Pediatric Pulmonary Department, Hopital Armand Trousseau, Research unit INSERM UMR S-938/National Reference Centre for Rare Lung Diseases, Pierre et Marie Curie-Paris 6 University, 28 Avenue du Docteur Arnold Netter, Paris, France.
Minerva Anestesiol. 2011 Nov;77(11):1108-14. Epub 2011 May 20.
Cystic fibrosis (CF) lung disease is characterized by progressive airflow obstruction, due to mucus plugging and inflammation within the bronchial walls, and destruction of the lung parenchyma secondary to bronchiectasis. These alterations result in an increase of the work of breathing, leading to alveolar hypoventilation predominantly during sleep, exercise and acute respiratory exacerbations. Noninvasive positive pressure ventilation (NPPV) has been shown to unload the respiratory muscles in patients with CF, which increases alveolar ventilation and improves gas exchange. NPPV has been shown to reduce oxygen desaturation during sleep, exercise and chest physiotherapy and may facilitate the recovery of a severe hypercapnic respiratory exacerbation. Several ventilatory modes may be used in cystic fibrosis patients but the most physiological mode is pressure support. However, validated criteria to start NPPV as well as data on long term outcome, notably in terms of improved survival and quality of life, are lacking and should be assessed in the future.
囊性纤维化（CF）肺部疾病的特征是气流阻塞进行性加重，这是由于支气管壁内的黏液堵塞和炎症，以及支气管扩张引起的肺实质破坏所致。这些改变导致呼吸功增加，主要在睡眠、运动和急性呼吸恶化期间导致肺泡通气不足。无创正压通气（NPPV）已被证明可减轻 CF 患者的呼吸肌负担，增加肺泡通气并改善气体交换。NPPV 已被证明可减少睡眠、运动和胸部理疗期间的血氧饱和度下降，并可能有助于严重高碳酸血症呼吸恶化的恢复。几种通气模式可用于囊性纤维化患者，但最生理的模式是压力支持。然而，目前缺乏启动 NPPV 的验证标准以及长期结果数据，特别是在提高生存率和生活质量方面的数据，未来应进行评估。
