Elsevier

The Lancet

Volume 337, Issue 8738, 16 February 1991, Pages 392-394
The Lancet

SHORT REPORTS
Aerosol α1 -antitrypsin treatment for cystic fibrosis

https://doi.org/10.1016/0140-6736(91)91167-SGet rights and content

Abstract

In cystic fibrosis neutrophil-dominated inflammation on the respiratory epithelial surface results in a chronic epithelial burden of the destructive enzyme, neutrophil elastase. α1-antitrypsin (α1AT), 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 α1AT reached 8 μmol/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.

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