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肝动脉畸形(又称Alagille综合征和Wat-son-Alagille综合征)是一种常染色体显性遗传性先天性疾病,伴不同的明显的外显率。由Watson和Miller首次报道,并由Alagille充实,病变缺陷可能位于20号染色体短臂。临床表现包括慢性肝内胆汁郁积(gi%)伴疾痒、典型面容(95%)(前
Hepatic artery malformations (also known as Alagille’s syndrome and Wat-son-Alagille syndrome) are an autosomal dominant inherited congenital disease with distinctly different penetrances. First reported by Watson and Miller and augmented by Alagille, the defect may lie on chromosome 20 short arm. Clinical manifestations include chronic intrahepatic cholestasis (gi%) with itchy, typical face (95%) (before