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primary sclerosing cholangitis


Definition: idiopathic chronic hepatobiliary disease characterized by diffuse inflammation and fibrosis of the extrahepatic biliary system resulting in patchy, irregular stricturing of the bile ducts. Progressive bile duct obliteration to cirrhosis, hepatic failure, and bile duct cancer may occur in up to 50% of patients. Frequently associated with inflammatory bowel disease.

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.