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迄今为止,原发性胆汁性肝硬化(PBC)的诊断依据是组织学检查发现有慢性非化脓性毁坏性胆管炎和抗线粒体抗体(AMA)阳性。这些患者常伴有血清γ-球蛋白、IgM和胆固醇升高。然而约5%病人的肝脏生化学和组织学特征与PBC相符,而AMA检测阴性。但在20% 经组织学和血清学诊断为自身免疫性慢性肝炎的患者中,AMA可呈阳性,提示AMA并非特异性。 作者等复习了Mayo医院诊断为PBC的597例患者,其中35例(5.8%)均经常规免疫萤光检测AMA阴性,内镜逆行胆管造影正常均为女性。为此,作者回顾性分析了AMA阳性和AMA阴性两组病人的血清AST、ALP、总胆红素、血清γ-球蛋白、免疫球蛋白(IgA、IgM和IgG)和血脂(总胆固醇和甘油三酯)浓度,以及比较了两组间的抗核抗体(ANA)和抗平滑肌抗体(SMA)的阳性率,结果如下。 AMA阳性和阴性组之间在年龄、血清AST、ALP、总胆红素、血清IgA和IgG、胆固醇或甘油三酯上均无差异,但IgM水平在AMA阳性组为584±383mg/dl,而AMA阴性组为368±249 mg/dl(P<0.002);γ-球蛋白在AMA阳性组为1.91±0.55,而AMA阴性组为1.80±0.85(P<0.03)。35例AMA阴性病人中,有29例测定了血清ANA,16例测定了血清SMA;在AMA阳性组测定的91例中有33例(36%)ANA阳性,而29例AMA阴性组中,23例(79%)ANA阳性(P<0.0001)。37例AMA?
So far, the diagnosis of primary biliary cirrhosis (PBC) is based on the histological examination of chronic non-suppurative destructive cholangitis and anti-mitochondrial antibody (AMA) positive. These patients often accompanied by serum γ-globulin, IgM and elevated cholesterol. However, about 5% of patients have liver biochemical and histological features consistent with PBC, whereas AMA negative. However, AMA was positive in 20% of histologically and serologically diagnosed patients with autoimmune chronic hepatitis, suggesting that AMA is not specific. The authors reviewed 597 patients diagnosed with PBC at Mayo Hospital, 35 of whom (5.8%) were routine immunofluorescent AMA-negative and endoscopic retrograde cholangiography was normal. To this end, the authors retrospectively analyzed serum AST, ALP, total bilirubin, serum gamma globulin, immunoglobulins (IgA, IgM and IgG) and blood lipids (total cholesterol and glycerol) in both AMA positive and AMA negative patients Triester) concentrations, and comparing the positive rates of anti-nuclear antibody (ANA) and anti-smooth muscle antibody (SMA) between the two groups, the results are as follows. There were no differences in age, serum AST, ALP, total bilirubin, serum IgA and IgG, cholesterol or triglycerides between AMA positive and negative groups, but IgM levels were 584 ± 383 mg / dl in the AMA positive group The AMA negative group was 368 ± 249 mg / dl (P <0.002). The γ-globulin was 1.91 ± 0.55 in the AMA positive group and 1.80 ± 0.85 in the AMA negative group (P <0.03). Serum ANA was detected in 29 of 35 AMA-negative patients and serum SMA was measured in 16 of the 30 AMA-negative patients; ANA was positive in 33 (36%) of the 91 AMA-positive patients and in 29 AMA-negative patients Cases (79%) ANA positive (P <0.0001). 37 cases of AMA?