中文摘要：

目的探讨用力肺活量（forced vital capacity，FVC）〈75％的肌萎缩侧索硬化（amyotrophic lateral sclerosis，ALS）患者早期接受无创正压通气（noninvasive positivepressure ventilation，NPPV）治疗1年后的疗效评估。方法测定60例肌萎缩侧索硬化症（amyothophic lateral sclerosis，ALs）患者肺通气功能，以用力肺活量（FVC％）是否低于75％和是否接受NPPV治疗为标准分3组，其中FVC％〈75％且配合NPPV治疗14例，作为第1组；FVC％〈75％且不能配合NPPV治疗18例，作为第2组；FVC％〉75％为第3组，即阳性对照组，共28例。结果第1组（FVC％〈75％且配合NPPV治疗）ALS患者1年内生存率与第2组（FVC％〈75％且不能配合NPPV治疗）ALS患者比较有统计学差异（P〈0．05），与第3组（阳性对照组）无统计学差异（P〉0．05）。第1组患者FVC％下降的幅度以及ALSFRS-R评分降低的幅度较第2组患者缓慢，差异具有统计学意义（P〈0．05）。结论在ALS患者FVC〈75％时引入NPPV治疗，可延缓疾病的进展，延长患者的生存时间。

英文摘要：

Objective To investigate 1 year survival of ALS patients with FVC 〈 75% treated with NPPV. Methods Sixty consecutive ALS patients were included in the present study. All ALS patients underwent pulmonary function and were further divided into three groups based on pulmonary function test. Thirty-three patients bad a FVC 〈 75% and showed nocturnal respiratory insufficiency. Among them, fourteen patients who accepted and could tolerate NPPV and twelve patients who refused or could not intolerate NPPY were categorized as the group one and group two, respectively. The rest twenty-eight ALS patients who had a FVC 〉 75%, and did not show nocturnal respiratory insufficiency were served as control group. Results The survival rate significantly increased in patients with FVC 〈 75% receiving NPPV compared with those who refused or could not tolerate NPPV （ P 〈 O. 05 ） at one year following NPPY. There was no significant differ- ence in the survival rate between patients with FVC 〈 75% receiving NPPV and controls （ P 〉 0.05 ）. The decline in FVC% was slower in patients with NPPY than in those without NPPV. Conclusions Early treatment with NPPV prolongs survival and reduces decline of FVC% in patients with ALS.