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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, Paris, France
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.