title: Inhalation-based therapies in the treatment of cystic fibrosis.
abstract: As most of the morbidity seen in cystic fibrosis (CF) is related to pulmonary complications, new therapies are being developed that seek to ameliorate these debilitating sequelae. Recently, there has been intense effort into the development of new aerosol-based therapies for CF. This review summarizes much of this recent investigation, with particular emphasis on therapies described in the literature within the past year. Agents that act on the airway epithelium to alter ionic fluxes, namely amiloride and uridine 5'-triphosphate, are outlined. The effects of tobramycin delivered via nebulizer in a cohort of stable CF patients is reported. Recombinant human DNase and distearoyl phosphatidylglycerol liposomes, agents that alter the adhesiveness of CF mucus, are outlined as possible strategies for CF treatment. Finally, antiprotease therapy is considered as a possible addition to the CF armamentarium.
authors.affiliation: Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: C E
authors.initials: CE
authors.lastname: Harris
authors.forename: R W
authors.initials: RW
authors.lastname: Wilmott
mesh_terms: D000280:Administration, Inhalation; D000336:Aerosols; D000584:Amiloride / Q000627:therapeutic use; D003550:Cystic Fibrosis / Q000188:drug therapy*; D003851:Deoxyribonucleases / Q000627:therapeutic use; D004337:Drug Carriers; D006801:Humans; D008081:Liposomes; D010715:Phosphatidylglycerols / Q000627:therapeutic use; D011480:Protease Inhibitors / Q000627:therapeutic use; D012138:Respiratory Therapy / Q000379:methods*; D014031:Tobramycin / Q000627:therapeutic use; D014544:Uridine Triphosphate / Q000627:therapeutic use
chemical_list: D000336:Aerosols; D004337:Drug Carriers; D008081:Liposomes; D010715:Phosphatidylglycerols; D011480:Protease Inhibitors; C084352:distearoyl phosphatidylglycerol; D000584:Amiloride; D003851:Deoxyribonucleases; D014544:Uridine Triphosphate; D014031:Tobramycin