Ty -Jour T1-波森坦在儿童JF中治疗肺动脉高压治疗的长期疗效 - 欧洲呼吸杂志JO -EUR RESSIR J SP -70 LP -77 LP -77 DO -10.1183/09031936.00053510 VL -38A.A.Au -Moledina，S。Au -Foster，H。Au -Schulze -Neick，I.Au -Haworth，S.G。Y1- 2011/07/01 UR -http：//www.qdcxjkg.com/content.com/content/38/1/70.Abstract N2-本研究的目的是评估肺动脉高压儿童（PAH）儿童中波森坦的5年经验。从2002年2月至2008年5月，对英国儿童（英国伦敦大奥蒙德街儿童医院，英国伦敦大奥蒙德街医院，英国伦敦）的儿童（大奥蒙德街儿童医院，英国伦敦）的儿童进行了回顾性观察研究6个月。对101名特发性PAH（IPAH）（n = 42）和与先天性心脏病相关的PAH（n = 59）的儿童进行了详细研究。在治疗之前，确定了世界卫生组织（WHO）功能类别，步行6分钟（6MWD），身高，体重和血液动力学数据。在6个月后分析评估，每年最多5年。中位治疗时间为31.5个月。WHO功能类别和6MWD的最初改进可保持长达3年。 Height and weight increased but the z-scores did not improve. After 3 yrs, bosentan was continued as monotherapy in only 21% of children with IPAH, but in 69% of repaired cases and 56% of those with Eisenmenger syndrome. The Kaplan–Meier survival estimates for the 101 patients were 96, 89, 83 and 60% at 1, 2, 3 and 5 yrs, respectively. A treatment regime that includes bosentan is safe and appears to be effective in slowing disease progression in children with PAH. ER -