@article {Smith1150, author = {Smith, IE and King, MA and Siklos, PW and Shneerson, JM}， title = {Prader-Willi综合征通气衰竭的治疗}，volume = {11}， number = {5}， pages ={1150—1152}，year = {1998}， publisher = {European Respiratory Society}，188bet官网地址高碳酸性呼吸衰竭是Prader-Willi综合征中常见的死亡原因。它与睡眠呼吸障碍的关系尚未确定，也没有其成功治疗的报告。我们回顾了四名患者的记录与综合征，谁发展通气衰竭。比较治疗前和治疗期间日间动脉血气张力和夜间血氧测定。每个患者都有严重的睡眠呼吸障碍，并伴有日间呼吸衰竭。中位夜间平均动脉氧饱和度(Sa、O2)为82\%，中位最小值仅为41.5\%。初期治疗为鼻间歇性正压通气，每例患者日间动脉血气张力均恢复正常。患者出院后夜间维持鼻腔持续气道正压通气。依从性良好，最终随访(平均4.8年)日间动脉气张力保持正常，夜间平均动脉氧饱和度为95.5%，最小值为84.5%。 This study of patients with the Prader-Willi syndrome shows that daytime ventilatory failure is associated with sleep-disordered breathing. It can be reversed with nocturnal noninvasive ventilation and maintenance treatment with continuous positive airway pressure is well tolerated, with no deterioration in respiratory parameters.}, issn = {0903-1936}, URL = {//www.qdcxjkg.com/content/11/5/1150}, eprint = {//www.qdcxjkg.com/content/11/5/1150.full.pdf}, journal = {European Respiratory Journal} }