Gower William A, Tamae-Kakazu Maximiliano, Shanthikumar Shivanthan, Srinivasan Saumini, Reardon Erin E, Barochia Amisha V, Charbek Edward, Calvo Charlotte, Cheng Pi Chun, Das Shailendra, Davies Stella M, Gross Jessica, Sheshadri Ajay, Towe Christoper T, Goldfarb Samuel B, Iyer Narayan P
Division of Pediatric Pulmonology, University of North Carolina School of Medicine, Chapel Hill, NC, USA.
Division of Pulmonary and Critical Care, Michigan State University College of Human Medicine and Spectrum Health, Grand Rapids, MI, USA.
BMC Pediatr. 2025 Mar 28;25(1):250. doi: 10.1186/s12887-025-05501-2.
Bronchiolitis obliterans syndrome (BOS) represents a significant source of morbidity and non-relapse mortality among children and young adults treated with allogeneic hematopoietic stem cell transplantation (aHSCT). Pulmonary function testing (PFT) pre- and post-aHSCT may allow for pre-symptomatic detection of BOS, and thus early intervention. Current guidelines and practices vary regarding which tests to perform and timing relative to transplant. A systematic review evaluating PFT before and after pediatric aHSCT was conducted to inform American Thoracic Society clinical practice guidelines on detection of BOS.
To determine the optimal approach to conducting PFT prior to and after pediatric aHSCT.
We performed a systematic review of the literature to identify studies of PFT in human aHSCT recipients < 25 years of age to address two questions: (1) Should pre-transplant screening PFT be performed in pediatric patients who will undergo aHSCT? (2) At what frequency should pediatric patients who have had aHSCT undergo PFT? We searched in Medline through August 2022 for studies that enrolled patients < 25 years of age being treated with aHSCT for whom PFT data were reported before or after transplant.
The 30 studies with pre-transplant PFT data showed a wide range of findings, with the majority demonstrating abnormalities. In studies reporting respiratory symptoms, 85-100% of patients were asymptomatic. In the 21 studies reporting post-transplant PFT, 11 used a surveillance strategy where at least one test was performed in the first year post-transplant. Median time to BOS diagnosis was 6-12 months in the regular surveillance studies, and 6-24 months in the others. Forced expiratory volume in one second at the time of BOS diagnosis was 38-84% predicted in studies with regular surveillance versus 44-57% predicted in studies with no surveillance. In the surveillance group, BOS was identified in some patients who were asymptomatic. Data quality in studies reviewed was moderate to very low.
Abnormalities in PFT are common in children prior to aHSCT. Regular monitoring in the first 1-2 years post-aHSCT may improve early and/or pre-symptomatic identification of BOS, but significant limitations may still be seen at the time of diagnosis. Higher quality data are needed.
闭塞性细支气管炎综合征（BOS）是接受异基因造血干细胞移植（aHSCT）的儿童和年轻人发病及非复发死亡的重要原因。aHSCT前后的肺功能测试（PFT）可能有助于在症状出现前检测出BOS，从而实现早期干预。目前关于进行哪些测试以及相对于移植的时间安排，指南和实践各不相同。我们进行了一项系统评价，评估儿科aHSCT前后的PFT，以为美国胸科学会关于BOS检测的临床实践指南提供参考。
确定儿科aHSCT前后进行PFT的最佳方法。
我们对文献进行了系统评价，以确定年龄小于25岁的人类aHSCT受者的PFT研究，以解决两个问题：（1）对于将接受aHSCT的儿科患者，是否应进行移植前筛查PFT？（2）接受aHSCT的儿科患者应多久进行一次PFT？我们在Medline中检索截至2022年8月的研究，纳入接受aHSCT治疗且报告了移植前后PFT数据的年龄小于25岁的患者。
30项有移植前PFT数据的研究结果差异很大，大多数显示异常。在报告有呼吸道症状的研究中，85%-100%的患者无症状。在21项报告移植后PFT的研究中，11项采用了监测策略，即在移植后的第一年至少进行一次测试。在定期监测的研究中，BOS诊断的中位时间为6-12个月，在其他研究中为6-24个月。在定期监测的研究中，BOS诊断时的一秒用力呼气量为预测值的38%-84%，而在无监测的研究中为44%-57%。在监测组中，一些无症状的患者被诊断为BOS。所审查研究的数据质量为中等至非常低。
PFT异常在儿科患者aHSCT前很常见。aHSCT后1-2年内进行定期监测可能会改善BOS的早期和/或症状前识别，但在诊断时仍可能存在明显局限性。需要更高质量的数据。
