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Sherlock's Diseases of the Liver and Biliary System, 12th Edition

James S. Dooley (Editor), Anna Lok (Editor), Andrew K. Burroughs (Editor), Jenny Heathcote (Editor)
ISBN: 978-1-4051-3489-7
792 pages
May 2011, Wiley-Blackwell
Awarded first prize in the Internal Medicine category of the British Medical Association Book of the Year Awards, 2012

Following a Tradition of Excellence

from reviews of previous editions:

"the best source of synthesized clinical wisdom"

"a tour de force in terms of knowledge and effort"
The New England Journal of Medicine

"the foremost liver book in the world"
The Journal of the American Medical Association

"beautifully produced"

Over the past 56 years, thousands of physicians have depended on Diseases of the Liver and Biliary System. Its didactic and reliable clinical guidance was - and still is - beyond comparison.

This brand-new edition, now named Sherlock's Diseases of the Liver and Biliary System, after the late Professor Dame Sheila Sherlock, continues to provide concise clinical guidance for all those treating patients with hepato-biliary disease.

Enabling clinicians to formulate incisive diagnoses and appropriate treatment strategies, this book has been updated to reflect the advances that have been made in the last 10 years, providing didactic and reliable clinical guidance in hepatology from the world’s leading experts.

A consistent chapter structure allows readers to access the information immediately, with summary boxes and key learning points throughout, and special emphasis on the latest in evidence-based clinical guidance. And for the first time, this edition now offers a free companion website providing the 680 full-color illustrations and figures in the book, for use in scientific presentations.

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List of Contributors.

Preface to the Twelfth Edition.

Preface to the First Edition.

1 Anatomy and Function (Jay H. Lefkowitch).

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.

Surface marking.

Methods of examination.

Microanatomy of the liver.

Hepatic ultrastructure (electron microscopy) and organelle functions.

Functional heterogeneity of the liver.

Dynamics of the hepatic microenvironment in physiology and disease.

Hepatocyte death and regeneration.

2 Assessment of Liver Function (Sandeep Mukherjee & John L. Gollan).

Selection of biochemical tests.

Bile pigments.

Serum enzyme tests.

Quantitative assessment of hepatic function.

Lipid and lipoprotein metabolism.

Bile acids.

Amino acid metabolism.

Plasma proteins.

Carbohydrate metabolism.

Effects of ageing on the liver.

3 Biopsy of the Liver (David Patch & Amar Paul Dhillon).

Selection and preparation of the patient.


Risks and complications.

Sampling variability.

Naked-eye appearances.

Preparation of the specimen.



Special methods.

4 Haematological Disorders of the Liver (Pramod K. Mistry & Dhanpat Jain).

The liver and blood coagulation.

Haemolytic jaundice.

The liver in haemolytic anaemias.

The liver in myelo- and lymphoproliferative disease.


Bone marrow transplantation.


Lipid storage diseases.

5 Acute Liver Failure (Shannan R. Tujios & William M. Lee).

Defi nition.

Epidemiology and aetiologies.

Clinical features.

Initial investigations.

Complications and management of acute liver failure.

Specific therapies.


Liver transplantation.

Liver support systems.


6 Hepatic Fibrogenesis (Meena B. Bansal & Scott L. Friedman).


Natural history of hepatic fi brosis.

Cellular and molecular features of hepatic fi brosis.

Clinical aspects of hepatic fibrosis.

Emerging antifi brotic targets and strategies.

7 Hepatic Cirrhosis (P. Aiden McCormick).


Causes of cirrhosis.

Anatomical diagnosis.

Reversible cirrhosis.

Clinical cirrhosis: compensated versus decompensated.

Vasodilatation and hyperdynamic circulation.

Prognosis (Child–Pugh score, MELD, UKELD).

Clinical and pathological associations.


8 Hepatic Encephalopathy in Patients with Cirrhosis (Marsha Y. Morgan).



Differential diagnosis.

Hepatic encephalopathy and liver transplantation.



Management of hepatic encephalopathy.


9 The Hepatic Artery, Portal Venous System and Portal Hypertension: the Hepatic Veins and Liver in Circulatory Failure (Andrew K. Burroughs).

The hepatic artery.

The portal venous system.

Haemodynamics of portal hypertension.

Clinical features of portal hypertension.

Diagnosis of varices.

Imaging the portal venous system.

Classifi cation of portal hypertension.

Extrahepatic portal venous obstruction.

Presinusoidal intrahepatic and sinusoidal portal hypertension.

Bleeding oesophageal varices.

Management of acute variceal bleeding.

The hepatic veins.

Budd–Chiari (hepatic venous obstruction) syndrome.

Circulatory failure.

10 Ascites (Guadalupe Garcia-Tsao).

Mechanisms of ascites formation.

Clinical features.

Differential diagnosis.

Spontaneous bacterial peritonitis.

Treatment of cirrhotic ascites.


Refractory ascites.

Hepatorenal syndrome.


11 Jaundice and Cholestasis (Elwyn Elias).


Classification of jaundice.

Physiology and pathophysiology.

Syndrome of cholestasis.

Investigation of the jaundiced patient.

Differential diagnosis.


Familial non-haemolytic hyperbilirubinaemias.

12 Gallstones and Benign Biliary Diseases (James S. Dooley).


Composition of gallstones.

Formation of cholesterol stones.

Factors in cholesterol stone formation.

Pigment gallstones.

Natural history of gallbladder stones.

Acute calculous cholecystitis.

Empyema of the gallbladder.

Emphysematous cholecystitis.

Chronic calculous cholecystitis.

Acalculous cholecystitis.


Postcholecystectomy bile duct damage.

Postcholecystectomy syndromes.

Non-surgical treatment of gallstones in the gallbladder.

Other gallbladder pathology.

Biliary fi stulae.

Gallstone ileus.

Bile peritonitis.

Association between cholecystectomy and colorectal cancer.

Common duct stones.

Management of duct stones.


Bile duct–bowel anastomotic stricture.

Chronic pancreatitis.

Primary sclerosing cholangitis and autoimmune pancreatitis.

Bile duct pathology following liver transplantation.

13 Malignant Biliary Diseases (Rahul S. Koti & Brian R. Davidson).

Carcinoma of the gallbladder.

Carcinoma of the bile duct (cholangiocarcinoma).

Intrahepatic cholangiocarcinoma.

Other biliary malignancies.

Metastases at the hilum.

Periampullary carcinoma.


14 Cysts and Congenital Biliary Abnormalities (Giorgina Mieli-Vergani & Nedim Hadžic).

Fibropolycystic diseases.

Adult polycystic disease.

Congenital hepatic fi brosis.

Caroli’s disease.

Microhamartoma (von Meyenberg complexes).

Choledochal cysts.

Congenital anomalies of the biliary tract.

15 Primary Biliary Cirrhosis (Margaret F. Bassendine).

Clinical features.



Epidemiology and genetics.



16 Sclerosing Cholangitis (Simon Rushbrook & Roger W. Chapman).


Primary sclerosing cholangitis.

Secondary sclerosing cholangitis.

17 Enterically Transmitted Viral Hepatitis: Hepatitis A and Hepatitis E (Peter Karayiannis & Howard C. Thomas).

General features of enterically transmitted viral hepatitis.

Hepatitis A virus.

Hepatitis E virus.

18 Hepatitis B (Anna S. F. Lok).


Hepatitis B virus.

Immune response and mechanisms of hepatic injury.




Clinical manifestations.

Natural history.


19 Hepatitis D (Patrizia Farci).


Hepatitis D virus.



Modes of infection and clinical course.




20 Hepatitis C (Geoffrey Dusheiko).




Pathology and pathogenesis.

Diagnostic tests for hepatitis C.

Acute hepatitis C.

Chronic hepatitis C.

21 Hepatitis due to Non-A–E Viruses (Antonio Craxì & Rosa Di Stefano).

General features of non-A–E hepatitides.

Hepatotropic viruses.

Systemic viral infections that often cause transient liver involvement.

22 HIV and the Liver (Marion G. Peters & Vincent Soriano).

Viral hepatitis and human immunodefi ciency virus (HIV) infection.

Cirrhosis and liver transplantation.

HIV-associated opportunistic infections and the liver.

HIV-associated neoplasms of the liver.

Antiretroviral-related liver injury in HIV.

23 Autoimmune Hepatitis and Overlap Syndromes (Gideon M. Hirschfi eld & E. Jenny Heathcote).


Disease overview.

Biological determinants of disease.

Disease presentation.

Laboratory features.


Liver biopsy and histological features.

Differential diagnosis.

Diagnostic dilemmas.

Making a diagnosis in practice.

Management strategies.

Pregnancy and autoimmune hepatitis.

Contraception choices for patients with autoimmune hepatitis.

The elderly and autoimmune hepatitis.

Childhood-onset autoimmune hepatitis.

Autoimmune hepatitis and liver transplantation.

Overlap syndromes.


24 Drug-Induced Liver Injury (Leonard B. Seeff & Robert J. Fontana).


Worldwide epidemiology.

Expressions of hepatotoxicity.

Classifi cation of hepatotoxicity.

Predictors of susceptibility and outcome in druginduced liver injury.

Mechanisms of injury, drug metabolism and pharmacokinetics.

Diagnostic approaches and causality assessment of drug-induced liver injury.

Clinical and biochemical presentations of drug-induced liver disease.

Assessment of suspected drug-induced liver disease.

Assessing causality for drug-induced liver disease.

Medical management.

Liver injury from specifi c drugs.

25 Alcohol and the Liver (Stephen Stewart & Chris Day).


Alcohol metabolism.



Histological features.

Clinical features.

Clinical syndromes.



26 Iron Overload States (Paul Adams).

Normal iron metabolism.

Iron overload and liver damage.

Genetic haemochromatosis.

Other iron storage diseases.

27 Wilson’s Disease (Eve A. Roberts).

Molecular genetics: pathogenesis.


Clinical picture.

Genetic strategies.

Diagnostic diffi culties.



Indian childhood cirrhosis.

28 Non-alcoholic Fatty Liver Disease and Nutrition (Stephen H. Caldwell & Curtis K. Argo).


Clinical features.

Laboratory testing.

Mitochondriopathies and lipodystrophy.

Epidemiology of non-alcoholic fatty liver disease.

Pathogenesis of non-alcoholic fatty liver disease and non-alcoholic steatohepatitis.

The natural history of non-alcoholic fatty liver disease (non-alcoholic steatohepatitis and non-NASH fatty liver).

Therapy of non-alcoholic fatty liver disease.

Other forms of non-alcoholic fatty liver.

29 The Liver in the Neonate, in Infancy and Childhood (Deirdre A. Kelly).

Investigation of liver disease in children.

Neonatal jaundice.

Neonatal liver disease (conjugated hyperbilirubinaemia).

Neonatal hepatitis syndrome.

Inherited disease in the neonate.

Genetic cholestatic syndromes.

Structural abnormalities: biliary atresia and choledochal cyst.

Acute liver failure in infancy.

Liver disease in older children.

Metabolic disease in older children.

Cirrhosis and portal hypertension.

Liver transplantation.

Tumours of the liver.

30 The Liver in Pregnancy (Andrew K. Burroughs & E. Jenny Heathcote).

Normal pregnancy.

Liver disease in pregnancy.

Diseases specifi c to pregnancy.

Diseases of late pregnancy.

Pregnancy in those with acute or chronic liver disease.

Hepatotoxic drugs and the pregnant woman.

Pre-existing liver disease.

Pregnancy in liver transplant recipients.

31 The Liver in Systemic Disease (Humphrey J. F. Hodgson).

Collagen-vascular and autoimmune disorders.

Hepatic granulomas.

The liver in diabetes mellitus.

Liver and thyroid.

Liver and adrenal.

Liver and growth hormone.



Non-metastatic complications of malignancy.

Bone-marrow/stem cell transplantation; graft-versushost disease.

32 The Liver in Infections (Christopher C. Kibbler).


Jaundice of infections.

Pyogenic liver abscess.

Hepatic amoebiasis.

Tuberculosis of the liver.

Hepatic actinomycosis.

Syphilis of the liver.



Relapsing fever.

Lyme disease.

Rickettsial infections.

Fungal infections.

Schistosomiasis (bilharzia).


Kala-azar (visceral leishmaniasis).

Hydatid disease.


Strongyloides stercoralis.


Toxocara canis (visceral larva migrans).

Liver flukes.

33 Space-Occupying Lesions: the Diagnostic Approach (Neil H. Davies & Dominic Yu).


Computed tomography.

Magnetic resonance imaging.

Radioisotope scanning.

Positron emission tomography.

MR spectroscopy.

Conclusions and choice of imaging technique.

34 Benign Liver Tumours (Ian R. Wanless).

Diagnosis of focal liver lesions.

Hepatocellular tumours.

Biliary and cystic lesions.

Mesenchymal tumours.

35 Primary Malignant Neoplasms of the Liver (Morris Sherman).

Hepatocellular carcinoma.


Other malignant neoplasms of the liver.

36 Hepatic Transplantation (Andrew K. Burroughs & James O'Beirne).

Selection of patients.

Candidates: outcome.

Absolute and relative contraindications.

General preparation of the patient.

Donor selection and operation.

The recipient operation.


Postoperative course.

Post-transplantation complications.


37 Liver Transplantation in Patients with Hepatitis B, C or HIV Infection (Norah Terrault).


Hepatitis B and liver transplantation.

Hepatitis C and liver transplantation.

HIV and liver transplantation.



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James Dooley, Editor-in-Chief, worked with Professor Dame Sheila Sherlock on this book for three editions before her death in December 2001. He is a Consultant Hepatologist at the Royal Free Hospital, London.

Andrew Burroughs is a Consultant Physician and Hepatologist at the Royal Free Hospital, Liver Transplantation and Hepato-biliary Medicine Unit. He qualified in Liverpool with a MB ChB Honours in 1976, gained his MRCP in 1978 and FRCP in 1991. He is a Fellow of the European Board of Gastroenterology. He has been Scientific Secretary of the European Association for the Study of the Liver 1997-1999. He continues his association with EASL as administrative secretary until 2001. He is a council member of the United European Gastroenterology Federation until 2002, and a council member of the International Association for the Study of the Liver. He is a member of the MRC advisory board in the UK, from 1997 onwards. He was a member of Council of the British Society of Gastroenterology from 1992 to 1995. Andrew Burroughs main research interests are portal hypertension, variceal bleeding and coagulopathy, primary biliary cirrhosis, prognosis in liver disease and liver transplantation. He has published over 200 peer-reviewed articles.

Anna Lok is a Professor of Internal Medicine and the Director of Clinical Hepatology at the University of Michigan Medical Center. Her research focuses on the epidemiology and treatment of hepatitis B and C viruses. Millions of people worldwide are infected and are at risk for hepatitis-associated cirrhosis and hepatocellular carcinoma. Dr. Lok has made seminal contributions to our understanding of the natural history of hepatitis B and the role of hepatitis B virus genotypes and variants in the outcome of chronic hepatitis B infection. She has also made contributions to the treatment of hepatitis-induced diseases. She is currently testing new antiviral therapies for hepatitis B, developing cost-effective methods for preventing recurrent hepatitis B infection following liver transplantation, and studying the long-term effects of interferon treatment in patients with hepatitis C. During her distinguished career, she has received numerous awards for research and teaching.

Jenny Heathcote, Toronto Western Hospital, Canada.

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"This is a concise, updated, and well-written book that justifies replacing the previous edition. It is definitively useful and exhibits the same high quality of other currently available books in hepatology. I know I will consult my copy on a frequent basis -- an unusual event in this era of online material." (Doody's, 16 March 2012)

"James Dooley is to be congratulated on his courage and dedication along with his clearly inspiring leadership to contributing authors has not allowed Sheila's volume to die with her passing. The Hepatology world, has been given a thoroughly up-to-date, exciting and cleverly produced volume which competitors will have greatest difficulty in matching." (Institute of Hepatology, 2011)


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