Respiratory Care Unit, Respiratory Medicine Department, Hospital Clínico Universitario, Valencia, Spain.
Am J Phys Med Rehabil. 2010 May;89(5):407-11. doi: 10.1097/PHM.0b013e3181d8a479.
To assess the efficacy of noninvasive ventilatory support and intermittent positive pressure ventilation via uncuffed tracheostomy tubes (uTIPPV) to prolong survival in amyotrophic lateral sclerosis.
Survival was prolonged by continuous noninvasive ventilatory support or TIPPV dependence. Once noninvasive ventilatory support was no longer adequate for six noninvasive ventilatory support users, they and 22 others underwent tracheotomy within 1 mo of measurement of spirometry (forced vital capacity and forced volume expired in 1 sec), peak cough flows, maximum insufflation capacity, manually assisted peak cough flows, and mechanically assisted peak cough flows. Glottic function was estimated by maximum insufflation capacity, FVC difference, and bulbar-innervated muscle function by Norris scale bulbar-innervated muscle subscore. Cuffless tubes were replaced by cuffed ones when hypoventilation developed despite increasing uTIPPV volumes.
The survival of 22 patients was prolonged by continuous noninvasive ventilatory support dependence for 7.8 +/- 8.1 mos (range, 1-36 mos; median, 5 mos) after 13.9 +/- 11.2 mos (range, 2-36 mos) of part-time noninvasive ventilatory support. Six of these and 22 others underwent tracheotomy and initially used uTIPPV effectively. For ten of the 28 (35.7%) patients, the tubes had to be replaced by cuffed ones after 5.7 +/- 7.8 mos with the other 18 still using tracheostomy intermittent positive pressure ventilation volumes via uncuffed tubes for 20.2 +/- 17.6 mos at data collection. Pretracheotomy FVC was significantly lower in the ten patients who eventually required cuffed tubes (0.70 +/- 0.44 vs. 1.06 +/- 0.43 L, P < 0.05). The bulbar-innervated muscle was significantly lower when the uTIPPV users required cuffed TIPPV than when beginning uTIPPV (Norris scale bulbar-innervated muscle subscore, 3.20 +/- 1.30 vs. 8.00 +/- 3.20, P < 0.05), respectively.
Noninvasive and tracheostomy IPPV via cuffless tubes can prolong survival for patients with amyotrophic lateral sclerosis until excessive air leak and hypoventilation necessitate a cuffed tube.
评估无创通气支持和经无囊气管切开管间歇性正压通气（uTIPPV）在延长肌萎缩侧索硬化患者生存时间方面的疗效。
通过持续的无创通气支持或 TIPPV 依赖来延长生存时间。一旦有 6 名无创通气支持者不再适合无创通气支持，并且在测量肺活量（用力肺活量和 1 秒用力呼气量）、峰值咳嗽流量、最大充气容量、手动辅助峰值咳嗽流量和机械辅助峰值咳嗽流量后 1 个月内，他们和其他 22 名患者接受了气管切开术。声门功能通过最大充气容量、FVC 差异和诺里斯量表球麻痹肌肉子评分来评估球麻痹肌肉功能。当尽管增加 uTIPPV 量仍出现通气不足时，用带囊气管切开管替换无囊管。
22 名患者通过持续的无创通气支持依赖性延长了生存时间，分别为 7.8±8.1 个月（范围 1-36 个月；中位数 5 个月）和 13.9±11.2 个月（范围 2-36 个月），然后进行部分时间的无创通气支持。其中 6 名患者和另外 22 名患者接受了气管切开术，并在最初有效地使用了 uTIPPV。在 28 名患者中的 10 名（35.7%）患者中，在使用 uTIPPV 5.7±7.8 个月后，需要更换带囊管，而其余 18 名患者在数据收集时仍使用无囊气管切开间歇正压通气 20.2±17.6 个月。在最终需要带囊管的 10 名患者中，术前 FVC 显著较低（0.70±0.44 比 1.06±0.43 L，P<0.05）。当 uTIPPV 使用者需要带囊 TIPPV 时，球麻痹肌肉明显低于开始 uTIPPV 时（诺里斯量表球麻痹肌肉子评分，3.20±1.30 比 8.00±3.20，P<0.05）。
无创和气管切开术 IPPV 通过无囊管可以延长肌萎缩侧索硬化患者的生存时间，直到出现过度漏气和通气不足需要带囊管。
