title: Aerosol alpha 1-antitrypsin treatment for cystic fibrosis.
abstract: In cystic fibrosis neutrophil-dominated inflammation on the respiratory epithelial surface results in a chronic epithelial burden of the destructive enzyme, neutrophil elastase. alpha 1-antitrypsin (alpha 1AT), the main inhibitor of neutrophil elastase in lung, was given in aerosol form to 12 cystic fibrosis patients. It suppressed neutrophil elastase in the respiratory epithelial lining fluid (ELF) and restored the ELF anti-neutrophil elastase capacity when ELF alpha 1AT reached 8 mumol/l. This treatment also reversed the inhibitory effect of cystic fibrosis ELF on pseudomonas killing by neutrophils, which suggests that it may augment host defence in cystic fibrosis.
authors.affiliation: Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892.
authors.forename: N G
authors.initials: NG
authors.lastname: McElvaney
authors.forename: R C
authors.initials: RC
authors.lastname: Hubbard
authors.forename: P
authors.initials: P
authors.lastname: Birrer
authors.forename: M S
authors.initials: MS
authors.lastname: Chernick
authors.forename: D B
authors.initials: DB
authors.lastname: Caplan
authors.forename: M M
authors.initials: MM
authors.lastname: Frank
authors.forename: R G
authors.initials: RG
authors.lastname: Crystal
mesh_terms: D000328:Adult; D000336:Aerosols; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry; D003550:Cystic Fibrosis / Q000188:drug therapy*; D005069:Evaluation Studies as Topic; D005260:Female; D006801:Humans; D019272:Leukocyte Elastase; D008297:Male; D010196:Pancreatic Elastase / Q000037:antagonists & inhibitors; D011550:Pseudomonas aeruginosa / Q000201:enzymology; D000515:alpha 1-Antitrypsin / Q000008:administration & dosage* / Q000627:therapeutic use
chemical_list: D000336:Aerosols; D000515:alpha 1-Antitrypsin; D010196:Pancreatic Elastase; D019272:Leukocyte Elastase