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Alagille综合征特点是肝内胆管形成不充分,但无肝外胆管梗阻。Alagille等[1]于1975年报告的肝内胆管形成不全病例中,15例具有特殊面容,以肺动脉狭窄为主的先天性心脏病、脊椎前弓异常、发育障碍、智能低下、性腺发育延迟等为特征。本病少见,儿童报道甚少,目前我省尚无报道。吉林大学第四医院儿科2013年6月诊治以肝功能异常为首发症状的儿童Alagille综合征1例,报告如下。4(
Alagille syndrome is characterized by inadequate intrahepatic bile duct formation but no extrahepatic bile duct obstruction. Alagille et al [1] in 1975 reported cases of intrahepatic bile ducts, 15 patients with special face, mainly pulmonary stenosis congenital heart disease, anterior spinal arch anomalies, dysplasia, mental retardation, delayed gonadal development For the characteristics. The rare disease, children rarely reported, at present there is no report in our province. Fourth Hospital of Jilin University pediatric June 2013 diagnosis and treatment of liver function abnormalities as the first symptom of children Alagille syndrome in 1 case, the report is as follows. 4 (