CAJ | 학술논문

易栓症是临床常见的多病因疾病，致死致残率高。从遗传性凝血、抗凝纤溶异常的角度去探讨其病因和发病机制，对于该病的预防十分重要。遗传性易栓症包括活化蛋白C抵抗（ APCR）、抗凝血酶缺陷症、蛋白C、蛋白S缺陷症、凝血因子Ⅴ Leiden和凝血酶原G20210A突变等。部分遗传性易栓症患者可能存在蛋白S缺陷和APCR两种缺陷症并存的可能即联合缺陷。该文对蛋白 C、蛋白S、APCR在易栓症中的联合缺陷抗凝体系予以综述。
역전증시림상상견적다병인질병，치사치잔솔고。종유전성응혈、항응섬용이상적각도거탐토기병인화발병궤제，대우해병적예방십분중요。유전성역전증포괄활화단백C저항（ APCR）、항응혈매결함증、단백C、단백S결함증、응혈인자Ⅴ Leiden화응혈매원G20210A돌변등。부분유전성역전증환자가능존재단백S결함화APCR량충결함증병존적가능즉연합결함。해문대단백 C、단백S、APCR재역전증중적연합결함항응체계여이종술。
Thrombophilia is a common clinical disease of multiple etiology,with high disability rate and mortality.From the perspectives of hereditary coagulation,anticoagulation and fibrinolysis abnormalities to investigate the etiology and pathogenesis of thrombophilia is very important to the prevention of the disease . Hereditary thrombophilia includes activated protein C resistance(APCR),antithrombin deficiency(AT.Ⅲ), protein C and protein S deficiencies,Factor V Leiden and prothrombin G20210A mutations and so on.There may be joint defects of APCR and protein S deficiency in some cases of hereditary thrombophilia .Here is to make a review of the anticoagulation system of joint defects of protein C,protein S,APCR in thrombophilia.