Summerville D A, Marks M, Treves S T
Department of Radiology, Children's Hospital, Boston, Massachusetts.
Pediatr Radiol. 1988;18(1):32-4. doi: 10.1007/BF02395757.
Hepatobiliary scintigraphy has proven to be of great utility in distinguishing biliary atresia from other causes of neonatal cholestasis. Arteriohepatic dysplasia (Alagille's syndrome) is an uncommon entity characterized by typical facial features, pulmonary artery stenosis, and a liver disorder which presents during the neonatal period as progressive jaundice. Two neonates, who were later shown to have Alagille's syndrome, underwent hepatobiliary scintigraphy to rule out biliary atresia. Findings on the hepatobiliary scans from the two patients were similar to those usually associated with biliary atresia and both finally required surgical exploration to rule out biliary atresia. The findings on hepatobiliary scans in these patients with Alagille's syndrome are discussed and compared with those associated with other forms of neonatal cholestasis.
肝胆闪烁显像已被证明在鉴别胆道闭锁与新生儿胆汁淤积的其他病因方面具有很大的实用价值。动脉肝发育不良（阿拉吉耶综合征）是一种罕见的病症，其特征为典型的面部特征、肺动脉狭窄以及一种在新生儿期表现为进行性黄疸的肝脏疾病。两名后来被诊断为阿拉吉耶综合征的新生儿接受了肝胆闪烁显像以排除胆道闭锁。这两名患者的肝胆扫描结果与通常与胆道闭锁相关的结果相似，最终两人均需要进行手术探查以排除胆道闭锁。本文讨论了这些患有阿拉吉耶综合征患者的肝胆扫描结果，并与其他形式的新生儿胆汁淤积相关的结果进行了比较。
