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目的分析温州市血友病患者因子Ⅷ、Ⅸ水平,因子Ⅷ抑制物,血液传播性疾病,抗凝物质及纤溶抑制物质水平及意义。方法对65例血友病患者检测、复测FⅧ、FⅨ、VWF,FⅧ抑制物,HBV、HCV、HIV、TPHA、TFPI;58例同时检测PC、FPS,AT,20例同时检测TAFIa水平。其中TFPI、PC、FPS,AT及TAFIa健康对照分别为20例及15例。FⅧ、FⅨ、FⅧ抑制物以凝血一期法检测;VWF、PC、FPS、AT、TFPI、HBV、HCV、HIV以ELISA法检测,AT以免疫发射比浊法检测;TAFIa以发色底物法检测;TPHA以凝集法检测。结果血友病A 54例,血友病B 11例,血友病A与血友病B比例为4.9∶1。FⅧ<1%41例(75.9%),1-5%6例(11.1%),>5%7例(12.9%)。FⅨ<1%6例(54.5%)。重型血友病共47例,占72.3%。VWF均在正常水平。FⅧ抑制物阳性3例(5.6%)。HBV阳性4例(6.1%),HCV阳性24例(36.9%),HIV及TPHA均阴性。与正常对照结果,其中AT,TFPI均值明显高于对照,P<0.05;TAFIa均值明显低于对照,P<0.001;PC、FPS均值P均>0.05,差异无显著性。结论我市血友病A型与B型之比例为4.9∶1,重型者占72.3%;FⅧ抑制物阳性者占5.6%;血液传播性疾病以丙型肝炎感染为主,阳性率为36.9%;抗凝物质AT及TFPI水平明显高于正常对照;TAFIa水平明显低于正常。提示AT、TFPI水平增高,TAFIa水平降低是血友病出血机制中的重要因素。
Objective To analyze the level and significance of factor Ⅷ and Ⅸ levels, factor Ⅷ inhibitor, blood-borne diseases, anticoagulants and fibrinolytic substances in patients with hemophilia in Wenzhou. Methods Sixty - five patients with hemophilia were tested for FⅧ, FIX, VWF, FⅧ inhibitors, HBV, HCV, HIV, TPHA and TFPI, and 58 cases of simultaneous detection of PC, FPS and AT were detected in 20 patients with hemophilia. TFPI, PC, FPS, AT and TAFIa healthy controls were 20 cases and 15 cases respectively. FⅧ, FIX and FⅧ inhibitors were detected by coagulation phase method; VWF, PC, FPS, AT, TFPI, HBV, HCV and HIV were detected by ELISA and AT by immunoexpneic turbidimetry; Detection; TPHA detected by agglutination. Results Hemophilia A 54 cases, hemophilia B 11 cases, hemophilia A and hemophilia B ratio 4.9: 1. FⅧ <1% in 41 cases (75.9%), 1-5% in 6 cases (11.1%) and> 5% in 7 cases (12.9%). FIX <1% in 6 cases (54.5%). Severe hemophilia in 47 cases, accounting for 72.3%. VWF are at normal levels. FⅧ inhibitor positive in 3 cases (5.6%). Four were HBV positive (6.1%), 24 were HCV positive (36.9%), and both HIV and TPHA were negative. Compared with the control group, the average values of AT and TFPI were significantly higher than those of the control group, P <0.05; mean of TAFIa was significantly lower than that of the control, P <0.001; Conclusion The ratio of hemophilia A to B in our city is 4.9: 1, accounting for 72.3% for heavy type, 5.6% for FⅧ inhibitor, and the rate of hepatitis C infection in blood-borne diseases is 36.9% ; AT and TFPI levels of anticoagulant substances were significantly higher than the normal control; TAFIa levels were significantly lower than normal. Tip AT, TFPI levels increased, decreased levels of TAFIa hemophilia bleeding mechanism is an important factor.