Les manifestations respiratoires de la mucoviscidose

Cystic fibrosis airways disease Cystic fibrosis is the most common lethal genetic disease affecting caucasians, due to a lesion of the CF gene encoding the CF transmembrane conductance regulator (CFTR). Lung disease is the primary cause of morbidity and mortality in CF. A permanent colonization of the airways with Haemophilus influenzae, Staphylococcus aureus and later Pseudomonas aeruginosa leads to progressive cycles of inflammation and infection cumulating in respiratory failure. Patients with CF are also predisposed to numerous bronchopulmorary complications as fungal infections and allergic bronchopulmorary aspergillosis, pneumothorax and hemoptysis. A standardized survey in a CF center and an early treatment of symptoms and complications delay respiratory deterioration.