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55例患者中女48例,男7例,平均年龄49岁(26～69岁)。14例在随访期间死亡者,其平均存活期(自诊断后算起)为6.6年。67％患者有搔痒和乏力症状,9例(16％)无症状但有血液生化改变。通过分析,未发现症状与预后相关。肝脾大小和初期有无黄疸,也与存活期无关。实验室检查:44例(80％)抗线粒体抗体阳性(间接免疫荧光法),全部患者起病时有硷性磷酸酶(ALP)和谷氨酸转肽酶(γ-GT)异常,48例(87％)谷草转氨酶升高,30例(54％)胆固醇升高,与预后均无相关性。初次就诊时,38例(69％)患者胆红素浓
Among the 55 patients, 48 were female and 7 were male, with an average age of 49 years (range 26-69 years). Fourteen patients who died during follow-up had an average survival of 6.6 years (since the diagnosis). Pruritus and fatigue symptoms were present in 67% of patients, and asymptomatic but biochemical changes in blood in 9 patients (16%). By analysis, no correlation between prognosis and symptoms was found. Liver and spleen size and initial jaundice, but also has nothing to do with survival. Laboratory tests: 44 (80%) were positive for anti-mitochondrial antibodies (indirect immunofluorescence), all patients had ALP and γ-GT abnormalities, and 48 (87%) aspartate aminotransferase increased, 30 cases (54%) increased cholesterol, and no correlation with prognosis. At initial visit, 38 patients (69%) had bilirubin concentrations