Aerosol alpha 1-antitrypsin treatment for cystic fibrosis.


Journal Article

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.

Full Text

Cited Authors

  • McElvaney, NG; Hubbard, RC; Birrer, P; Chernick, MS; Caplan, DB; Frank, MM; Crystal, RG

Published Date

  • February 16, 1991

Published In

Volume / Issue

  • 337 / 8738

Start / End Page

  • 392 - 394

PubMed ID

  • 1671425

Pubmed Central ID

  • 1671425

International Standard Serial Number (ISSN)

  • 0140-6736

Digital Object Identifier (DOI)

  • 10.1016/0140-6736(91)91167-s


  • eng

Conference Location

  • England