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Chapter 21 - Sclerosing Cholangitis in Children

from Section III - Hepatitis and Immune Disorders

Published online by Cambridge University Press:  19 January 2021

By
William Balistreri  and
Simon Lam
Edited by
Frederick J. Suchy ,
Ronald J. Sokol  and
William F. Balistreri
Edited in association with
Jorge A. Bezerra ,
Cara L. Mack  and
Benjamin L. Shneider
Frederick J. Suchy
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Ronald J. Sokol
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
William F. Balistreri
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Jorge A. Bezerra
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Cara L. Mack
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Benjamin L. Shneider
Affiliation:
Texas Children’s Hospital, Houston
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Summary

Primary sclerosing cholangitis (PSC) is an idiopathic progressive fibrosing cholangiopathy characterized by chronic inflammation and injury of the intra- and/or extrahepatic bile ducts, leading to fibrosis and cholestasis. Although much work has been done over the last several decades to further our understanding of this disease, the underlying mechanisms of pathogenesis remain elusive. To date, there are no effective therapies, and PSC continues to be a well-recognized cause of end-stage liver disease requiring liver transplantation in adults and children.

Type
Chapter
Information
Liver Disease in Children , pp. 333 - 347
Publisher: Cambridge University Press
Print publication year: 2021

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