Current developments in the treatment of pulmonary disease in patients with cystic fibrosis.
Cystic fibrosis is a genetic disease that affects one in 2500 live births. A basic defect in chloride transport leads to impaired clearance of airway secretions and a susceptibility to bacterial infection. Once infection is established there is a vicious cycle that leads to progressive inflammation and infection. Although cystic fibrosis is a multisystem disorder, pulmonary disease is the main cause of morbidity and respiratory failure remains the main cause of death. This review discusses the strategies for treating pulmonary disease in patients with cystic fibrosis and focuses on some of the therapeutic developments.
Shah
P L
PL
Department of Cystic Fibrosis, Royal Brompton Hospital and Imperial College at the National Heart and Lung Institute, Sydney Street, London, SW3 6NP, UK.