Protease-antiprotease imbalance in the lungs of children with cystic fibrosis. Academic Article uri icon

Overview

MeSH

  • Adolescent
  • Bronchoalveolar Lavage Fluid
  • Cell Count
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Leukocyte Elastase
  • Male
  • Neutrophils
  • Proteinase Inhibitory Proteins, Secretory
  • Secretory Leukocyte Peptidase Inhibitor

MeSH Major

  • Cystic Fibrosis
  • Lung
  • Pancreatic Elastase
  • Proteins
  • Serine Proteinase Inhibitors
  • alpha 1-Antitrypsin

abstract

  • Cystic fibrosis (CF) is characterized in the lung by chronic purulent bronchitis culminating in pulmonary insufficiency. There is evidence to suggest that neutrophil elastase (NE) released by neutrophils on the respiratory epithelial surface plays a major role in the pathogenesis of this lung disease. This study sought to determine the age of onset of the chronic neutrophil-dominated inflammation in CF and the consequences to the NE-anti-NE screen on the respiratory epithelial surface of the CF lung. NE and anti-NE defensive molecules were evaluated in respiratory epithelial lining fluid (ELF) in 27 children with stable CF (1 to 18 yr of age). Despite normal antigenic concentrations of alpha 1-antitrypsin (alpha 1AT) and secretory leukoprotease inhibitor (SLPI), 25 of 27 children with CF had neutrophil-dominated inflammation (> 500 neutrophils/microliters ELF). Active NE was found in ELF in 20 of 27 children, including two of four aged 1 yr. Western blot analysis showed the majority of alpha 1AT and SLPI molecules to be complexed and/or degraded. These observations demonstrate that a chronic imbalance of the NE-anti-NE protective screen develops early on the respiratory epithelial surface in persons with CF and is likely well established by 1 yr of age, with resultant potential for lung damage.

publication date

  • July 1994

has subject area

  • Adolescent
  • Bronchoalveolar Lavage Fluid
  • Cell Count
  • Child
  • Child, Preschool
  • Cystic Fibrosis
  • Female
  • Humans
  • Infant
  • Leukocyte Elastase
  • Lung
  • Male
  • Neutrophils
  • Pancreatic Elastase
  • Proteinase Inhibitory Proteins, Secretory
  • Proteins
  • Secretory Leukocyte Peptidase Inhibitor
  • Serine Proteinase Inhibitors
  • alpha 1-Antitrypsin

Research

keywords

  • Journal Article

Identity

Language

  • eng

Digital Object Identifier (DOI)

  • 10.1164/ajrccm.150.1.7912987

PubMed ID

  • 7912987

Additional Document Info

start page

  • 207

end page

  • 213

volume

  • 150

number

  • 1