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Published on: November 10, 2023
Bronchiolitis obliterans in children
Siby P Moonnumakal1, Leland L Fan
1Pediatric Pulmonary Section, Department of Pediatrics, Texas Children's Hospital, USA.
Insights
Bronchiolitis obliterans in children, often caused by adenovirus, is a rare obstructive lung disease. Diagnosis relies on clinical signs, lung function, and CT scans, with supportive treatment and variable prognosis.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Pediatric Critical Care
Background:
- Bronchiolitis obliterans is a rare, irreversible obstructive lung disease.
- It results from severe lower airway injury leading to small airway fibrosis.
- In children, adenovirus infection is the most frequent cause.
Purpose of the Study:
- To review current knowledge on the etiology, pathology, and clinical aspects of bronchiolitis obliterans.
- To discuss diagnostic methods, treatment strategies, and outcomes in pediatric patients.
- To focus on the nontransplant, pediatric population with this condition.
Main Methods:
- Literature review of recent advances in bronchiolitis obliterans research.
- Analysis of clinical presentations, diagnostic criteria, and treatment outcomes.
- Synthesis of information on etiology, pathology, and pathogenesis.
Main Results:
- Diagnosis is confident using clinical presentation (e.g., adenovirus history), pulmonary function tests, and CT findings (mosaic perfusion, bronchiectasis).
- Lung biopsy is often unnecessary.
- Postinfectious cases are typically chronic and nonprogressive, while Stevens-Johnson syndrome cases can be progressive and require transplantation.
Conclusions:
- Bronchiolitis obliterans is a rare chronic obstructive lung disease in children following severe respiratory insult.
- Adenovirus infection is the primary cause in the nontransplant pediatric population.
- Treatment is supportive, and prognosis depends on the cause and initial injury severity.
Purpose Of Review:
In this review, we discuss recent advances in our understanding of the etiology, pathology and pathogenesis, clinical presentation, diagnosis, treatment, and outcome of bronchiolitis obliterans in the nontransplant, pediatric population.
Recent Findings:
The diagnosis of bronchiolitis obliterans in children can be made with confidence based on clinical presentation, particularly with a history of adenovirus bronchiolitis or pneumonia, fixed obstructive lung disease on pulmonary function testing, and characteristic changes of mosaic perfusion, vascular attenuation, and central bronchiectasis on chest high-resolution computed tomography, thus avoiding the need for lung biopsy in most patients. Patients with postinfectious bronchiolitis obliterans generally have chronic, nonprogressive disease; in contrast, patients with bronchiolitis obliterans from Stevens-Johnson syndrome often have progressive disease that may require lung transplantation.
Summary:
Bronchiolitis obliterans is a rare form of chronic obstructive lung disease that follows a severe insult to the lower respiratory tract, resulting in fibrosis of the small airways. In the nontransplant pediatric population, adenovirus infection is the most common cause. Treatment is largely supportive and prognosis is mainly related to the underlying cause and to the severity of the initial insult.
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