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

Jurgen Ludwig, Russell H. Wiesner, Nicholas F La Russo

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Abstract

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
Volume7
Issue number2
DOIs
StatePublished - 1988

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Biliary Liver Cirrhosis
Liver Cirrhosis
Liver Diseases
Intrahepatic Bile Ducts
Inflammatory Bowel Diseases
Sclerosing Cholangitis
Cholangitis
Bile Ducts
Liver Transplantation
History
Antibodies
Pharmaceutical Preparations

ASJC Scopus subject areas

  • Gastroenterology

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Idiopathic adulthood ductopenia. A cause of chronic cholestatic liver disease and biliary cirrhosis. / Ludwig, Jurgen; Wiesner, Russell H.; La Russo, Nicholas F.

In: Journal of Hepatology, Vol. 7, No. 2, 1988, p. 193-199.

Research output: Contribution to journalArticle

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