易栓症神内病例课件.ppt

易栓症 fibrin formation coagulation factors anticoagulant proteins gain-of-function mutations fibrinolysis antiphospholipid Ab 易栓症（Thrombophlilia） 指由于抗凝蛋白、凝血因子、纤溶蛋白等的遗传性或获得性缺陷，或存在获得性危险因素，而容易发生血栓栓塞的疾病状态。 不是单一的疾病。 血栓栓塞类型：静脉血栓栓塞 Annual incidence of DVT  Whites 0.8-1.2 x 1000 Hong Kong Chinese 0.16 x 1000 Liu et al, Hong Kong Med J 2002 VTE: a multifactorial disease Genetic risk factors … the old story. Prevalence of deficiencies general unselected population VTE patients antithrombin 0.02 - 0.2 % 1 % protein C 0.1 - 0.5 % 3 % protein S ? 1 - 2 % Risk of VTE associated with deficiencies increase relative risk antithrombin 5 - 50 protein C 7 - 15 protein S 6 - 10 Genetic risk factors … the old story. Age and VTE Cancer and VTE … the old story. FURY F：family history（尤其是强家族史，即家族中有至少两例同类型血栓患者） U：unusual locations of thrombosis（腹腔、颅内等部位） R：recurrent episodes of thrombosis Y：year（45y） 符合上述任1条均建议筛查易栓症指标 The balance hangs down In Western Countries, the incidence of VTE is approximately 1 per one thousand persons per year, but it varies among different populations. As you can see, the incidence of DVT is definitely lower in Asians than in other ethnic groups. Not only incidence of DVT (either idiopathic or secondary to various transient risk factors such as …) but this study showed that also mortality rate from PE appears to be markedly lower in asian/pacific Islanders than in African Americans and whites. In general, blood flowing in the vessel. when the vessel is damaged, the fibrin under the endothelial is exposed. Then the fibrinogen and von Willebrand factor facilitate the platelet adhesion. Following the adhesion, the platelet are stimulated to re