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目的观察原发性胆汁性肝硬化(PBC)的临床、生化特点,进一步提高对本病的认识。方法对52例原发性胆汁性肝硬化患者的一般资料、临床表现、生化特点进行分析。结果本组患者中女47例,男女比例为1∶9,确诊时平均年龄(54.6±8.9)岁,发病至确诊时间2～184个月,其中表现乏力41例(78.8%),黄疸40例(76.9%),皮肤瘙痒22例(42.3%),肝肿大24例(46.2%),脾肿大28例(53.8%)。血清丙氨酸氨基转移酶和天冬氨酸氨基转移酶轻中度升高[ALT(75.9±41.8)IU/L,AST(90.2±54.7)IU/L],血清谷氨酰转肽酶和碱性磷酸酶明显升高[GGT(283.6±186.5)IU/L,ALP(381.9±227.6)IU/L],血脂水平[TC(5.3±1.7)mmol/L,TG(1.6±0.7)mmol/L,LDL(2.8±0.5)mmol/L]升高,在疾病早期尤为明显。其中43例患者(82.7%)血清IgM升高,48例患者(92.3%)抗线粒体抗体(AMA)和AMA-M2阳性。结论我国原发性胆汁性肝硬化多见于中年女性,最常见的表现是乏力、黄疸。血清ALP、GGT、血脂水平升高为本病特点,AMA和AMA-M2亚型阳性有助于本病的诊断。
Objective To observe the clinical and biochemical characteristics of primary biliary cirrhosis (PBC) and further improve the understanding of this disease. Methods 52 cases of primary biliary cirrhosis in patients with general information, clinical manifestations, biochemical characteristics were analyzed. Results 47 patients were female, the ratio of male to female was 1: 9, the mean age at diagnosis was (54.6 ± 8.9) years old, and the time from onset to diagnosis was 2 to 184 months, 41 cases (78.8% (76.9%), pruritus in 22 cases (42.3%), hepatomegaly in 24 cases (46.2%) and splenomegaly in 28 cases (53.8%). Serum alanine aminotransferase and aspartate aminotransferase slightly increased moderately [ALT (75.9 ± 41.8 IU / L, AST (90.2 ± 54.7) IU / L], serum glutamyl transpeptidase and ALT (381.9 ± 227.6) IU / L], blood lipid levels [TC (5.3 ± 1.7) mmol / L and TG (1.6 ± 0.7) mmol / L, LDL (2.8 ± 0.5) mmol / L] increased in the early stages of the disease is particularly evident. Serum IgM was elevated in 43 patients (82.7%), and positive in 48 patients (92.3%) with anti-mitochondrial antibody (AMA) and AMA-M2. Conclusion Primary biliary cirrhosis in our country is more common in middle-aged women, the most common manifestation is fatigue, jaundice. Serum ALP, GGT, elevated blood lipid levels for the characteristics of this disease, AMA and AMA-M2 subtype positive contribute to the diagnosis of the disease.