进行性家族性肝内胆汁淤积(PFIC)是一组常染色体隐性遗传肝细胞源性儿童胆汁淤积症。根据病因可以分为3型：1型源于ATP8B1基因的突变,2型源于ABCB11基因的突变,3型源于MDR3基因的突变。临床以有胆汁淤积以及严重的皮肤瘙痒为特征。治疗方法包括药物治疗、外科部分胆道外分流术和肝移植三种方法。 Progressive familial intrahepatic cholestasis (PFIC) is a group of autosomal recessive hepatocyte-derived cholestasis of children. According to the etiology can be divided into 3 types: 1 type from the ATP8B1 gene mutation, type 2 from ABCB11 gene mutation, type 3 from MDR3 gene mutation. Clinically with cholestasis and severe skin itching is characterized. Treatment includes drug treatment, surgical partial biliary shunt and liver transplantation in three ways.