Pinto Susana, Carvalho Mamede de
Translational Clinical Physiology Unit, Institute of Molecular Medicine, Faculty of Medicine, University of Lisbon, 1648-028 Lisbon, Portugal.
Neurodegener Dis Manag. 2014;4(1):83-102. doi: 10.2217/nmt.13.74.
In the last three decades, improvements in respiratory management are responsible for increasing survival and improving quality of life for amyotrophic lateral sclerosis (ALS) patients. Nowadays, ALS patients with respiratory involvement are offered a support treatment other than the traditional respiratory palliative care. Knowledge about available respiratory support potentialities is essential for appropriate, customized and effective treatment of ALS, which should probably be started sooner than the conventional approach. There is evidence supporting that respiratory support has a larger impact than riluzole on survival. Noninvasive ventilation is essential in the treatment of ALS patients with respiratory involvement. In this article methods to determine respiratory failure in ALS, mechanical invasive and noninvasive ventilation, telemetry, diaphragm pacing, cough aids and respiratory exercise are reviewed, after a brief overlook of respiratory insufficiency in ALS.
在过去三十年中，呼吸管理的改善提高了肌萎缩侧索硬化症（ALS）患者的生存率并改善了其生活质量。如今，有呼吸功能受累的ALS患者除了接受传统的呼吸姑息治疗外，还可获得支持性治疗。了解现有的呼吸支持潜力对于对ALS患者进行恰当、个性化且有效的治疗至关重要，这种治疗可能应比传统方法更早开始。有证据支持呼吸支持对生存率的影响比利鲁唑更大。无创通气对于治疗有呼吸功能受累的ALS患者至关重要。在简要概述ALS患者的呼吸功能不全后，本文对确定ALS患者呼吸衰竭的方法、有创和无创机械通气、遥测、膈肌起搏、咳嗽辅助和呼吸锻炼进行了综述。
