[Hypoplasia of intra- and extrahepatic bile ducts associated with congenital abnormalities].
The present report deals with a patient affected by Alagille's syndrome. The clinical hallmark of this syndrome is chronic cholestasis with ductular and extrahepatic hypoplasia and associated multiple congenital malformations. The diagnosis was made by hepatobiliary scanning with TC99-HIDA, transendoscopic cholangiography and liver biopsy.
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D001652:Bile Ducts / Q000002:abnormalities* / Q000000981:diagnostic imaging; D002648:Child; D002779:Cholestasis / Q000000981:diagnostic imaging / Q000209:etiology*; D002908:Chronic Disease; D006801:Humans; D008297:Male; D011877:Radionuclide Imaging; D013577:Syndrome
Ipoplasia delle vie biliari intra ed extraepatiche con malformazioni congenite associate.