48例原发性胆汁性肝硬化的临床资料分析

Clinical characteristics of primary biliary cirrhosis : A report of 48 cases

目的 分析原发性胆汁性肝硬化 (primarybiliarycirrhosis ,PBC)患者的临床特征 ,以指导对该病的诊断和治疗。方法 对 48例PBC患者的一般资料、病程、临床表现、血清化学指标、免疫学及病理学改变等进行回顾性分析。结果 本组患者中女性 44例 ,女 :男比例为 11:1,确诊时年龄 2 6～ 82岁 ( 5 4.2 3± 12 .5岁 ) ,初诊至确诊的时间平均为 46.69月 ( 10天～ 2 64月 )。伴有迁延性丙氨酸氨基转移酶、天门冬氨酸氨基转移酶异常、高胆红素血症、高碱性磷酸酶、γ 谷氨酰基转移酶血症、高球蛋白血症 ,存在自身抗体 ,93 .7%的患者抗线粒体抗体和 /或抗线粒体M2亚型抗体阳性。结论 PBC在女性多见 ,肝脏损伤严重 ,确诊时基本至晚期 。

ObjectiveTo study the clinical features of primary biliary cirrhosis (PBC) in order to facilitate recognition of the disease. MethodsGeneral status, clinical manifestations and laboratory findings of 48 patients with PBC were reviewed. ResultsAmong the 48 patients ,44 were females with a female/male ratio of 11:1 and the age range was 26～82 years old (mean 54.23±12.5岁). The mean time interval between the first visit to physicians to the time of diagnosis was about 46.69 months (10 days～264mon). There was elevated level of serum ALT and AST, hyperbilirubinemia, hyperglobulimenia, with predominant elevation of ALP, GGT and different kinds of auto-antibodies, for example AMA, AMA-M2.ConclusionPBC affects females clinically and has severe liver impairment.