Plasma biomarkers and cystic fibrosis lung disease.

Purpose: The purpose of this study was to determine whether plasma biomarkers reflect changes in lung function and respiratory exacerbations associated with CF lung disease.

Methods: Plasma human leukocyte elastase/alpha1 antitrypsin complex (pHLE complex) values were measured in 28 adult CF patients and 47 healthy volunteers and correlated with forced expiratory volume (FEV1) and forced vital capacity (FVC). pHLE complexes were studied during respiratory exacerbations and after antibiotic therapy.

Infection of polarized airway epithelial cells by normal and small-colony variant strains of Staphylococcus aureus is increased in cells with abnormal cystic fibrosis transmembrane conductance regulator function and is influenced by NF-κB.

The infection of nonphagocytic host cells by Staphylococcus aureus and more particularly by small-colony variants (SCVs) may contribute to the persistence of this pathogen in the lungs of cystic fibrosis (CF) patients. The development of chronic infections is also thought to be facilitated by the proinflammatory status of CF airways induced by an activation of NF-κB. The aim of this study was to compare the infection of non-CF and CF-like airway epithelial cells by S.

Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers.

Rationale: Cigarette smoke extract inhibits chloride secretion in human bronchial epithelial cells. Oxidants decrease gene expression, protein expression, and function of the cystic fibrosis transmembrane conductance regulator (CFTR).

Objectives: Because cigarette smoke is a rich source of oxidants, we verified the hypothesis that CFTR may be suppressed by exposure to cigarette smoke in vitro and in vivo.

Oxidant stress suppresses CFTR expression.

Epithelial mucous membranes are repeatedly exposed to oxidants and xenobiotics. CFTR plays a role in glutathione transepithelial flux and in defining the hydration and viscoelasticity of protective mucus. We therefore hypothesized that CFTR expression and function may be modulated by oxidant stress.