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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease biliary cirrhosis, primary, 1
Comorbidity C0008370|bile duct obstruction
Sentences 1
PubMedID- 26357635 Main complications are irregular bile duct obstruction, development of secondary biliary cirrhosis, and cholangiocarcinoma.36 pathogenesis of psc is incompletely understood, but this entity has been described as an immune-mediated phenomenon triggered by environmental factors in genetically susceptible individuals.37 the perfect animal model that summarizes all the attributes of psc (cholangitis of the intra- and extra-hepatic bile ducts in association with gut inflammation and development of cholangiocarcinoma and/or secondary biliary cirrhosis) and exhibits a male predominance does not exist.

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