原发性胆汁性肝硬化31例临床分析

Clinical analysis of primary biliary cirrhosis: a report of 31 cases

目的 对原发性胆汁性肝硬化患者的临床特征进行回顾性分析 ,以提高对该病诊治的认识。方法 分析 31例原发性胆汁性肝硬化患者的一般资料、临床表现、生化、免疫学及病理学等改变。结果 本组患者中女性 2 5例 ,确诊时的平均年龄为 (4 9.2± 10 .7)岁。症状以黄疸最为多见 (74 .2 % ) ,其次为皮肤瘙痒 (5 1.6 % )和乏力 (32 .3% ) ,3例患者 (9.7% )合并腹水。所有患者血清碱性磷酸酶、γ 谷氨酰转肽酶及胆红素水平明显升高 [分别为 (388.9± 2 77.5 )U/L、(381.6± 2 13.2 )U/L和 (176 .4± 176 .1) μmol/L],血清丙氨酸转氨酶 (ALT)、天冬氨酸转氨酶 (AST)水平呈轻至中度升高 [分别为(79.7± 4 6 .3)U/L及 (119.8± 6 1.2 )U/L],患者血清IgM升高 (3.0± 1.9)g/L ,行线粒体抗体检查者92 % (2 3/ 2 5 )阳性 ,6 1.3%患者熊去氧胆酸治疗短期内显示一定疗效。结论 原发性胆汁性肝硬化主要累及中年女性 ,血清碱性磷酸酶及γ 谷氨酰转肽酶水平升高、抗线粒体抗体阳性、血清IgM升高有助于诊断本病 。

Objective Clinical features of primary biliary cirrhrosis(PBC) were reviewed in order to improve its diagnosis and treatment. Method The general conditions, clinical manifestations, biochemical and immunological changes, and pathological findings were assessed in 31 patients. Result Twenty five cases were females, the mean age at definite diagnosis was (49.2±10.7)years. Jaundice(74.2%) was the most frequent symptoms, pruritus (51.6%) and fatigues (32.3%)were the second and thrid, respectively. Three patients (9.7%)were complicated by ascites. Serum alkline phosphatase (ALP) , glutamyl transpeptidase (γ GT) and bilirubin levels were markedly elevated ((388.9±277.5)U/L, (381.6±213.2)U/L and( 176.4 ±176.1)μmol/ L, respectively).ALT and AST levels were mildly or moderately elevated ((79.7±46.3) U/L and(119.8±61.2)U/L, respectively),mean level of IgM was also elevated to (3.0±1.9)g/L. 92% (23/25) of patients had positive anti mitochondrial antibody(AMA). Ursodeoxycholic acid (UDSA) was efficitive in 61.3% of patients. Conclusions PBC most frequently affects middle aged women. The elevated level of ALP, γ GT and IgM and AMA positive may be crucial to diagnosis of PBC. Liver biopsy can help to identify the diagnosis and carry on pathological staging.