Mucoviscidose

In children affected by cystic fibrosis, the lung clearance index is an advance concerning the assessment of lung function; it appears not only as a tool for early screening of respiratory injury but also as a marker of lung hyperinflation. In adults, estrogen's implication in the rate of exacerbations and in the infection by mucoïd strains of Pseudomonas aeruginosa brings an explanation for the poor prognosis of cystic fibrosis in female patients. Regarding treatments, macrolides confirm their long-term efficacy in cystic fibrosis but questions persist about the mechanisms and the long-term effects. This year a new treatment appears : ivacaftor, first treatment targeting CFTR protein and showing an efficacy on lung function and weight gain in cystic fibrosis children and adults with G551D mutation.