This article presents a rare case of portal biliopathy, a secondary cholangiopathy, in a young man whose first clinical manifestation was bleeding from esophageal varices. Portal biliopathy may mimic primary sclerosing cholangitis and it can develop secondary to portal vein thrombosis usually acquired in early childhood. Narrowing and dilatation of biliary tree develops as a consequence of compression of common bile duct by collaterals. Clinical symptoms are mainly cholestatic jaundice and abdominal pain. Complications include recurrent cholangitis and secondary biliary cirrhosis. The article provides information on the pathogenesis, clinical symptoms and treatment of portal biliopathy and problems related to differential diagnosis with primary sclerosing cholangitis (PSC).
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