Division of Allergology Pulmonology and Cystic fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Division for Stem Cell Transplantation and Immunology, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Pediatr Pulmonol. 2020 Jul;55(7):1725-1735. doi: 10.1002/ppul.24801. Epub 2020 May 5.
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.
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).
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.
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.
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.
闭塞性细支气管炎综合征（BOS）是一种严重的气道慢性炎症，导致细支气管阻塞。到目前为止，仅有少数研究关注 BOS 患儿肺部损伤的长期发展。
本研究旨在探讨接受异基因造血干细胞移植（HSCT）的儿童中 BOS 的发生率和长期结局。
回顾性分析了 2000 年至 2017 年在德国法兰克福接受 HSCT 的 526 名儿童的病历，结果确定了 14 名 BOS 患儿。对这 14 名 BOS 患儿共进行了 271 次肺功能（肺活量和体描法）、26 次肺清除指数（LCI）和 46 次胸部高分辨率计算机断层扫描（HRCT）检查。
14 名患儿在 HSCT 后发生 BOS（2.7%），其中观察到 3 种不同类型的肺功能损伤模式：14 名患儿中的 3 名表现为进行性肺功能下降；2 名死亡，1 名接受肺移植。14 名 BOS 患儿中有 5 名患儿的肺功能持续存在严重阻塞性和继发性限制性模式（用力肺活量 [FVC]＜60%，1 秒用力呼气量 [FEV1]＜50%，FEV1/FVC＜0.7）和 LCI 升高（11.67-20.9）；14 名患儿中有 6 名患儿在 HRCT 上出现 BOS 后肺功能中度受损且有相关迹象，其完全恢复。长期 FVC 的绝对值增加表明患儿仍有肺生长。
我们的研究结果表明，儿童 BOS 的发生率较低。BOS 与高死亡率相关，可能导致持续性阻塞性肺病；然而，肺生长仍在继续。
