An Ex Vivo Model of Post Infectious Bronchiolitis Obliterans in Children Using Reconstituted Human Bronchial

Julie Mazenq1,2, Léa Moreno2, Jean-Christophe Dubus1,2

  • 1Pediatric Pulmonology Department and Reference Center for Rare Lung Disease RespiRare, Assistance Publique des Hôpitaux de Marseille (AP-HM), Hôpital Universitaire Timone-Enfants, 13385 Marseille, France.

Biomolecules
|May 27, 2026
PubMed

Insights

Children with post-infectious bronchiolitis obliterans (PIBO) may have structurally normal airways but altered immune responses. This study suggests PIBO epithelium has reduced alarmins and increased interferon secretion, indicating a distinct antiviral profile.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Cell Biology
  • Chronic Lung Disease Research

Background:

  • Post-infectious bronchiolitis obliterans (PIBO) is a severe chronic lung disease in children.
  • The characteristics of the respiratory epithelium in PIBO are not well understood.
  • An ex vivo model was used to investigate PIBO epithelial cells.

Purpose of the Study:

  • To characterize the respiratory epithelium in children with PIBO.
  • To analyze epithelial cell composition, barrier function, and mediator production.
  • To explore potential differences in inflammatory and antiviral responses.

Main Methods:

  • Used an air-liquid interface culture model with bronchial biopsies from PIBO patients and controls.
  • Assessed epithelial cell composition, barrier integrity, and mediator secretion (mucins, inflammatory cytokines, interferons).
  • Evaluated remodeling markers like MMP-9 and YKL-40.

Main Results:

  • Ex vivo PIBO epithelia showed similar cohesion and cell composition to controls.
  • Mucin expression and secretion were comparable between PIBO and control groups.
  • PIBO epithelia exhibited reduced IL-33 and TSLP but increased IFN-λ and IFN-β secretion, with no changes in remodeling markers.

Conclusions:

  • The ex vivo PIBO epithelial model suggests preserved structural characteristics and mucin production.
  • PIBO epithelial cells display a distinct immune profile with lower alarmin and higher interferon secretion.
  • These findings suggest a potential shift towards enhanced antiviral response rather than structural airway remodeling in PIBO.
Abstract

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