53例原发性胆汁性肝硬化临床和病理分析

目的分析原发性胆汁性肝硬化（PBC）患者的临床表现、实验室检查及病理特征。方法回顾性分析53例PBC患者的临床表现、实验室检查及病理特征。结果53例PBC患者中女性46例（86．8％），发病至确诊时间（中位数）28个月，主要症状包括反复肝功能异常、乏力、黄疸、瘙痒、肝脾肿大等；53例患者GGT、ALP均有明显升高；73．6％（39／53）患者AMA和（或）AMA-M2亚型阳性，另14例血清AMA和AMA-M2亚型阴性，经病理检查证实为PBC；53例患者中Ⅰ期为13例（24．5％），Ⅱ期20例（37．7％），Ⅲ期16例（30．2％），Ⅳ期4例（7．5％）；Ⅰ期、Ⅱ期（33例）与Ⅲ期、Ⅳ期（20例）患者肝功能等计量资料只有GGT、ALP差异有显著性意义（P〈0．05），病理改变严重者GGT、ALP升高明显。结论临床判断PBC患者病情的轻重应综合分析临床症状、生化改变（尤其是GGT、ALP）、自身抗体检测以及组织学检查等，而不应局限于用黄疸作为衡量指标。

Clinicopathological analysis of patients with primary biliary cirrhosis

Objective To analyze the clinical features,laboratory tests and the pathological changes of patients with primary biliary cirrhosis. Methods A retrospective study was designed to analyze the clinical manifestation,the laboratory tests and the pathological changes of the 53 PBC patients. Results Of the 53 cases,46 （86.8%）were females. The median times from the onset of illness to diagnosis was 28 months. The main clinical manifestations included repeated hepatic dysfunction,hypodynamia,jaundice,itching,hepatosplenomegaly,etc. GGT and ALP were abnormal;73.6%（39/53）of patients＇ AMA and（or）AMA-M2 were positive,while another 14 patients＇AMA and AMA-M2 negative. All were diagnosed as PBC by biopsy of liver. The pathological changes were as follows：stage Ⅰ（13cases）,stage Ⅱ （20cases）,stage Ⅲ（16casses）and stage Ⅳ（4cases）. The patients were pathologically divided into stage Ⅰ or Ⅱ （n=33）and stage Ⅲ or Ⅳ（n=20）. The GGT and ALP levels were found significantly different between the two groups （P〈0.05）. Patients＇GGT and ALP were higher when their pathological changes were severe. Conclusion Diagnosis of PBC should be made not only by the serum bilirubin levels,but also by the clinical features and laboratory（especially GGT,ALT,auto-antibody）as well as histopathology examination.