Noninvasive Ventilation for Cystic Fibrosis

Cystic fibrosis (CF) is a genetic disease caused by mutations in the cystic fibrosis transmembrane regulator (CFTR) gene responsible for defective functioning in the protein CFTR, that is a transmembrane apical channel deputed to hydroelectrolyte exchanges between the two sites of the cell membrane. CFTR protein is expressed in the airways, intestine, pancreas, liver, bone, genital system and sweat gland. The defective function of CFTR protein determines dehydration of the extracellular fluids and viscous secretion.