TY - T1的血栓形成的危险因素在肺动脉高压JF -欧洲呼吸杂志》乔和J SP - 395 LP - 399欧元六世- 15 - 2非盟-狼,米非盟- Boyer-Neumann C AU -父母,F AU - Eschwege, V盟——Jaillet H AU -迈耶维盟——Simonneau在慢性血栓栓塞性肺动脉高压(CTEPH)和原发性肺动脉高压(PPH)中均可观察到血栓性病变。然而，血栓形成是否与抗血栓通路缺陷或先前的血管损伤有关尚不清楚。因此，本研究分析了CTEPH和PPH中遗传和获得性血栓形成危险因素的频率。在作者的研究机构中，147例连续CTEPH患者与99例连续PPH患者进行了比较。在116例CTEPH患者和83例PPH患者中，采用免疫和凝血两种方法分析了磷脂依赖抗体(抗磷脂抗体和狼疮抗凝剂)。自1994年以来入组的患者(46 CTEPH和64 PPH)也测定了遗传性血栓危险因素。采用功能测定法测定抗凝血酶、蛋白C和蛋白S活性。通过聚合酶链反应鉴定了因子V和因子II的突变。PPH或CTEPH患者的遗传性血栓危险因素患病率均未增加。 In contrast, a high frequency of phospholipid-dependent antibodies was observed in PPH (10%) and more notably in CTEPH (20%). Moreover, in PPH, antibodies were present only in low titre whereas in CTEPH, half of the patients with antiphospholipid antibodies had high titres. In addition, in CTEPH all but one of the patients with lupus anticoagulant also had antiphospholipid antibodies. The most striking finding of this study was the high prevalence of phospholipid-dependent antibodies but their clinical relevance appears to be different in primary pulmonary hypertension and chronic thromboembolic pulmonary hypertension. In primary pulmonary hypertension, these antibodies in low titre probably reflect endothelial dysfunction. In contrast, in chronic thromboembolic pulmonary hypertension the presence of antibodies in high titre associated with lupus anticoagulant, underlines the role of thrombosis in the pathogenesis of this condition. ER -