Department of Respiratory, Children's University Hospital, Temple Street, Dublin 1.
Department of Pharmacy, Children's University Hospital, Temple Street, Dublin 1.
Ir Med J. 2020 May 7;113(5):70.
Aim The aim of this study was to explore risk factors for acute changes in lung function following initiation of lumacaftor/ivacaftor (LUM/IVA) in children with cystic fibrosis. Methods Retrospective review of all children commenced on LUM/IVA treatment over a one-year period. CT Thorax images were reviewed for evidence of air trapping using the Brody score. Results Data was collected from 15 children. A transient decline in ppFEV1 was observed after initiation of LUM/IVA in 93% (n=14) of patients with an absolute mean decline of -10.8%. There was a statistically significant inverse relationship between ΔFEV1 and baseline ppFEV1. There was no relationship between air trapping score and ΔFEV1 (p=0.41). Conclusion Pre-existing small airways disease is not a risk factor for acute changes in lung function following initiation of LUM/IVA. Our results suggest that a LUM/IVA-related decline in lung function is more significant in CF children with higher baseline FEV1.
目的 本研究旨在探讨在囊性纤维化（CF）儿童中，开始使用卢美哌南/依伐卡托（LUM/IVA）后肺功能急性变化的风险因素。
方法 对一年期间开始接受 LUM/IVA 治疗的所有儿童进行回顾性审查。使用 Brody 评分对胸部 CT 图像进行空气潴留的证据进行评估。
结果 共收集了 15 名儿童的数据。93%（n=14）的患者在开始 LUM/IVA 治疗后，ppFEV1 出现一过性下降，绝对值平均下降-10.8%。ΔFEV1 与基线 ppFEV1 呈显著负相关。空气潴留评分与 ΔFEV1 之间无相关性（p=0.41）。
结论 存在先前的小气道疾病并不是开始使用 LUM/IVA 后肺功能急性变化的风险因素。我们的结果表明，基线 FEV1 较高的 CF 儿童，LUM/IVA 相关的肺功能下降更为显著。
