Ongoing planned clinical trials investigating the pulmonary management of cystic fibrosis.
Cystic fibrosis (CF) is a genetic disease where abnormalities in ion transport lead to poor clearance of viscoelastic secretions and a susceptibility to bacterial colonisation. Once established the infection/inflammatory cascade appears to be self-perpetuating. Treatment is therefore based on a number of strategies. Recently, advances have been concentrated at correcting the gene defect. Other strategies include correction of the ion transport defect. These may be crucial in patients if started before there is significant pulmonary disease. Developments aimed at improving the rheological properties of secretions, controlling airway infection and inflammation are essential once bronchopulmonary sepsis is established. This report looks at some of the clinical trials that are ongoing or planned in the treatment of pulmonary disease in CF.
Shah
P L
PL
Department of Cystic Fibrosis, Royal Brompton Hospital and National Heart & Lung Institute, Imperial College, Sydney Street, London SW3 6NP, UK.