原发性胆汁性肝硬化114例

目的:总结原发性胆汁性肝硬化(primary bili-ary cirrhosis,PBC)的特征,探讨血清抗线粒体抗体(antimitochondrial antibody,AMA)及分型对疾病的诊断意义及病情评价的临床意义.方法:对114例PBC患者的临床表现、血清生化指标、血清抗体及病理学特征等进行回顾性的分析.通过在M2抗体阳性者中,根据M4及M9是否阳性进行分组,即分为单纯M2阳性者、M2-M9阳性者、M2-M4阳性者及M2-M4-M9阳性者4组,并比较各组间的血清学指标及其并发症.结果:PBC临床表现主要为乏力、黄疸、皮肤瘙痒等;生化学主要以碱性磷酸酶(alkalinity phosphatase,ALP)、-谷氨酰转肽酶(-glutamyl transpeptadase,GGT)升高为主,伴有谷丙转氨酶(alanine transaminase,ALT)、谷草转氨酶(automatic spanning tree,AST)及总胆红素(totalbilirubin,TBIL)不同程度升高;血清免疫球蛋白以IgM升高为主;血清抗体中AMA及M2型抗体对该病的诊断具有较高的特异性及敏感性.通过分组比较发现M2-M4阳性者ALP及IgM高于单纯M2阳性者,P值分别为0.010、0.014,M2-M4-M9阳性者AST、TBIL及IgG高于M2-M9阳性者,P值分别为0.039、0.016、0.039;M2-M4-M9阳性者TBIL明显高于单纯M2阳性者,P=0.023,单纯M2阳性者IgG高于M2-M9阳性者,P=0.031,上述差异均有统计学意义(P<0.05).故血清M4、M9抗体对该病的病情判断具有一定的临床意义.结论:掌握PBC的特征,做到早诊断早干预,不仅延缓病程的进展,还能改善预后.

Clinical features of primary biliary cirrhosis:An analysis of 114 cases

AIM:To summarize the clinical characteristics of primary biliary cirrhosis(PBC) and to explore the clinical significance of serum anti-mitochondrial antibodies(AMAs) in the diagnosis and assessment of severity of PBC.METHODS:A retrospective analysis of clinical manifestations,serum biochemical parameters,serum antibodies and pathological features was performed in 114 patients with PBC.M2 antibody-positive patients(105 cases) were divided into four groups:those positive for only M2(61 cases),for both M2 and M9(9 cases),for both M2 and M4(25 cases),and for M2,M4,and M9(10 cases).Serological markers and complications were compared among the four groups.RESULTS:Main clinical manifestations of PBC include fatigue,jaundice,and skin itching.The levels of alkalinity phosphatase(ALP),-glutamyl transpeptidase(GGT),alanine aminotransferase(ALT),aspartate aminotransferase(AST),and total bilirubin(TBIL) increased to varying degrees.Serum AMA and M2 antibodies had a very high specificity and sensitivity in diagnosing PBC.Compared to the M2-positive group,the levels of ALP and IgM in M2-M4positive group were higher(P = 0.010,0.014,P < 0.05).Compared to the M2-M9-positive group,the levels of AST,TBIL,and IgG in the M2M4-M9 positive group were higher(P = 0.039,0.016,0.039,P < 0.05).Compared to the M2-positive group,the level of TBIL in the M2-M4-M9 positive group was higher(P = 0.023,P < 0.05).Compared to the M2-M9 positive group,the level of IgG in the M2-positive group was higher(P = 0.031,P < 0.05).Thus,serum M4 and M9 levels have clinical significance in the diagnosis of PBC.CONCLUSION:Understanding the clinical characteristics of PBC can help improve the diagnosis,treatment,and prognosis of this disease.