Idiopathic adulthood ductopenia. A cause of chronic cholestatic liver disease and biliary cirrhosis

Jurgen Ludwig, Russell H. Wiesner, Nicholas F. LaRusso

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137 Scopus citations


We describe three adult patients who had chronic cholestatic liver disease associated with unexplained loss of interlobular bile ducts; two of these patients eventually required orthotopic liver transplantation. We have named this condition 'idiopathic adulthood ductopenia' because (1) the etiology is unknown, (2) the age of the patients may be the only distinguishing feature between this newly described condition and neonatal or infantile nonsyndromatic paucity of intrahepatic bile ducts, and (3) morphologic demonstration of ductopenia is an indispensable finding. Our three patients, all males, had a negative drug history, absence of antimitochondrial antibodies, normal cholangiographic findings, and no evidence of inflammatory bowel disease. Idiopathic adulthood ductopenia may be a representation of (1) late onset of infantile paucity of intrahepatic bile ducts, (2) destructive viral cholangitis, and (3) isolated small-duct primary sclerosing cholangitis - that is, 'pericholangitis' unassociated with inflammatory bowel disease.

Original languageEnglish (US)
Pages (from-to)193-199
Number of pages7
JournalJournal of hepatology
Issue number2
StatePublished - 1988

ASJC Scopus subject areas

  • Hepatology


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