患儿男性,3岁3个月,因皮肤搔痒、肝功异常3年余入院。出生后患儿家长即发现患儿皮肤搔痒,当地医院检查肝功异常,予熊去氧胆酸等药物保肝、降酶治疗,症状无好转,转氨酶、胆红素反复异常。既往无明确病毒性肝炎密切接触史,患儿无输血及血液制品应用史,无不洁饮食史。查体:神志清晰,全身皮肤、巩膜无黄染,皮肤干燥,胸背、四肢可见多 Children with children, 3 years old and 3 months, due to skin itching, abnormal liver function more than 3 years admitted to hospital. Children born after the parents found that children with itchy skin, local hospitals to check abnormal liver function, to the ursodeoxycholic acid drugs such as liver, reducing enzyme treatment, no improvement in symptoms, transaminases, bilirubin repeated abnormalities. Past no clear history of close contact with viral hepatitis, children with no history of blood transfusion and blood products, no history of dirty food. Physical examination: clear mind, body skin, sclera no yellow dye, dry skin, chest and back, visible limbs and more