【摘要】 目的检测血友病患者凝血酶激活的纤维蛋白溶解抑制物活性(TAFIa)水平,探讨其临床意义。方法对20例血友病患者(血友病A15例,血友病B 5例)及15例正常人对照以发色底物法检测TAFIa水平,对比二者的差异。按临床出血程度将年出血次数≤2次,无关节畸形者列为临床轻型组(n=8),年出血次数>2次,且有严重关节畸形者列为临床重型组(n=12),比较二组的TAFIa水平。结果血友病组和正常对照组TAFIa均值分别为86.00±15.67%及107.00±9.63%,二组比较P<0.001,差异有显著性。血友病A和血友病B TAFIa均值分别为87.73±15.50%及80.80±16.72%,二组之间P>0.05,差异无显著性。二组与正常对照P值均<0.001,有显著差异。TAFIa值与临床出血关系:8例轻型组者均值为81.38±15.85%,12例重型组者为87.41±16.04%,两组比较P>0.05,差异无显著性。结论血友病患者TAFIa水平显著低于正常对照,表明其下调纤溶机制显著减弱,导致纤溶活性增强,促进了血友病患者的出血倾向。提示抗纤溶治疗在血友病出血治疗中占有重要位置。未证明血友病患者临床出血严重程度与TAFIa水平相关。更多还原

【Abstract】 Objective To detect the levels of TAFIa for exploring the activity of fibrinolysis in the patients with hemophilia.Methods The levels of TAFIa were measured by the synthetic chromogenic substrate method in 15 patients with hemophilia A,5 patients with hemophilia B and 15 healthy controls.The patients with hemophilia were divided into two groups,according to the risk of bleeding: the mild bleeding group(≤2 bleeds/year,absence of joint deformity;n=8);the severe bleeding group(>2 bleeds/year,with severe joint deformity;n=12).The levels of TAFIa in both group were compared.Results A statistically significant decrease in TAFIa was found in the patients with hemophilia compared to the controls(86.00±15.67%;107.00±9.63%,P<0.001).There was no difference in the TAFIa levels between the patients with hemophilia A and hemophilia B(87.73±15.50%;80.80±16.72%,P>0.05).No significant difference was observed between the mild bleeding group and the severe bleeding group in the levels of TAFIa(81.38±15.85%;87.41±16.04%,P>0.05).Conclusion The levels of TAFIa in the patients with hemophilia were significantly decrease as compared with the controls and the results showed that the hemophilia patients had a defect of down regulation fibrinolysis and the drug of antifibrinolysis could be used in the treatment of bleeding.There was no difference between the group with mild bleeding and with severe bleeding in the levels of TAFIa.更多还原