婴儿胆汁淤积症的病因研究进展

婴儿胆汁淤积症是一类多因素引起的以高胆红素血症为临床特点的综合征,病因谱广泛,包括感染、中毒、肝内外胆管发育异常及遗传代谢缺陷等.感染、胆道闭锁患儿占胆汁淤积症患儿半数以上,除了常见病因,citrin缺陷症、Alagille综合征、进行性家族性胆汁淤积症等新病因不断被认识,这些疾病临床症状与胆道闭锁相似,预后及治疗各不...

Progress of the causes of infantile cholestasis

Infantile cholestasis is a syndrome caused by multiple factors with conjugated hyperbilirubinemia for clinical characteristics. The etiologic factors include infection, poisoning, inherited metabolic defects and bile duct abnormalities. Infection, biliary atresia accounts for more than half of cholestasis children. Recently, the new causes have been recognized, for example citrin deficiency, Alagille syndrome,progressive familial cholestasis symptoms caused by different causes are similar to biliary atresia, but the prognosis and treatment are quite different. If misdiagnosed, the condition will exacerbate. Understanding the various causes plays an important role for the early diagnosis and differential diagnosis of infantile cholestasis.