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Updated: Jul 14, 2026

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
08:00

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study

Published on: April 11, 2018

Airway remodelling in children with cystic fibrosis

Tom N Hilliard1, Nicolas Regamey, Janis K Shute

  • 1Department of Gene Therapy, National Heart and Lung Institute, Imperial College, London, UK. tom.hilliard@ubht.nhs.uk

Thorax
|May 29, 2007
PubMed

Insights

Early cystic fibrosis (CF) lung disease shows two airway remodeling types: inflammation-related matrix breakdown impacting lung function, and TGF-beta(1)-related reticular basement membrane thickening.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Cystic Fibrosis Research

Background:

  • The interplay between airway structural changes and inflammation in early cystic fibrosis (CF) lung disease remains poorly understood.
  • This study investigated airway remodeling in children with CF compared to controls.

Purpose of the Study:

  • To determine the characteristics of airway remodeling in children with CF.
  • To compare airway structural changes in CF patients with those in primary ciliary dyskinesia (PCD) and chronic respiratory symptoms (CRS) groups.

Main Methods:

  • Cross-sectional study involving 43 children with CF, 7 with PCD, 26 with CRS, and 7 healthy controls.
  • Bronchoalveolar lavage (BAL) and endobronchial biopsy were performed.
  • Analysis included inflammatory cells, cytokines, proteases, matrix constituents in BAL fluid, and reticular basement membrane (RBM) thickness.

Main Results:

  • Elevated elastin, glycosaminoglycans, and collagen in BALF of CF patients correlated with age and inflammatory markers.
  • Matrix breakdown markers negatively correlated with pulmonary function (forced expiratory volume in 1s) in CF patients.
  • RBM thickness was significantly greater in CF patients and correlated with transforming growth factor-beta(1) (TGF-beta(1)).

Conclusions:

  • Two distinct airway remodeling patterns were identified in pediatric CF: matrix breakdown linked to inflammation and impaired lung function, and RBM thickening associated with TGF-beta(1).
  • RBM thickening appears independent of other inflammatory markers in early CF lung disease.
Abstract

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