[Physiopathology of cystic fibrosis and of infections associated to this disease]

Abstract

Cystic fibrosis (CF) is one of the most common lethal inherited diseases in the caucasian population. The hope of understanding the physiopathology of CF appeared after identification of the gene which encodes CF. The alteration in CFTR protein breaks the hydric balance of the mucosal secretions, producing mucus with increased viscosity. Broncho-pulmonary infections are constant encountered because of the stasis of the mucus. The most common bacterial pathogens in the sputum of patients with MV are Haemophilus influenzae, Staphylococcus aureus, Pseudomonas aeruginosa, Burkholderia cepacia. MV remains a severe, incurable disease, but the evolution is getting better because of the improvements of care methods.