20. Chronic Cholestatic Liver Disease and its Management in Adults and Children

  1. E. Jenny Heathcote MB, BS, MD, FRCP, FRCP(C)4,5,6,7
  1. Gideon M. Hirschfield MB, BChir, MRCP, PhD1,2,5 and
  2. Binita M. Kamath MBBChir, MRCP, MTR3,5

Published Online: 4 SEP 2012

DOI: 10.1002/9781118314968.ch20

Hepatology: Diagnosis and Clinical Management

Hepatology: Diagnosis and Clinical Management

How to Cite

Hirschfield, G. M. and Kamath, B. M. (2012) Chronic Cholestatic Liver Disease and its Management in Adults and Children, in Hepatology: Diagnosis and Clinical Management (ed E. J. Heathcote), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118314968.ch20

Editor Information

  1. 4

    Francis Family Chair in Hepatology Research, Toronto, Ontario, Canada

  2. 5

    University of Toronto, Toronto, Ontario, Canada

  3. 6

    Patient Based Clinical Research Division, Toronto Western Research Institute, Toronto, Ontario, Canada

  4. 7

    University Health Network/Toronto Western Hospital, Toronto, Ontario, Canada

Author Information

  1. 1

    Liver Centre, Division of Gastroenterology, Toronto Western Hospital, University Health Network, Toronto, Ontario, Canada

  2. 2

    Centre for Liver Research, University of Birmingham, Birmingham, UK

  3. 3

    Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada

  4. 5

    University of Toronto, Toronto, Ontario, Canada

Publication History

  1. Published Online: 4 SEP 2012
  2. Published Print: 12 OCT 2012

ISBN Information

Print ISBN: 9780470656174

Online ISBN: 9781118314968

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Keywords:

  • primary biliary cirrhosis;
  • primary sclerosing cholangitis;
  • cholestasis;
  • biliary atresia;
  • Alagille syndrome

Summary

Cholestatic liver diseases span a wide spectrum of etiologies and represent important chronic liver diseases in children and adults. Treatment has broadly improved over time, perhaps in some part due to early diagnosis in the asymptomatic period. Treatment should be targeted to the specific disease. Liver transplantation is an important component of care, particularly for patients with biliary atresia and primary sclerosing cholangitis. In contrast, for primary biliary cirrhosis, the widespread use of ursodeoxycholic acid has likely contributed to the ongoing improved outcomes. Patient management must also include adequate treatment of symptoms, and surveillance for complications of cirrhosis and portal hypertension.