Recent advances in the treatment of cystic fibrosis.
Cystic fibrosis is a serious, common genetic condition that causes recurrent pulmonary infections, malabsorption, and increased sweat electrolytes. Despite significant improvements in clinical treatment, individuals continue to die from progressive, obstructive pulmonary disease as children and young adults. This article reviews the current status of our understanding of cystic fibrosis: the basic defect, animal models, current therapy, and new approaches to the pulmonary disease.
Wilmott
R W
RW
Department of Pediatrics, University of Cincinnati College of Medicine, Ohio.
Fiedler
M A
MA
D000584:Amiloride / Q000627:therapeutic use; D000818:Animals; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002897:Chromosomes, Human, Pair 7; D002986:Clinical Trials as Topic; D003550:Cystic Fibrosis / Q000188:drug therapy / Q000235:genetics / Q000628:therapy*; D003850:Deoxyribonuclease I / Q000627:therapeutic use; D015316:Genetic Therapy; D006801:Humans; D016040:Lung Transplantation; D011994:Recombinant Proteins / Q000627:therapeutic use; D012138:Respiratory Therapy; D000515:alpha 1-Antitrypsin / Q000627:therapeutic use
D000900:Anti-Bacterial Agents; D011994:Recombinant Proteins; D000515:alpha 1-Antitrypsin; D000584:Amiloride; C085693:DNASE1 protein, human; D003850:Deoxyribonuclease I