Recent advances in the understanding and treatment of cystic fibrosis.
Great progress has been made both in understanding the pathophysiology of cystic fibrosis and in providing comprehensive medical care for both children and adults with this illness. Cystic fibrosis is the most common genetic disease affecting white people in the United States. Whereas 30 years ago a minority of patients reached their teens, now the median survival is about 30 years and is steadily increasing. Considerable work remains to be done in order to better understand how the defect in the cystic fibrosis transmembrane conductance regulator interacts with other ion channels in the lung to create an environment of chronic infection and inflammation. There is promise in the fact that various treatment modalities are in different stages of investigation and that the improvement of the outcomes for patients with cystic fibrosis, and ultimately a cure for this disease, may be forthcoming.
Department of Medicine, University of California, San Diego 92093, USA.
J R
JR
Resnikoff
D J
DJ
Conrad
D000328:Adult; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003550:Cystic Fibrosis / Q000209:etiology / Q000628:therapy*; D004359:Drug Therapy, Combination; D015316:Genetic Therapy; D006801:Humans; D016040:Lung Transplantation; D011379:Prognosis