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Updated: May 12, 2026

Development of Obliterative Bronchiolitis in a Murine Model of Orthotopic Lung Transplantation
10:01

Development of Obliterative Bronchiolitis in a Murine Model of Orthotopic Lung Transplantation

Published on: July 10, 2012

Managing bronchiolitis obliterans syndrome (BOS) and chronic lung allograft dysfunction (CLAD) in children: what does

Gregory I Snell1, Miranda Paraskeva, Glen P Westall

  • 1National Paediatric Lung Transplant Service, Alfred Hospital and Monash University, Melbourne 3004, Australia. g.snell@alfred.org.au

Paediatric Drugs
|April 23, 2013
PubMed

Insights

Pediatric lung transplant recipients face long-term graft dysfunction, including bronchiolitis obliterans syndrome (BOS) and restrictive allograft syndrome (RAS). Further research is needed to address these chronic lung allograft dysfunction (CLAD) challenges in children.

Area of Science:

  • Pediatric Pulmonology
  • Transplant Immunology
  • Critical Care Medicine

Background:

  • Long-term graft dysfunction limits pediatric lung transplant success, historically attributed to bronchiolitis obliterans syndrome (BOS).
  • Chronic lung allograft dysfunction (CLAD) encompasses other etiologies, including restrictive allograft syndrome (RAS), characterized by restrictive spirometry.
  • RAS, though unstudied in pediatrics, is linked to poor compliance, antibody-mediated rejection (AMR), and viral infections, necessitating pediatric attention.

Purpose of the Study:

  • To highlight the emerging challenge of restrictive allograft syndrome (RAS) within chronic lung allograft dysfunction (CLAD) in pediatric lung transplantation.
  • To underscore the need for pediatric lung transplant teams to recognize and investigate RAS, considering its associations with AMR and viral infections.
  • To advocate for collaborative research efforts to understand and manage CLAD, including RAS, in pediatric lung transplant recipients.

Main Methods:

  • Review of existing studies on bronchiolitis obliterans syndrome (BOS) to identify cases with restrictive spirometric patterns.
  • Analysis of factors associated with RAS, such as poor compliance, antibody-mediated rejection (AMR), and post-infectious lung damage.
  • Proposal for joint projects through collaborative groups like the International Pediatric Lung Transplant Collaborative.

Main Results:

  • Current therapies for BOS primarily involve adjusting immunosuppression and minimizing infection risk.
  • The efficacy of long-term macrolide therapy for lung transplant recipients with CLAD is not definitively established.
  • The specific characteristics and prevalence of RAS in pediatric lung transplant recipients remain understudied.

Conclusions:

  • Chronic lung allograft dysfunction (CLAD), encompassing both obstructive (BOS) and restrictive (RAS) patterns, is a significant long-term complication in pediatric lung transplantation.
  • Understanding the associations of RAS with AMR and viral infections is crucial for pediatric lung transplant management.
  • Collaborative research and review of existing BOS data are essential next steps to address CLAD and RAS in pediatric lung transplant recipients.

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