Effect to prognosis by early or late use of noninvasive positive pressure ventilation in patients with amy- otrophic lateral sclerosis

目的：研究肌萎缩侧索硬化（amyotrophic lateral sclerosis，ALS）患者使用无创正压通气（noninvasive positive pressure ventilation，NIPPV）治疗对其生存时间的影响。方法回顾性分析2003～2012年本院神经内科ALS数据库资料，根据国际E1 Escorial诊断标准入选并随访全部患者，采用统一设计的病例报告表（CRF）记录患者相关临床数据，将用力肺活量（FVC）≥50%或临床尚无呼吸困难症状时开始使用NIPPV治疗的患者纳入早期使用组、FVC＜50%或临床已有呼吸困难症状时开始使用NIPPV治疗的患者纳入晚期使用组。每3个月对患者进行电话随访并记录变化信息。结果1697例ALS患者中，208例使用了NIPPV治疗，其中，有完整FVC或呼吸困难症状资料者147例，早期使用组47例，晚期使用组100例，两组患者在诊断分型、起病部位、确诊延迟时间、开始使用NIPPV治疗时的球部症状、体质指数（BMI）等方面存在显著差异；将两组按1︰1进行巢式配对，并对相关数据进行单因素及多因素分析，结果显示，以FVC 50%和呼吸症状作为使用NIPPV治疗的指标对生存时间无显著影响。结论依据FVC 50%和呼吸症状作为使用NIPPV治疗的指标并未明显延长ALS患者的生存时间。ObjectiveTo observe the effect to survival time of amyotrophic lateral sclerosis (ALS) patients by early or late onset use of noninvasive positive pressure ventilation (NIPPV).MethodRetrospectively analyzed the patients in the database of ALS patients admitted from outpatient in neurology department of Peking University Third Hospital from 2003 to 2012. Chose the patients who were diagnosed as ALS according to the Escorial Criteria and whose diagnosing grade was definite, probable or possible, another criteria was that their diagnosis as ALS didn’t change during our follow-up, used the same designed recording book to record their clinic materials, and followed up them by phone every 3 months and also recorded the related clinic materials.ResultIn the database of 1697 ALS patients in our department from 2003 to 2012, 208 patients used NIPPV. We deifned the patients who began to use NIPPV when their FVC≥50% or they didn’t have respiratory symptom as early-onset users, and those who began to use NIPPV when their FVC<50% or they had respiratory symptom as late-onset users. Then we analyzed the 147 ALS patients (47 early-onset users, 100 late-onset users) who had complete data and use NIPPV, and we found there was signiifcant difference between early-onset and late-onset users in these factors, namely, subtype of diagnosis, subtype of diagnosis, onset site, delay time of diagnosis, exist bulbar symptom and BMI when patients began to use NIPPV. So we matched the 47 early-onset users to 47 late-onset users and divided them into groups with or without bulbar symptom when they began to use NIPPV in order to make sure that every factor was compatible. We analyzed the data above using the single-factor analysis and multiple-factor analysis and foundthat survival time was compatible between early-onset and late-onset users.ConclusionUsing FVC and respiratory symptom as criteria to deifne early-onset and late-onset users, our retrospective nonsynchronous control research suggested that early-onset users of NIPPV didn’t extend their survival time than late-onset users.国家自然科学基金重点项目中国科技核心期刊(ISTIC)911-1