[Alagille syndrome, a rare differential diagnosis of intrahepatic cholestasis].
In Alagille's syndrome cholestasis is caused by a congenital hypoplasia of the intrahepatic bile ducts. If cardiac and arterial malformations are present in conjunction with the typical-but not pathognomonic-facies, this rare syndrome must be considered. ERCP shows a highly rarified bile duct system. Liver histology with a paucity of interlobular bile ducts establishes the diagnosis. Inheritance appears to be autosomal dominant. The relatively good prognosis of the disease calls for caution concerning invasive procedures.
Abteilung für Gastroenterologie, Universitätsklinikums Frankfurt am Main.
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Kurtz
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Strohm
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Lambrecht
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U
Leuschner
D000015:Abnormalities, Multiple / Q000473:pathology; D000293:Adolescent; D001653:Bile Ducts, Intrahepatic / Q000002:abnormalities* / Q000473:pathology; D002780:Cholestasis, Intrahepatic / Q000151:congenital* / Q000473:pathology; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D007690:Polycystic Kidney Diseases / Q000151:congenital; D013577:Syndrome
Das Alagille-Syndrom, eine seltene Differentialdiagnose der intrahepatischen Cholestase.