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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。
Objective To investigate the effect of noninvasive positive pressure ventilation (NIPPV) on the survival time of patients with amyotrophic lateral sclerosis (ALS). Methods The data of ALS database of neurology department in our hospital from 2003 to 2012 were retrospectively analyzed. According to the international E1 Escorial diagnostic criteria, all the patients were enrolled and followed up. CRF was used to record the clinical data of patients. FVC, Patients who started NIPPV at ≥50% or who did not have clinical signs of dyspnea were enrolled in the late-use group when they were enrolled in the early-use group, those with FVC <50% or those who started clinical symptoms of dyspnea using NIPPV. Telephone follow-up of patients every 3 months and record changes. Results Among 1697 patients with ALS, 208 patients were treated with NIPPV. Among them 147 patients had complete FVC or dyspnea symptom information, 47 patients in the early stage and 100 patients in the later stage were diagnosed as type 2 Site, confirmed the delay time, the beginning of the use of NIPPV treatment of ball symptoms, body mass index (BMI) there are significant differences; the two groups by 1: 1 nested pairs, and the relevant data by single factor and multivariate analysis The results showed no significant effect on survival time with FVC 50% and respiratory symptoms as indicators of treatment with NIPPV. Conclusions The use of 50% FVC and respiratory symptoms as indicators of NIPPV therapy did not significantly prolong the survival of patients with ALS.