Aerosol α1 -antitrypsin treatment for cystic fibrosis Academic Article Article uri icon


MeSH Major

  • Gene Expression
  • Lung Diseases
  • Mutation
  • Respiration
  • Smoking


  • 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.

publication date

  • February 16, 1991



  • Academic Article


Digital Object Identifier (DOI)

  • 10.1016/0140-6736(91)91167-S

PubMed ID

  • 1671425

Additional Document Info

start page

  • 392

end page

  • 4


  • 337


  • 8738