[Adult idiopathic ductopenia. 1 case].
Idiopathic adult ductopenia is very rare. We report one case in a 30-year-old man, whose clinical course was characterized by jaundice and pruritus. Laboratory investigations revealed cholestasis and polyclonal hypergammaglobulinemia. Serum antinuclear, antimitochondrial, and anti-smooth muscle antibodies and serological markers for viral hepatitis were negative. Endoscopic retrograde cholangiography showed no liver or biliary tract abnormalities. Histological examination of a liver specimen showed a vanishing bile duct syndrome and moderate portal infiltration with lympho-histiocytic cells; there were no granulomas. Liver transplantation was performed due to rapid development of cirrhosis. The differential diagnosis of idiopathic adult ductopenia with small duct primary sclerosing cholangitis, auto-immune cholangiopathy, and non syndromic paucity of intrahepatic bile ducts is unclear.
Service d'Hépato-Gastroentérologie, CHU Nord, Amiens.
F
F
Brazier
J C
JC
Duchmann
H
H
Sevestre
D
D
Capron
J P
JP
Capron
D000328:Adult; D001649:Bile Duct Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000473:pathology; D006644:Histiocytes / Q000473:pathology; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation; D008214:Lymphocytes / Q000473:pathology; D008297:Male
Ductopénie idiopathique de l'adulte. Un cas.