Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Paediatr Respir Rev. 2010 Mar;11(1):18-23. doi: 10.1016/j.prrv.2009.10.002. Epub 2009 Dec 2.
Paediatric neuromuscular disease compromises both the gas exchange and pump functions of the respiratory system. This can have profound implications for both growth and development of the respiratory system, as well as morbidity and mortality. Aspiration lung disease is common, and leads to increasingly restrictive pulmonary physiology over time. Abnormal lung and chest wall mechanics, and weak respiratory muscles, can combine to cause respiratory failure. Improving the balance between the work of breathing (by decreasing the respiratory load) and the respiratory pump (by improving respiratory muscle strength and decreasing respiratory muscle fatigue) can help prevent the onset of respiratory failure. Airway clearance techniques and non-invasive ventilation are two important tools in this effort. Better ways of assessing the respiratory pump, mechanical function, control and fatigue are needed especially in children.
儿科神经肌肉疾病会影响呼吸系统的气体交换和泵功能。这可能对呼吸系统的生长和发育以及发病率和死亡率产生深远影响。吸入性肺病很常见，随着时间的推移，会导致肺部生理功能逐渐受限。异常的肺和胸壁力学以及呼吸肌无力会共同导致呼吸衰竭。通过降低呼吸负荷和改善呼吸肌力量、减少呼吸肌疲劳来改善呼吸做功的平衡，可以帮助预防呼吸衰竭的发生。清除气道分泌物技术和无创通气是这方面的重要工具。特别是在儿童中，需要更好的方法来评估呼吸泵、机械功能、控制和疲劳。
