摘要目的 综合分析国内对原发性胆汁性肝硬化(PBC)的临床研究情况,了解我国PBC患者的临床特点.方法 计算机检索已发表的国内PBC患者相关临床文献,汇总临床及预后资料,采用RevMan 4.2软件对相关病例对照研究进行Meta分析.结果 ①纳入91篇文献,观察2315例PBC患者.②临床症状以乏力多见(54.54%);抗线粒体抗体(AMA)阳性率范围74.1%～100%,其中AMA-M2阳性率范围45%～83%;抗核抗体(ANA)阳性率范围20%～83.78%,核膜型多见38.65%;免疫球蛋白以IgM[(2.8±0.7)～(7.3±5.1)g/L]、IgG[(16.5±4.9)～(20.5±5.9)g/L]升高为主;病理分期以Ⅱ期常见(149例,占36.61%);干燥综合征(SS)为最常见合并症(范同1.96%～34.61%);至随访期(5～96个月)结束,3.80%(52/1370)患者死亡.③Meta分析结果显示:AMA阳性与阴性组患者碱性磷酸酶(ALP)、丙氨酸转移酶(ALT)、天冬氨酸转氨酶(AST)、总胆红素(TBil)、谷氨酰转肽酶(GGT)、IgG指标及性别比较差异无统计学意义,IgM值在阴性组更低;熊去氧胆酸治疗后患者ALP水平下降;老年患者ALP、TBil水平偏低,但病死率高.结论 国内PBC患者大部分临床特点与国外报道相似,病例对照研究样本量较少、缺乏高质量的双盲、随机、对照研究是国内临床研究的缺陷.

abstractsObjective To investigate the clinical features of patients with primary biliary cirrhosis (PBC) in China. Methods The reported articles about clinical analysis of patients with PBC in China were searched. The quality of included studies was critically evaluated. Meta-analysis was performed using RevMan 4.2 software about controlled trials. Results ① Ninety-one literatures including 2315 patients wuth PBC were included. ②The common symptoms in PBC were fatigue (54.54％). AMA was found in 74.1％～100％ of patients with PBC, as well as the prevalence rate of anti-M2 range from 45％ to 83％. ANA antibodies present in 20％～83.78％ of patients, the most common antinuclear patterns were nuclear-envelope (38.65％).Increased levels of IgM [(2.8±0.7)～(7.3±5.1) g/L], IgG [(16.5±4.9)～(20.5±5.9) g/L] were found in these patients, and the most common liver histologic classification was type Ⅱ (36.6％). Sjogren's syndrome occured significantly more frequently in PBC (1.96％～34.61％). To the end of follow-up period (five months to nintysix months ), 3.80％ of patients were dead. ③ Meta-analysis performed in several case control studies, showed no significant differences was found in liver tests including of alkaline phosphatase (ALP), alanine aminotransferase (ALT), aspartate aminotrans ferase (AST), total bilirubin (TBil), or gamma-glutamyltransferase (GGT), IgG levels between AMA positive groups and AMA negative groups. However, lower levels of IgM presented in the latter. While it also showed that levels of ALP decreased after ursodeoxycholic acid (UDCA)therapy. The levels of TBil were lower in the elderly than in younger group, but the mortality ratio for liver diseases was higher in the former. Conclusion The clinical characteristics of the present series in China are mostly similar to those reported in other countries. Because of the low quality and the small number of included studies, larger sample-size, randomized, double-blinded controlled trials are needed.