ABSTRACT
Cystic brosis (CF) lung disease is characterized by progressive airow obstruction, due to infection, mucus plugging and inammation within the bronchial walls, and destruction of the lung parenchyma secondary to bronchiectasis, leading to a progressive respiratory failure. is characteristic involvement of the airways and the lung parenchyma explains the abnormalities of the respiratory physiology and mechanics in CF. Although CF lung disease may be considered as a chronic obstructive pulmonary disease (COPD), the alterations observed in respiratory mechanics dier from those of the “classical” non-CF COPD. Indeed, CF lung disease is associated with both an obstructive and a restrictive ventilatory defect, with an early decline in lung compliance, which constitutes the major determinant of the increase in respiratory muscle work.1
