ty -jour t1-肺动脉高压JF中的血栓形成危险因素 - 欧洲呼吸杂志au -eschwege，v au -jaillet，h au -meyer，d au -simonneau，g y1-2000/02/01 ur -http：//www.qdcxjkg.com/content.com/content/15/2/2/2/395.abtract-395.abstract N2--在慢性血栓栓塞性肺动脉高压（CTEPH）中始终观察到血栓性病变，并在原发性肺动脉高压（PPH）中经常发现。然而，血栓形成是与抗血栓形成途径的缺陷有关还是与以前的血管损伤有关。因此，这项研究分析了CTEPH和PPH中遗传性和获得性血栓形成危险因素的频率。将在作者机构进行了一百四十七名连续研究的CTEPH患者与连续99例PPH患者进行了比较。在116名CTEPH患者和83例PPH患者中，通过免疫学和凝血测定法分析了磷脂依赖性抗体（抗磷脂抗体和狼疮抗凝剂）。自1994年以来入学的患者（46个CTEPH和64个PPH），还确定了遗传性血栓形成危险因素。抗凝血酶，蛋白C和蛋白质的活性通过功能测定测量。通过聚合酶链反应鉴定出因子V和因子II的突变。 The prevalence of hereditary thrombotic risk factors was not increased in patients with either PPH or CTEPH. 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 -