48例原发性胆汁性肝硬化临床分析

目的：分析原发性胆汁性肝硬化（PBC）患者的临床表现，试验室检查及治疗情况。方法：回顾性分析48例PBC患者临床资料。结果：93．8％是中年女性患者，平均年龄53．2±8．73。主要的临床表现包括肝功能异常（ALT、GGT、AKP升高）95．8％，乏力纳差83．3％，黄疸79．2％，瘙痒66．6％，肝肿大62．5％，脾大58．3％等。常合并干燥综合征（25％），类风湿关节炎（16．7％）等自身免疫性疾病及结缔组织疾病。所以患者AMA及AMA—M2均为阳性。全部病例使用熊去氧胆酸治疗，但仅31．3％患者病情有不同程度好转。结论：加强对PBC的认识，重视对AMA或AMA—M2的检测，尤其对长期不明原因肝功能异常的女性患者。

Clinical Aanalysis of 48 Patients with Primary Biliary Cirrhosis

Objective： To evaluate the clinical features, laboratory tests and the treatment of patients with primary biliary cirrhosis（PBC）. Methods.. The clinical data of 48 cases were analyzed retrospectively. Results： Of the 48 cases （93.8 % ） were middle-aged＇females. The main clinical manifestations included hepatic dysfunction, hypodynamia, jaundice,itching, etc. All of patients＇ AMA and（or） AMA-M2 were positive. The effects of treatment with ursodeoxycholic acid（UDCA, 10-15mg/kg）were poor. Conclusion：The awareness to recognize PBC is important in making an early diagnosis and treatment. The positive AMA/AMA-M2 is valuable to confirm the diagnosis of PBC for middle-aged women with hepatic dysfunction of unknown origin.