HBV on the waiting list
Preventing HBV recurrence post-transplantation
Donors with positive hepatitis B serology
Management of recurrent HBV in liver transplant recipients
Retransplantation
Hepatitis C and liver transplantation
Natural history and factors associated with severe recurrent disease
Use of anti-HCV-positive donors
Overview of management of HCV infection in transplant recipients
Antiviral therapy for HCV-infected transplant candidates and recipients
Pre-transplant treatment of HCV-infected patients on the waiting list (Table 38.4)
Prophylactic and pre-emptive antiviral therapy (Table 38.4)
Post-transplant antiviral therapy for recurrent disease (Table 38.4)
Retransplantation
HIV and liver transplantation
HBV and HIV coinfected liver transplant recipients
HCV-HIV liver transplant recipients
References
Index
EULA

List of Contributors

Paul Adams MD
Professor of Medicine
Chief of Gastroenterology
University Hospital
University of Western Ontario
London, ON, Canada

Graeme Alexander MA, MD, FRCP
UCL Professor
Institute for Liver and Digestive Health
UCL Medical School
London, UK

Meena B. Bansal MD
Associate Professor of Medicine
Division of Liver Diseases
Icahn School of Medicine at Mount Sinai
New York, NY, USA

Carl L. Berg MD
Professor of Medicine
Medical Director of Abdominal Transplantation
Duke University Health System
Durham, NC, USA

Annalisa Berzigotti MD, PhD
Swiss Liver Center, Hepatology
University Clinic for Visceral Surgery and Medicine Inselspital
University of Bern
Bern, Switzerland

Sanjay Bhagani BSc, MB BCh, FRCP
Consultant Physician and Honorary Senior Lecturer
Department of Infectious Diseases/HIV Medicine
Royal Free Hospital
Research Department of Infection, UCL
London, UK

Kirsten Muri Boberg MD, PhD
Professor of Hepatology
Department of Transplantation Medicine
Oslo University Hospital Rikshospitalet
Oslo, Norway

Jaime Bosch MD, PhD, FRCP, FAASLD
Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clinic, IDIBAPS, University of Barcelona Barcelona, Spain;
CIBERehd, Instituto de Salud Carlos III, Madrid, Spain;
Swiss Liver Center, Hepatology, University Clinic for Visceral Surgery and Medicine, Inselspital, University of Bern
Bern, Switzerland

Rinjal D. Brahmbhatt MD
Division of Gastroenterology and Hepatology
Michigan Medicine, University of Michigan
Ann Arbor, MI, USA

John Bridgewater MA, FRCP, PhD, MB BS
Professor and Consultant in Medical Oncology
University College London Medical School and Cancer Institute
London, UK

Stephen H. Caldwell MD
Professor, Director of Hepatology
Division of Gastroenterology and Hepatology
University of Virginia
Charlottesville, VA, USA

Ian Cropley MA, MB BS, FRCP, DTM&H
Consultant Physician and Honorary Senior Lecturer
Department of Infectious Diseases/HIV Medicine
Royal Free Hospital
London, UK

Brian R. Davidson MB BCh, MD, FRCS (Glas), FRCS (Eng)
Consultant HPB and Liver Transplant Surgeon
HPB and Liver Transplant Unit
Royal Free Hospital, London
Professor of Surgery
Head of HBP and Liver Transplant Research
University College London Medical School, Royal Free Campus,
London, UK

Neil H. Davies MB BS, FRCS, FRCR
Consultant Interventional Radiologist
Department of Radiology
Royal Free Hospital
London, UK

Christopher P. Day FMedSci
Professor of Liver Medicine
Institute of Cellular Medicine, Newcastle University
Newcastle upon Tyne, UK

Frank W. DiPaola MD
Department of Pediatrics
University of Michigan Medical School
Ann Arbor, MI, USA

James S. Dooley MD, FRCP
Emeritus Reader in Medicine, UCL Institute for Liver and Digestive Health, and Honorary Consultant Hepatologist, Sheila Sherlock Liver Centre, Royal Free Hospital
London, UK

Adam Doyle MBBS, BMedSc, FRACP
Department of Gastroenterology and Hepatology
Royal Perth Hospital
Perth, WA, Australia

Geoffrey Dusheiko FCP(SA), FRCP, FRCP(Edin)
Emeritus Professor of Medicine, UCL Institute for Liver and Digestive Health, and Kings College Hospital
London, UK

Patrizia Farci MD
Chief, Hepatic Pathogenesis Section
Laboratory of Infectious Diseases
National Institute of Allergy and Infectious Diseases
National Institutes of Health
Bethesda, MD, USA

Robert J. Fontana, MD
Professor of Medicine
Department of Internal Medicine, University of Michigan Medical School
Ann Arbor, MI, USA

Ewan Forrest MD, FRCPS (Glasgow), FRCP (Edin)
Consultant Hepatologist
Liver Unit, Glasgow Royal Infirmary
Glasgow, UK

Scott L. Friedman MD
Fishberg Professor of Medicine
Chief, Division of Liver Diseases
Icahn School of Medicine at Mount Sinai
New York, NY, USA

Guadalupe Garcia-Tsao MD
Professor of Medicine
Section of Digestive Diseases
Yale School of Medicine
New Haven, CT;
Veterans Affairs Connecticut Healthcare System
West Haven, CT, USA

Kurinchi S. Gurusamy MB BS, MRCS, PhD
Reader in Surgery
University College London Medical School, Royal Free Campus, and Royal Free Hospital, London, UK

Nedim Hadzic MD, MSc, FRCPCH, FAALD
Professor and Consultant in Paediatric Hepatology
Paediatric Centre for Hepatology, Gastroenterology and Nutrition
King's College Hospital
London, UK

Timothy Hardy MB BS, BSc (Hons)
MRC Clinical Research Fellow
Institute of Cellular Medicine, Newcastle University
Newcastle upon Tyne, UK

Gideon M. Hirschfield MA, MB BChir, PhD, FRCP
Professor and Honorary Consultant Hepatologist
Centre for Liver Research, NIHR Biomedical Research Centre, University of Birmingham
Birmingham, UK

Nicolas M. Intagliata MD
Assistant Professor of Medicine, Gastroenterology and Hepatology
University of Virginia
Charlottesville, VA, USA

Rajiv Jalan MBBS, MD, PhD, FRCP, FRCPE, FAASLD
Professor of Hepatology
Liver Failure Group, Institute for Liver and Digestive Health, Division of Medicine, University College London
London, UK

Ashnila Janmohamed BSc, MBBS, MRCP
Clinical Research Fellow in Hepatology Centre for Liver Research, NIHR Biomedical Research Centre, University of Birmingham
Birmingham, UK

Peter L. M. Jansen MD, PhD
Professor of Hepatology
Department of Gastroenterology and Hepatology
Academic Medical Center
University of Amsterdam
Amsterdam, The Netherlands

David E. J. Jones BM, BCh, MA, PhD, FRCP
Professor of Liver Immunology
Medical School, Newcastle University
Honorary Consultant Hepatologist
Freeman Hospital
Newcastle upon Tyne, UK

Tom Hemming Karlsen MD, PhD
Professor of Internal Medicine
Senior Consultant of Gastroenterology and Hepatology
Department of Transplantation Medicine
Oslo University Hospital Rikshospitalet
Oslo, Norway

Deirdre A. Kelly MD, FRCP, FRCPI, FRCPCH
Professor of Paediatric Hepatology
Birmingham Children's Hospital and University of Birmingham
Birmingham, UK

Lindsay Y. King MD, MPH
Assistant Professor of Medicine
Duke University Health System
Durham, NC, USA

Rahul S. Koti MD, FRCS
Honorary Senior Lecturer in Surgery
Royal Free Hospital and University College London Medical School
London, UK

William M. Lee MD, FACP
Professor of Internal Medicine
University of Texas Southwestern Medical Center
Dallas, TX, USA

Jay H. Lefkowitch MD
Professor of Clinical Pathology
College of Physicians and Surgeons, Columbia University
New York, NY, USA

Anna S. F. Lok MBBS, MD, FRCP
Alice Lohrman Andrews Research Professor in Hepatology
Director of Clinical Hepatology
Division of Gastroenterology and Hepatology
Michigan Medicine, University of Michigan
Ann Arbor, MI, USA

Tu Vinh Luong MD
Consultant Histopathologist
Department of Cellular Pathology, Royal Free Hospital;
Honorary Senior Lecturer,
UCL Institute for Liver and Digestive Health
London, UK

Avik Majumdar MBBS(Hons), MPHTM, FRACP
UCL Institute for Liver and Digestive Health and Sheila Sherlock Liver Centre
Royal Free Hospital, London, UK;
AW Morrow Gastroenterology and Liver Centre Australian National Liver Transplant Unit Royal Prince Alfred Hospital, Sydney, NSW, Australia

Christopher McNamara FRACP, FRCPA, FRCPath
Department of Haematology
University College London Hospitals
London, UK

P. Aiden McCormick MD, FRCP, FRCPI, FEBGH, FAASLD
Consultant Hepatologist and Newman Clinical Research Professor
St Vincent's University Hospital and University College Dublin
Dublin, Ireland

George Mells MB BS, PhD, MRCP
Consultant Hepatologist
Cambridge Liver Unit
Addenbrooke's Hospital
Cambridge, UK

Manuela Merli MD
Associate Professor, Gastroenterology
Department of Clinical Medicine, Sapienza University of Rome
Rome, Italy

Marsha Y. Morgan MB ChB, FRCP
Principal Research Associate and Honorary Consultant Physician
UCL Institute for Liver and Digestive Health Division of Medicine University College London
London, UK

Grazia Anna Niro, MD
Gastroenterology Unit
IRCCS "Casa Sollievo Sofferenza" Hospital
San Giovanni Rotondo
Foggia, Italy

David Patch MB BS, FRCP
Consultant Hepatologist
Sheila Sherlock Liver Centre, Royal Free Hospital and UCL Institute for Liver and Digestive Health
London, UK

Massimo Pinzani MD, PhD, FRCP, FAASLD
Professor of Medicine
UCL Institute for Liver and Digestive Health and Sheila Sherlock Liver Centre
Royal Free Hospital
London, UK

Eve A. Roberts MD, PhD, FRCPC
Departments of Paediatrics, Medicine, and Pharmacology and Toxicology
University of Toronto
Toronto, ON, Canada

Morris Sherman MB BCh, PhD, FRCP(C), FAASLD
Professor of Medicine
Department of Medicine, Division of Gastroenterology
University of Toronto and...

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