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Long-term pulmonary function testing in pediatric bronchiolitis obliterans syndrome after hematopoietic stem cell
Sophie Walther1, Eva Rettinger2, Hannah Miriam Maurer1
1Division of Allergology Pulmonology and Cystic fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Insights
Bronchiolitis obliterans syndrome (BOS) is rare in children after stem cell transplants but can cause severe lung issues. Despite risks, some children show lung growth, though mortality remains high.
Area of Science:
- Pediatric Pulmonology
- Hematology
- Transplantation Medicine
Background:
- Bronchiolitis obliterans syndrome (BOS) is a severe airway inflammation causing bronchiolar obstruction.
- Long-term pulmonary outcomes in pediatric BOS post-hematopoietic stem cell transplantation (HSCT) are understudied.
Purpose of the Study:
- To determine the incidence and long-term outcomes of BOS in children following allogeneic HSCT.
- To characterize the patterns of lung function impairment and recovery in pediatric BOS patients.
Main Methods:
- Retrospective analysis of 526 pediatric HSCT cases (2000-2017).
- Evaluation of pulmonary function tests (spirometry, body plethysmography), lung clearance index (LCI), and high-resolution computed tomography (HRCT) in 14 identified BOS patients.
Main Results:
- BOS occurred in 2.7% (14/526) of pediatric HSCT recipients.
- Observed patterns included progressive decline (3 patients), persistent severe obstruction (5 patients), and complete recovery (6 patients).
- Despite impairment, long-term forced vital capacity (FVC) indicated continued lung growth in survivors.
Conclusions:
- Pediatric BOS post-HSCT is infrequent but associated with significant mortality and potential for persistent obstructive lung disease.
- While lung growth continues, careful monitoring for long-term pulmonary complications is essential.
Rationale:
Bronchiolitis obliterans syndrome (BOS) is a severe, chronic inflammation of the airways leading to an obstruction of the bronchioles. So far, there are only a few studies looking at the long-term development of pulmonary impairment in children with BOS.
Objective:
The objective of this study was to investigate the incidence and long-term outcome of BOS in children who underwent allogeneic hematopoietic stem cell transplantation (HSCT).
Methods:
Medical charts of 526 children undergoing HSCT in Frankfurt/Main, Germany between 2000 and 2017 were analyzed retrospectively and as a result, 14 patients with BOS were identified. A total of 271 lung functions (spirometry and body plethysmography), 26 lung clearance indices (LCI), and 46 chest high-resolution computed tomography (HRCT) of these 14 patients with BOS were evaluated.
Results:
Fourteen patients suffered from BOS after HSCT (2.7%), whereby three distinctive patterns of lung function impairment were observed: three out of 14 patients showed a progressive lung function decline; two died and one received a lung transplant. In five out of 14 patients with BOS persisted with a severe obstructive and secondarily restrictive pattern in lung function (forced vital capacity [FVC] < 60%, forced expiratory volume in 1 second [FEV1] < 50%, and FEV1/FVC < 0.7) and increased LCI (11.67-20.9), six out of 14 patients recovered completely after moderate lung function impairment and signs of BOS on HRCT. Long-term FVC in absolute numbers was increased indicating that the children still have lung growth.
Conclusion:
Our results showed that the incidence of BOS in children is low. BOS was associated with high mortality and may lead to persistent obstructive lung disease; although, lung growth continued to exist.
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