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目的:分析原发性胆汁性肝硬化(PBC)和自身免疫性胆管炎(AIC)患者的临床表现、肝组织病理学特征及其与HLA-DRB1等位基因的相关性.方法:回顾性分析PBC患者94例,筛选出13例AIC、9例PBC和自身免疫性肝炎(AIH)重叠综合征(PBC/AIH重叠综合征),分析AIC、PBC/AIH重叠综合征和单纯PBC患者的临床表现和肝组织病理学特征,采用序列特异性多聚酶链反应(PCR-SSP)进行HLA-DRB1等位基因分析.结果:AIC和单纯PBC比较,在年龄、性别、血生化及肝组织病理学上不能区分二者,但是AIC患者治疗前AIH计分(8.8±0.9vs4.6±0.8,t=17.45,P<0.01)、ANA和/或SMA阳性率(84.6%vs22.2%,c2=17.0039,P<0.01)明显高于单纯PBC.AIC患者血清ALT、AST、IgG明显低于PBC/AIH重叠综合征患者.PBC患者的HLA-DRBl*08基因频率比健康对照组的显著增高(9.7%vs3.3%,RR=4.42,P<0.05),而比AIC患者有增高的趋势.结论:AIC可能仅是PBC的一种变异形式,中国汉族PBC的发病与HLA-DR8相关.
Objective: To analyze the clinical manifestations, liver histopathological features and its association with HLA-DRB1 alleles in patients with primary biliary cirrhosis (PBC) and autoimmune cholangitis (AIC) .Methods: A retrospective analysis Ninety-four PBC patients were screened for 13 cases of AIC, 9 cases of PBC and autoimmune hepatitis (AIH) overlap syndrome (PBC / AIH overlap syndrome), clinical manifestations of AIC, PBC / AIH overlap syndrome and PBC alone And HLA-DRB1 alleles were analyzed by sequence-specific polymerase chain reaction (PCR-SSP) and liver histopathological features.Results: AIC and pure PBC in age, sex, blood biochemistry and liver histopathology can not However, the AIH score of AIC patients before treatment was 8.8 ± 0.9 vs 4.6 ± 0.8, t = 17.45, P <0.01. The positive rates of ANA and / or SMA were 84.6% vs22.2%, c2 = 17.0039, P <0.01) was significantly higher than that of PBC alone.The serum levels of ALT, AST and IgG in patients with AIC were significantly lower than those in patients with PBC / AIH overlap syndrome.The frequency of HLA-DRB1 * 08 gene in PBC patients was significantly higher than that in healthy controls (9.7% vs3 .3%, RR = 4.42, P <0.05), but higher than those in AIC.Conclusion: AIC may be only a variant form of PBC. The incidence of PBC in Chinese Han and HLA-DR8 related.