Zissimopoulos Athanassios, Cassimos Dimitrios, Deftereos Savas, Prassopoulos Panos, Xinias Ioannis, Pavlidou Christina, Mavroudi Antigone, Vrani Olga, Chatzimichael Athanassios
Nuclear Medicine Department, Medical School University of Alexandroupolis, Thrace, Greece.
Hell J Nucl Med. 2009 May-Aug;12(2):158-60.
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.
动脉肝发育不良或先天性小叶间胆管缺乏——阿拉吉耶综合征，是一种明确的综合征，其特征为五大主要表现，包括慢性胆汁淤积、后胚胎毒素、蝶形椎弓缺损、外周肺动脉发育不全或狭窄以及面部畸形。该疾病非常罕见。希腊仅报告过3例，且无一例有肾脏受累情况。肝胆扫描是诊断此类患者的重要工具，因此我们呈现以下病例。
