RT期刊文章SR电子T1睡眠和肺气道丛状纤维瘤的临床试验结果NF1摩根富林明神经病学神经学乔FD Lippincott Williams &威尔金斯SP向OP S20 10.1212 / WNL。首页0000000000002933签证官87是7补充1 A1斯科特·r·普罗金A1斯蒂芬妮·d·戴维斯A1肯特·a·罗伯逊A1 Srivandana Akshintala A1朱利安·艾伦A1迈克尔·j·费舍尔A1 Jaishri o·布莱克利正在A1林c Widemann A1罗莎莉e .蕨A1卡罗尔·l·马库斯A1的缰绳国际合作年2016 UL //www.ez-admanager.com/content/87/7_Supplement_1/S13.abstract AB目的:丛状纤维瘤(pn)是复杂的,良性神经鞘瘤发生在大约25% - -50%的患者神经纤维瘤病1型(NF1)。首页pn,引起气道狭窄或肺功能障碍是少见但临床上重要的。因为睡眠质量的改善或气道功能代表了直接的临床效益,睡眠和肺功能可能是更有意义的比肿瘤大小在治疗临床试验针对气道PN端点。方法:响应评价神经纤维瘤和Schwannomatosis功能结果组审查现有端点对睡眠和肺反应评价结果和发展共识建议NF临床试验。结果:患者气道pn,多导睡眠图,应该执行脉冲高频指示,肺量测定法来识别异常的函数,将代理在临床研究的目标。的官能团支持使用睡眠呼吸暂停指数(AHI)作为主要的端点,和肺阻力10赫兹(R10)或在1或0.75秒用力呼气量(FEV1或FEV0.75)作为主要肺端点。定义的组AHI最小变化,R10和残或FEV0.75响应标准。二次睡眠结果包括稀释和血碳酸过多症在睡眠和觉醒指数。继发性肺结果包括肺电阻和电抗测量在5、10、20赫兹;用力肺活量; peak expiratory flow; and forced expiratory flows.Conclusions: These recommended sleep and pulmonary evaluations are intended to provide researchers with a standardized set of clinically meaningful endpoints for response evaluation in trials of NF1-related airway PNs.AASM=American Academy of Sleep Medicine; AHI=apnea hypopnea index; CPAP=continuous positive airway pressure; FEV0.75=forced expiratory volume in 0.75 seconds; FEV1=forced expiratory volume in 1 second; FVC=forced vital capacity; NF1=neurofibromatosis type 1; OSA=obstructive sleep apnea; PEF=peak expiratory flow; PN=plexiform neurofibroma; PSG=polysomnography; REiNS=Response Evaluation in Neurofibromatosis and Schwannomatosis