Hepatobiliary scan in Alagille syndrome; arteriohepatic dysplasia.
Arteriohepatic dysplasia or congenital paucity of interlobular bile ducts - Alagille Syndrome, is a well defined syndrome characterized by five major features, including chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects, peripheral pulmonary artery hypoplasia or stenosis and facial dysmorphy. The disease is very rare. Only three cases have been reported in Greece and none with renal involvement. Hepatobiliary scan was a fundamental tool in the patients diagnosis and therefore we present the following case.
Zissimopoulos
Athanassios
A
Nuclear Medicine Department, Medical School University of Alexandroupolis, Thrace, Greece. azissim@yahoo.gr
Cassimos
Dimitrios
D
Deftereos
Savas
S
Prassopoulos
Panos
P
Xinias
Ioannis
I
Pavlidou
Christina
C
Mavroudi
Antigone
A
Vrani
Olga
O
Chatzimichael
Athanassios
A
D016738:Alagille Syndrome / Q000000981:diagnostic imaging* / Q000473:pathology*; D001659:Biliary Tract / Q000000981:diagnostic imaging* / Q000473:pathology*; D006801:Humans; D007223:Infant; D008099:Liver / Q000000981:diagnostic imaging* / Q000473:pathology*; D008297:Male; D011877:Radionuclide Imaging