Aerosol α1 -antitrypsin treatment for cystic fibrosis

N. G. McElvaney, R. C. Hubbard, P. Birrer, Ronald Crystal, M. S. Chernick, M. M. Frank, D. B. Caplan

Research output: Contribution to journalArticle

279 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)392-394
Number of pages3
JournalThe Lancet
Volume337
Issue number8738
DOIs
Publication statusPublished - 16 Feb 1991
Externally publishedYes

Fingerprint

Aerosols
Cystic Fibrosis
Leukocyte Elastase
Neutrophils
Secretory Proteinase Inhibitory Proteins
Therapeutics
Pseudomonas
Inflammation
Lung
Enzymes

ASJC Scopus subject areas

  • Medicine(all)

Cite this

McElvaney, N. G., Hubbard, R. C., Birrer, P., Crystal, R., Chernick, M. S., Frank, M. M., & Caplan, D. B. (1991). Aerosol α1 -antitrypsin treatment for cystic fibrosis. The Lancet, 337(8738), 392-394. https://doi.org/10.1016/0140-6736(91)91167-S

Aerosol α1 -antitrypsin treatment for cystic fibrosis. / McElvaney, N. G.; Hubbard, R. C.; Birrer, P.; Crystal, Ronald; Chernick, M. S.; Frank, M. M.; Caplan, D. B.

In: The Lancet, Vol. 337, No. 8738, 16.02.1991, p. 392-394.

Research output: Contribution to journalArticle

McElvaney, NG, Hubbard, RC, Birrer, P, Crystal, R, Chernick, MS, Frank, MM & Caplan, DB 1991, 'Aerosol α1 -antitrypsin treatment for cystic fibrosis', The Lancet, vol. 337, no. 8738, pp. 392-394. https://doi.org/10.1016/0140-6736(91)91167-S
McElvaney NG, Hubbard RC, Birrer P, Crystal R, Chernick MS, Frank MM et al. Aerosol α1 -antitrypsin treatment for cystic fibrosis. The Lancet. 1991 Feb 16;337(8738):392-394. https://doi.org/10.1016/0140-6736(91)91167-S
McElvaney, N. G. ; Hubbard, R. C. ; Birrer, P. ; Crystal, Ronald ; Chernick, M. S. ; Frank, M. M. ; Caplan, D. B. / Aerosol α1 -antitrypsin treatment for cystic fibrosis. In: The Lancet. 1991 ; Vol. 337, No. 8738. pp. 392-394.
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