婴儿胆汁淤积性肝病的病因复杂,临床主要表现为高结合胆红素血症、粪便颜色改变、胆汁酸增加,可伴或不伴肝大,质地异常,肝功能异常;部分患儿还可伴皮肤瘙痒、营养不良等表现。治疗的主要原则为保肝、利胆、营养和病因治疗。预后因病因而异。 The etiology of infantile cholestatic liver disease is complex, the main clinical manifestations of hyperbilirubinemia, stool color changes, increased bile acid, with or without hepatomegaly, texture abnormalities, liver dysfunction; some children with Itchy skin, malnutrition and other performance. The main principles of treatment for the liver, gallbladder, nutrition and cause of treatment. Prognosis varies due to illness.