Clinical information and laboratory data of the CF patients enrolled in the study.

*Cystic Fibrosis mutation database reference: http://www3.genet.sickkids.on.ca/cftr/app.**As determined by Sweat test performed according to the Gibson and Cooke method (Sweat testing: Sample Collection and Quantitative Analysis – Approved Guideline – Second Edition – The National Committee for Clinical Laboratory Standards (NCCLS) – document C34 A2 [ISBN – 56238 – 407 – 4]; USA . Pa: Pseudomonas Aeruginosa chronic infection according to Doring et al [44]; PI (pancreatic insufficiency requiring oral enzyme supplementation); NPD: Nasal Potential Difference; nd: not done; CF: cystic fibrosis.***Numbers indicate NPD values calculated as described by Wilschanski et al. [27].