TY - JOUR T1 -特发性肺动脉高压弥散能力严重降低:病人特点和治疗反应JF -欧洲呼吸杂志》乔和J SP - 1575 LP - 1585欧元——六世- 42是10.1183/09031936.00184412 - 6盟之旅,Pia AU - Nossent,以斯帖J . AU -德曼,弗朗西丝·s . AU - van den伯克,英奇A.H.盟——Boonstra Anco盟——Groepenhoff赫尔曼AU -雷特,爱德华·m . AU - Westerhof尼科AU -格伦伯格,Katrien盟——Bogaard Harm-Jan AU - Vonk-Noordegraaf,一组特发性肺动脉高压(IPAH)患者肺对一氧化碳的扩散能力(DLCO)严重降低，预后不良。它们的特征目前尚不清楚。本研究的目的是对比重度缩小和保留更多DLCO的ipah患者的临床特征和治疗反应。回顾性研究纳入了166例IPAH患者，并根据DLCO截断值45% pred进行分组(IPAH<45%和IPAH≥45%)。比较两组患者的临床特点、治疗效果及生存期。45%的患者年龄较大，多为男性，有更频繁的冠状动脉疾病病史，接触烟草较多。1 s用力呼气量(FEV1)、FEV1/用力肺活量、总肺活量和肺泡容积值略低，ct扫描异常在低DLCO患者中更为常见。年龄和包龄数与DLCO和lt;45% pred独立相关。 IPAH<45% showed no different haemodynamic profile, yet worse exercise performance and a worse survival rate, which were both related to age, sex and the presence of coronary disease. To conclude, a severely reduced DLCO in IPAH is associated with advanced age and a greater tobacco exposure. These patients have a worse exercise performance despite a similar hemodynamic profile. We confirm the decreased survival in this patient group and now show that this poor outcome is related to age, sex and the presence of coronary disease. Severely reduced DLCO in IPAH is associated with advanced age and a greater tobacco exposure http://ow.ly/pkQ5F ER -