新疆哈萨克族遗传性易栓症人群中活化C蛋白抵抗、蛋白C和蛋白S的联合缺陷

Combined defects of activated protein C resistance,protein C,and protein S in the the Xinjiang Kazakh hereditary thrombophilia crowd

目的:(1)探究哈萨克族人群中是否存在PS缺陷和APC-R并存的患者,该患者的血浆内PS活性与APC-R值是否存在相关性;(2)探究哈萨克族人群中是否存在PC缺陷和APC-R并存的患者,该患者的血浆内PC活性与APC-R值是否存在相关性。方法:通过横断面调查,选择新疆伊犁哈萨克自治州常驻哈萨克族人群,测定该健康人群PC、PS活性及APC-R。结果:共纳入1 005名哈萨克族健康志愿者(男性332名,女性673名),平均年龄(41.13±11.50)。通过研究得出:PS缺陷和APC-R并存的患者有32例,这些患者血浆中PS活性与APC-R值存在显著正相关;PC缺陷和APC-R并存的患者有27例,这些患者血浆中PC活性与APC-R值存在正相关。结论:在新疆哈萨克族联合缺陷患者中,APC-R与PS缺陷、APC-R与PC缺陷可能相互存在关联,共同作用于血栓性疾病的发生、发展。

Objective： （1）To explore whether there are patients with the combined defects of the the PS defect and APC-R in Hasake. Whether there is a con-elation of the PS activity and APC-R values in the plasma of patients ; （2） To ex- plore whether there are patients with the combined defects of the the PC defect and APC-R in Hasake. Whether there is a correlation of the PC activity and APC-R values in the plasma of patients. Methods：Through cress-sectional survey and se- lect Kazakh in the Ili Kazak Autonomous Prefecture Permanent , and determining PC, PS activity and the APC-R of the healthy population. Results：There are 1005 Kazak （332 male,673 female） ,mean age years （41.13 ＋ 11.50） in healthy volunteers. In this studies：There are 32 patients with co-existence of PS defects and APC-R,and there is a significant posi- tive con＇elation of PS activity and APC-R value in the plasma of these patients. Meanwhile, ：There axe 27 patients with co- existence of PC defects and APC-R ,and there is a positive correlation of PC activity and APC-R value in the plasma of these patients. Conclusion： APC-R and PS deficiency, APC-R and PC deficiency correlate with each other,play an important role in the occurrence and the development of thrombotic disease in patients with the combined clefeets in Hasake.