TY  - JOUR
A1  - Walther, Sophie
A1  - Rettinger, Eva
A1  - Maurer, Hannah Miriam
A1  - Pommerening, Helena
A1  - Jarisch, Andrea
A1  - Sörensen, Jan
A1  - Schubert, Ralf
A1  - Berres, Martin
A1  - Bader, Peter
A1  - Zielen, Stefan
A1  - Jerkic, Silvija-Pera
T1  - Long‐term pulmonary function testing in pediatric bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation
T2  - Pediatric Pulmonology
N2  - 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.
KW  - body plethysmography
KW  - bronchiolitis obliterans syndrome
KW  - lung function
KW  - spirometry
Y1  - 2020
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/56226
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-562267
SN  - 1099-0496
SN  - 8755-6863
VL  - 55
IS  - 7
SP  - 1725
EP  - 1735
PB  - Wiley-Blackwell
CY  - Hoboken, NJ
ER  -