@Article {Shlobin1901747，作者= {舍氏岛，oksana A.和Kouranos，Vasilis和Barnett，Scott D.和阿尔马德，ESAM H.和Culver，Daniel A.和Barney，Joseph和Cordova，Francis C.和Carmona，Eva M。和Scholand，Mary Beth和Wijsenbeek，Marlies和Ganesh，Sivagini和Lower，Olyse E.和Engel，彼得J.和Wort，John和Price，Laura和Worts，Athol U.和Nathan，Steven D.和Baughman，RobertP.}，标题= {患有结节病相关的肺动脉高压患者的生存率的生理预测因素：来自国际注册表}的结果，体积= {55}，Number = {5}，Elocation-Id = {1901747}，年= {2020}，DOI = {10.1183 / 13993003.01747-2019}，出版商= {欧洲呼吸社会}188bet官网地址，摘要= {介绍结节病相关的肺动脉高压（SAPH）与单中心研究中的存活率降低有关。建立了长期随访的Saph（退休表）的国际登记处，以丰富我们对这种结节病的并并发症的了解。该分析旨在阐明与Saph患者的无流体存活相关的因素。由于登记登记时间以来，含水患者含有前瞻性收集的Saph患者的结果。分析的信息包括右心导管数据，肺功能测试，胸部射线照相，诈骗阶段和6分钟步行距离（6MWD）。COX回归模型用于识别无自由移植存活的独立预测因子。来自215名患者的数据数据，用于平均{\ textpm} SD 2.5 {\ textpm} 1.9岁以进行分析。在159例前列患者中，Kaplan {\ Textendash} Meier调整后的1-，3-和5年的无移植存活分别为89.2 \％，71.7 \％和62.0 \％。事件组中的Kaplan {\ TextEndash} Meier调整的1-，3-和5年的无移植生存率分别为83.5 \％，70.3 \％和58.3％，普遍存在的组是94.7 \％， 72.2\% and 66.3\%, respectively. Patients with reduced diffusing capacity of the lung for carbon monoxide (DLCO) (\<35\% predicted) and 6MWD \<300 m in the precapillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved forced expiratory volume (FEV1)/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort.Conclusion Reduced DLCO (\<35\% pred) and 6MWD (\<300 m) at the time of registry enrolment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV1/FVC ratio was identified as an independent risk factor for worsened outcomes.Decreased 6-min walk distance and reduced diffusion capacity are associated with decreased survival in patients with sarcoidosis-associated pulmonary hypertension http://bit.ly/2UQfWJh}, issn = {0903-1936}, URL = {//www.qdcxjkg.com/content/55/5/1901747}, eprint = {//www.qdcxjkg.com/content/55/5/1901747.full.pdf}, journal = {European Respiratory Journal} }