A Case of Idiopathic Adulthood Ductopenia

Abstract

Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic liver disease of unknown etiology characterized by adult onset, an absence of autoantibodies, inflammatory bowel disease, and a loss of interlobular bile ducts. In the present report, a case fulfilling the IAD criteria is described. A 19-year-old man was admitted to the hospital for persistent elevation of transaminases and alkaline phosphatase without clinical symptoms. Viral hepatitis markers and autoantibodies were absent. The patient had a normal extrahepatic biliary tree and had no evidence of inflammatory bowel disease. A liver biopsy specimen showed absence of interlobular bile ducts from 58% of the portal tracts. He was diagnosed with IAD and was treated with ursodeoxycholic acid.

Idiopathic adulthood ductopenia with elevated transaminase only: A case report

“…In recent years, the pathology and pathogenesis of bile duct loss have been widely studied, and a number of hepatobiliary diseases have been added to the list of ductopenic diseases[ 4 ]. IAD is a special type of ductopenic disease that is characterized by adult onset, autoantibodies negative, inflammatory bowel disease and absence of interlobular bile ducts[ 5 ].…”

Idiopathic adulthood ductopenia manifesting as jaundice in a young male

“…The evidence of ductopenia in liver biopsy or cholangiographic evidence of paucity of bile ducts leads to confirmation of diagnosis. Exclusion of other conditions with chronic cholestasis that are associated with ductopenia is very much needed to support the diagnosis of IAD [2].…”