The University of Pennsylvania School of Medicine, Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Paediatr Respir Rev. 2010 Mar;11(1):3-8. doi: 10.1016/j.prrv.2009.10.005. Epub 2009 Nov 24.
Subjects with progressive neuromuscular diseases undergo a typical sequence of respiratory compromise, leading from normal unassisted gas exchange to nocturnal hypoventilation with normal daytime gas exchange, and eventually to respiratory failure requiring continuous ventilatory support. Several different abnormalities in respiratory pump function have been described to explain the development of respiratory failure in subjects with neuromuscular weakness. Early in the progression of respiratory failure, the use of nocturnal assisted ventilation can reverse both night- and day-time hypercapnia. Eventually, however, diurnal hypercapnia will persist despite correction of nocturnal hypoventilation. The likely beneficial effects of mechanical ventilatory support include resting fatigue-prone respiratory muscles and resetting of the central chemoreceptors to PaCO(2). Recent experience shows that select patients who require daytime ventilation can be supported with non-invasive ventilation continuously to correct gas exchange abnormalities while avoiding detrimental aspects of tracheostomy placement.
患有进行性神经肌肉疾病的患者会经历典型的呼吸功能障碍过程，从正常的自主气体交换发展为夜间低通气但日间气体交换正常，最终发展为需要持续通气支持的呼吸衰竭。已经描述了几种不同的呼吸泵功能异常，以解释神经肌肉无力患者呼吸衰竭的发生机制。在呼吸衰竭进展的早期，夜间辅助通气的使用可以逆转夜间和日间的高碳酸血症。然而，尽管夜间低通气得到纠正，但白天仍会持续存在高碳酸血症。机械通气支持的可能有益效果包括使易疲劳的呼吸肌得到休息，并重置中枢化学感受器对 PaCO2 的反应。最近的经验表明，一些白天需要通气的特定患者可以通过持续的无创通气来支持，以纠正气体交换异常，同时避免气管造口术的不利方面。
