Farmacotherapie bij cystiche fibrosse

Drug therapy in cystic fibrosis is focused on pulmonary infection and inflammation, intestinal obstruction and the substitution of pancreas enzymes. Most morbidity and mortality arises from the pulmonary infection and subsequent tissue destruction and deterioration of lung function. Antibiotic treatment is the mainstay of therapy. Nebulised antibiotics improve lung function and the incidence of acute exacerbations is reduced in patients chronically infected with Pseudomonas. Macrolides play a special role probably due to an antiinflammatory effect. Inhalation of rhDNAse improves lung function and decreases deterioration of lung function. New therapeutics directed at the CFTR defect are not yet available. Pharmacokinetics in CF patients may differ from those in healthy volunteers, probably due to differences in body composition.