Alagille综合征是一种常染色体显性遗传病,常以婴儿期胆汁淤积为突出表现,并可累及心脏、骨骼、眼睛、颜面及肾脏等多系统,国外报道该病的发病率约为1/70000[1],国内少见报道,我院诊断1例,现报告如下。1病例资料患儿男,15岁余,因“发现黄疸15年”于2012-07-16入首都儿科研究所附属儿童医院。 Alagille syndrome is an autosomal dominant genetic disease, often highlighted in infantile cholestasis and can affect the heart, bones, eyes, face and kidney and other systems, the incidence of foreign reports of the disease is about 1 / 70000 [1], rare domestic reports, our hospital diagnosis of 1 case, are as follows. 1 case data children male, 15 years old, because “found jaundice 15 years ” in 2012-07-16 into the capital of Pediatric Institute affiliated children’s hospital.