易栓症——神内病例.ppt
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1、易栓症,Normal hemostasis(止血),adhesion 粘附,aggregation 聚集,coagulation pathway 凝血途径,Coagulation balance,anticoagulant mechanisms,procoagulant mechanisms,fibrin formation,Fibrin monomer,Endothelial cell surface,coagulation factors anticoagulant proteins gain-of-function mutations fibrinolysis antiphospholi
2、pid Ab,fibrin formation,anticoagulant mechanisms,procoagulant mechanisms,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,rate ratio (vs white
3、) African Americans 1.27 (1.07-1.51) Hispanic 0.60 (0.54-0.67) Asians/Pacific Islanders 0.26 (0.22-0.30)* White et al, Ann Intern Med 1998,*idiopathic or secondary,VTE: a multifactorial disease,acquired,genetic,mixed,transient,VTE,risk factors, the old story.,deficiency of anticoagulant proteins yea
4、r of discovery antithrombin 1965 protein C 1981 protein S 1984,ANTITHROMBIN, PROTEIN C, PROTEIN S: type of deficiency,Type I: Quantitative deficiency,Type II: Qualitative deficiency, antigenic test, functional test, functional test,= antigenic test,Prevalence of deficiencies,general unselected popul
5、ation 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,year of discovery resistance to activated protein C 1993/94 and factor V Leiden (G1691A
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