Aerosol alpha 1-antitrypsin treatment for cystic fibrosis

Lancet. 1991 Feb 16;337(8738):392-4. doi: 10.1016/0140-6736(91)91167-s.


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 types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aerosols
  • Bronchoalveolar Lavage Fluid / chemistry
  • Cystic Fibrosis / drug therapy*
  • Evaluation Studies as Topic
  • Female
  • Humans
  • Leukocyte Elastase
  • Male
  • Pancreatic Elastase / antagonists & inhibitors
  • Pseudomonas aeruginosa / enzymology
  • alpha 1-Antitrypsin / administration & dosage*
  • alpha 1-Antitrypsin / therapeutic use


  • Aerosols
  • alpha 1-Antitrypsin
  • Pancreatic Elastase
  • Leukocyte Elastase