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Published on: July 10, 2012
Diagnosing and managing bronchiolitis obliterans in children
Ema Kavaliunaite1, Paul Aurora1,2
1a Respiratory Unit , Great Ormond Street Hospital for Children NHS Foundation Trust , London , UK.
Insights
Bronchiolitis obliterans (BO) is a serious lung disease in children, often post-infectious. Current understanding of its development and effective treatments remains limited, necessitating expert care.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
Background:
- Bronchiolitis obliterans (BO) is a chronic, irreversible obstructive lung disease affecting small airways.
- Key entities include post-infectious BO (PIBO), and BO post lung or bone marrow transplantation (BMT/HSCT).
- Similarities in histopathology and development pathways exist across BO entities.
Purpose of the Study:
- To review current evidence on the diagnosis and management of bronchiolitis obliterans in children.
- To highlight diagnostic approaches and current treatment limitations.
Main Methods:
- Review of existing literature on pediatric bronchiolitis obliterans.
- Analysis of diagnostic criteria, including clinical, radiological, and histopathological findings.
- Evaluation of current treatment evidence, particularly for post-infectious BO.
Main Results:
- Diagnosis often combines clinical history, imaging, and sometimes lung biopsy.
- Understanding of BO development mechanisms is incomplete.
- Strong evidence for effective treatments is lacking, with most data from adult transplant patients.
Conclusions:
- Bronchiolitis obliterans management in children requires a multidisciplinary approach.
- Care should be centralized in specialized centers due to complexity and limited treatment options.
- Further research is needed to elucidate BO pathogenesis and develop targeted therapies.
Introduction:
Bronchiolitis obliterans (BO) is a chronic and irreversible obstructive lung disease leading to the obstruction and/or obliteration of the small airways. Three main BO entities are distinguished: post-infectious BO (PIBO); BO post lung transplantation; and BO after bone marrow transplantation (BMT) or hematopoietic stem cell transplantation (HSCT). All three entities are separate, however, there are similarities in histopathological characteristics and possibly in aspects of the development pathway. Areas covered: We review current evidence of bronchiolitis obliterans diagnosis and management in children. The diagnosis of BO is usually based on a combination of history, clinical and radiological findings, although lung biopsy and histopathology remain the gold standard approaches to confirm BO. Expert opinion: At present, we do not have a clear understanding of the mechanisms of the development of BO and lack strong evidence for treatment. Although most BO in children is post-infectious, most of the current evidence for treatment originates from studies analyzing BO in adult lung transplant and HSCT patients. BO management requires multidisciplinary approach and care in specialized centers.
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