Lechtzin Noah, Scott Yanille, Busse Anne M, Clawson Lora L, Kimball Richard, Wiener Charles M
Johns Hopkins University School of Medicine, MD 21205, USA.
Amyotroph Lateral Scler. 2007 Jun;8(3):185-8. doi: 10.1080/17482960701262392.
Non-invasive positive pressure ventilation (NPPV) can improve survival in ALS patients with advanced respiratory impairment, but it is not known if it is beneficial earlier in the disease course. A retrospective cohort study of patients with ALS was performed comparing survival from time of diagnosis in subjects who started NPPV use when their FVC was >or=65% predicted (Early NPPV) with subjects who started NPPV when their FVC was below 65% predicted (Standard NPPV). The Early group (n = 25) and the Standard group (n = 67) were similar except for pulmonary function (mean FVC in Early NPPV group = 74.3+/-10.1% predicted and 48.3+/-11.3 in Standard group, p<0.001). The median time from ALS diagnosis to death was significantly longer in the Early NPPV group (2.7 years vs. 1.8 years, p = 0.045). This remained significant after adjustment for potential confounding factors (H.R. = 0.55, 95% CI 0.31-0.98). Survival from time of diagnosis was nearly one year longer in the Early group. Until more definitive data are available from randomized trials, our findings suggest that clinicians either encourage earlier use of NPPV or use more sensitive tests for respiratory muscle impairment than upright FVC.
无创正压通气（NPPV）可提高晚期呼吸功能不全的肌萎缩侧索硬化症（ALS）患者的生存率，但在疾病进程的早期使用是否有益尚不清楚。我们进行了一项针对ALS患者的回顾性队列研究，比较了用力肺活量（FVC）≥预测值65%时开始使用NPPV的患者（早期NPPV组）与FVC低于预测值65%时开始使用NPPV的患者（标准NPPV组）从诊断时起的生存率。早期组（n = 25）和标准组（n = 67）除肺功能外相似（早期NPPV组平均FVC = 预测值的74.3±10.1%，标准组为48.3±11.3，p<0.001）。早期NPPV组从ALS诊断到死亡的中位时间显著更长（2.7年对1.8年，p = 0.045）。在对潜在混杂因素进行调整后，这一差异仍具有统计学意义（风险比 = 0.55，95%置信区间0.31 - 0.98）。早期组从诊断时起的生存期几乎长一年。在从随机试验获得更确切的数据之前，我们的研究结果表明，临床医生要么鼓励更早使用NPPV，要么使用比直立位FVC更敏感的呼吸肌功能损害检测方法。
