Idiopathic ductopenia

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Idiopathic ductopenia

Synonyms: IAD | Idiopathic adult ductopenia

A rare biliary tract disease characterized by loss of interlobular bile ducts resulting in chronic cholestasis without any known cause. Loss of less than 50% of interlobular bile ducts is associated with a mild disease course while loss of the majority of ducts results in a severe form potentially leading to cirrhosis and liver failure. Patients typically present as young or middle-aged adults with episodic jaundice pruritus and elevated liver enzymes.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: // Data version October 2023.

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