Abstract
Cystic fibrosis (CF) is an inherited disease caused by mutations in the gene for transmembrane conductance regulator (CFTR) with pulmonary manifestations and mimoplicními. Pharmacotherapy of pulmonary disease in CF is primarily targeted at mukostázu, bacterial infection and neutrophilic inflammation.
Treatment of extrapulmonary manifestations of CF is particularly pancreatic insufficiency, deficiency of fat-soluble vitamins, intestinal obstruction, diabetes, osteoporosis and hepatopathy. In the research phase is presently CF gene therapy and targeted therapy for individual CFTR gene mutations.