An adult case of Alagille's syndrome.

T, Morioka; Shimizu, Mitsugu; Motoo, Yoshiharu; Urabe, Takeshi; Yoneshima, Manabu; Unoura, Masashi; Tanaka, Nobuyoshi; Kobayashi, Kenichi; Hattori, Nobu; Nakanuma, Yasuni

doi:10.2957/kanzo.30.364

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1991

1995

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“…Obviously, idiopathic adulthood ductopenia comprises a clinical spectrum with some patients having progressive while others have stable disease. Even in Alagille's syndrome progression seems to be variable, as an adult patient with late manifestation has been described recently (25).…”

Section: Discussionmentioning

confidence: 99%

Manifestation late in life of idiopathic adulthood ductopenia

Müller

Walter

Penner

1995

Liver

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Idiopathic adulthood ductopenia is a chronic cholestasic liver disease of unknown etiology characterized by the loss of interlobular bile ducts. We describe three patients who fulfilled the diagnostic criteria of idiopathic adulthood ductopenia, but differed from the cases reported so far in late manifestation of the disease and a benign clinical course despite histologic evidence of ongoing cholangitis. Treatment with ursodeoxycholic acid in one patient resulted in improvement of biochemical markers of cholestasis, suggesting that chronic cholestasis in idiopathic adulthood ductopenia can be influenced beneficially.

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Section: Discussionmentioning

confidence: 99%