Aerosol alpha 1-antitrypsin treatment for cystic fibrosis. Academic Article uri icon

Overview

MeSH

  • Adult
  • Aerosols
  • Bronchoalveolar Lavage Fluid
  • Evaluation Studies as Topic
  • Female
  • Humans
  • Leukocyte Elastase
  • Male
  • Pancreatic Elastase
  • Pseudomonas aeruginosa

MeSH Major

  • Cystic Fibrosis
  • alpha 1-Antitrypsin

abstract

  • 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

has subject area

  • Adult
  • Aerosols
  • Bronchoalveolar Lavage Fluid
  • Cystic Fibrosis
  • Evaluation Studies as Topic
  • Female
  • Humans
  • Leukocyte Elastase
  • Male
  • Pancreatic Elastase
  • Pseudomonas aeruginosa
  • alpha 1-Antitrypsin

Research

keywords

  • Journal Article

Identity

Language

  • eng

PubMed ID

  • 1671425

Additional Document Info

start page

  • 392

end page

  • 394

volume

  • 337

number

  • 8738