[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.
Kurtz
W
W
Abteilung für Gastroenterologie, Universitätsklinikums Frankfurt am Main.
Strohm
W D
WD
Lambrecht
E
E
Leuschner
U
U
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.