60例原发性胆汁性肝硬化的临床病理观察

Clinical and pathological observation on 60 cases of primary biliary cirrhosis

目的分析原发性胆汁性肝硬化（PBC）的临床和病理特征有助于该病的早期诊断和治疗。方法回顾分析60例PBC患者资料。结果患者中女性47例，主要表现为黄疸、乏力、瘙痒。多数患者γ-谷氨酰转肽酶（GGT）与碱性磷酸酶（ALP）显著增高，血清丙氨酸氨基转移酶（ALT）、天冬氨酸氨基转移酶（AST）、总胆红素（TBil）、直接胆红素（DBil）不同程度升高；66．7％患者免疫球蛋白IgM增高；75％抗线粒体抗体M2亚型（AMA-M2）阳性，是PBC的重要特征。28．17％gp210阳性且男性略多。35例肝穿PBCⅠ-Ⅱ期16例，Ⅲ-Ⅳ期19例，统计学显示IgM在PBCⅠ-Ⅱ期增高明显（P〈0．05）。结论PBC中年女性多见，应结合临床、生化、免疫学及病理检查及时诊断，IgM有助于早期诊断。

Objective In order to improve the cognition and early diagnosis of primary billiary cirrhosis （PBC） ,we investigated clinical and biochemical characteristics of 60 PBC. Methods Clinical data of 60 PBC patients together with pathological characteristics 43 PBC patients were reviewed. Results Among the 60 PBC patients,47were women and the mean age at diagnosis was（54. 98 ± 13.05）years. The most frequent clinical presentations were jaundice （ 60% ）, fatigue （ 48. 3% ） pruritus （ 56.7% ）. Alkaline phosphatase （ALP） levels were markedly elevated in all patient, and gamma-glutamy transpeptidase（GGT） levels were elevated in 52 patients. Alanine aminotransferase （ALT） and aspartate aminotransferase （AST） were slight elevated in these patients. Total bilirubin（ TBil）, especial direct bilirubin （DBil）concentration were usually elevated distinctively in PBC. 66. 7% patients have an elevated IgM concentration. 75% AMA -M2 were positive and it is the character of PBC. 28.17% gp210 were positive and it seems to be found mostly in male. 35 cases received liver biopsy. 16 patient were in stage Ⅰ -Ⅱ ,others in stageⅢ-Ⅳ. We find IgM was benefit to early diagnosis of PBC. Conclusion PBC is mostly found in middle-aged women. Accurate and prompt diagnosis of PBC should be based on clinical presentation, biochemical and immunological changes. Liver biopsy is important to lean the degree of the disease. IgM will be benefit to early diagnosis.