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首页> 外文期刊>European journal of neurology: the official journal of the European Federation of Neurological Societies >Impact of an early respiratory care programme with non‐invasive ventilation adaptation in patients with amyotrophic lateral sclerosis
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Impact of an early respiratory care programme with non‐invasive ventilation adaptation in patients with amyotrophic lateral sclerosis

机译：早期呼吸护理程序对肌营养侧面硬化症患者的非侵入性通气适应的影响

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Background and purpose Forced vital capacity ( FVC ) 80% is one of the key indications for starting non‐invasive ventilation ( NIV ) in amyotrophic lateral sclerosis ( ALS ). It was hypothesized that a very early start of NIV could lengthen the free interval before death compared to later‐start NIV ; as a secondary outcome, the survival rate of patients on NIV without tracheotomy was also evaluated. Methods This retrospective study was conducted on 194 ALS patients, divided into a later group ( LG ) with FVC 80% at NIV prescription ( n ?=?129) and a very early group ( VEG ) with FVC ?≥80% at NIV prescription ( n ?=?65). Clinical and respiratory functional data and time free to death between groups over a 3‐year follow‐up were compared. Result At 36?months from diagnosis, mortality was 35% for the VEG versus 52.7% for the LG ( P ?=?0.022). Kaplan?Meier survival curves adjusted for tracheotomy showed a lower probability of death ( P ?=?0.001) for the VEG as a whole ( P ?=?0.001) and for the non‐bulbar ( NB ) subgroup ( P ?=?0.007). Very early NIV was protective of survival for all patients [hazard ratio ( HR ) 0.45; 95% confidence interval ( CI ) 0.28–0.74; P ?=?0.001] and for the NB subgroup ( HR 0.43; 95% CI 0.23–0.79; P ?=?0.007), whilst a tracheotomy was protective for all patients ( HR 0.27; 95% CI 0.15–0.50; P ?=?0.000) and both NB ( HR 0.26; 95% CI 0.12–0.56; P ?=?0.001) and bulbar subgroups ( HR 0.29; 95% CI 0.11–0.77; P ?=?0.013). Survival in VEG patients on NIV without tracheotomy was three times that for the LG (43.1% vs. 14.7%). Conclusion Very early NIV prescription prolongs the free time from diagnosis to death in NB ALS patients whilst tracheotomy reduces the mortality risk in all patients.
机译：背景和目的强制生命能力（FVC）<80％是用于在肌营养的外侧硬化剂（ALS）中起始非侵入性通气（NIV）的关键适应症之一。假设尼维希尔的早期开始可以延长死亡前的自由间隔与后期开始效率相比;作为次要结果，还评估了没有气管切开术的NIV患者的存活率。方法对194年的患者进行了该回顾性研究，分为含有FVC的后期（LG），在NIV处方（N？= 129），并且具有FVC的一个非常早期的组（VEG）≥80％ niv处方（n？=？65）。比较了临床和呼吸功能数据和在3年后续随访之间的组之间自由死亡。结果在诊断中36？几个月，蔬菜的死亡率为35％，而LG对52.7％（p？= 0.022）。 Kaplan？用于气管切开术的Meier生存曲线显示蔬菜的死亡概率较低（p？= 0.001），作为整体（p？= 0.001）和非凸形（Nb）子组（p？= 0.007 ）。 NIV初期对所有患者的生存是保护的[危险比（HR）0.45; 95％置信区间（CI）0.28-0.74; p？= 0.001]和Nb亚组（HR 0.43; 95％CI 0.23-0.79; p？= 0.007），而气管切开术对所有患者进行了保护性（HR 0.27; 95％CI 0.15-0.50; p？ =？0.000）和Nb（HR 0.26; 95％CI 0.12-0.56; P？= 0.001）和凸形亚组（HR 0.29; 95％CI 0.11-0.77; P？= 0.013）。没有气管切开术的蔬菜患者的生存是LG的三倍（43.1％vs.14.7％）。结论，NB ALS患者中，NB ALS患者中的诊断到死亡的空闲时间延长了诊断的空闲时间，而气管切开术，则降低所有患者的死亡风险。

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