CFTR and cystic fibrosis: A genetically defective cystic fibrosis transmembrane conductance regulator (CFTR) leads to mucociliary dysfunction, increased bacterial colonization and inflammation1 that may be even endogenous, based on observations of inflammation preceding infection in CF neonates and young children2–5, where bronchoalveolar lavage fluid showed increased levels of IL-8 even in the absence of bacterial infection3,6.