Department of Pediatrics and Adolescent Medicine, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
Pediatric Pulmonary Service, Department of Pediatrics and Adolescent Medicine, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
Pediatr Pulmonol. 2019 Jul;54(7):1029-1038. doi: 10.1002/ppul.24340. Epub 2019 Apr 19.
Pulmonary chronic graft-vs-host disease (cGvHD) after hematopoietic stem cell transplantation (HSCT) is characterized by impairment of the small airways. Assessment of lung clearance index (LCI) gained from multiple breath washout (MBW) is more sensitive than spirometry in detection of small airways disease. The aim of this study was to describe the development of LCI during the first year after pediatric HSCT and how LCI relates to other pulmonary function parameters and cGvHD.
This prospective, longitudinal study included 28 pediatric HSCT-recipients. Spirometry, Sulfur hexafluoride MBW and diffusion capacity of the lungs were performed before and at 3, 6, 9, and 12 months after HSCT. Respiratory symptoms and signs of cGvHD were recorded at each visit.
Before HSCT, 47.8% had abnormal LCI and 12.5% had abnormal forced expiratory volume in 1 second (FEV ) Patients with persisting respiratory symptoms 12 months post-HSCT had higher median LCI (factor 5.7, P = 0.0018) and lower FEV z-scores (-1.5, P = 0.033) post-HSCT compared to patients free of respiratory symptoms. Overall, post-HSCT LCI values were 3.49 times higher and FEV was 2.31 z-scores lower in eight patients with cGvHD in any organ system compared with patients without cGvHD (P = 0.0089 and P < 0.0001). LCI values during the first 3 months were not predictive of pulmonary cGvHD.
LCI is a sensitive marker for cGvHD and high LCI values were associated with persisting respiratory symptoms after 1 year. Further evaluation of MBW in early detection of HSCT-related pulmonary complications require larger patient cohorts and closer follow-up during the first months after HSCT.
造血干细胞移植（HSCT）后肺部慢性移植物抗宿主病（cGvHD）的特征是小气道受损。从多次呼吸冲洗（MBW）中获得的肺清除指数（LCI）评估比肺活量计更能检测到小气道疾病。本研究的目的是描述儿童 HSCT 后第一年 LCI 的发展情况，以及 LCI 与其他肺功能参数和 cGvHD 的关系。
这项前瞻性、纵向研究纳入了 28 名儿童 HSCT 受者。在 HSCT 前和 HSCT 后 3、6、9 和 12 个月进行肺活量计、六氟化硫 MBW 和肺扩散能力检查。在每次就诊时记录呼吸症状和 cGvHD 的体征。
在 HSCT 前，47.8%的患者 LCI 异常，12.5%的患者第一秒用力呼气量（FEV ）异常。HSCT 后 12 个月仍有呼吸症状的患者 LCI 中位数较高（倍数 5.7，P = 0.0018），FEV z 评分较低（-1.5，P = 0.033）。总体而言，与无 cGvHD 的患者相比，任何器官系统均有 cGvHD 的 8 名患者的 HSCT 后 LCI 值高 3.49 倍，FEV 值低 2.31 z 分（P = 0.0089 和 P < 0.0001）。HSCT 后前 3 个月的 LCI 值不能预测肺部 cGvHD。
LCI 是 cGvHD 的敏感标志物，高 LCI 值与 1 年后持续存在的呼吸症状相关。需要更大的患者队列和在 HSCT 后最初几个月内更密切的随访来进一步评估 MBW 在早期检测 HSCT 相关肺部并发症中的作用。
