Cystic fibrosis. Clinical update for radiologists

The clinical features and current management of cystic fibrosis are reviewed, with emphasis on pulmonary manifestations. Pulmonary radiographic findings in cystic fibrosis are illustrated. The role of transplantation as a therapeutic avenue is discussed. Recent discoveries--the specific genetic mutation in cystic fibrosis, the elucidation of the cellular defect, and the development of gene-transfer techniques to correct the cellular defect in vitro--have generated fresh excitement in the search for an ultimate cure for cystic fibrosis.