Introduction: Previous studies using magnetic resonance imaging (MRI) demonstrated early onset and progression of chronic rhinosinusitis (CRS) from infancy to school age, and response to lumacaftor/ivacaftor (LUM/IVA) therapy in children with cystic fibrosis (CF). However, the effect of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on CRS detected by MRI in children with CF and at least one F508del mutation, and potential incremental effects of ELX/TEZ/IVA compared to LUM/IVA in F508del homozygous children have not been studied.

Methods: 30 children with CF with at least one F508del mutation underwent three longitudinal paranasal sinus MRI before (MRI1), without (n = 16) or with LUM/IVA therapy (n = 14, MRI2), and with ELX/TEZ/IVA therapy (MRI3, mean age at therapy initiation 11.1 ± 3.4y, range 6–16y). MRI were evaluated using the CRS-MRI score.

Results: After therapy initiation with ELX/TEZ/IVA, the prevalence and in maxillary and sphenoid sinuses the dominance of mucopyoceles decreased (35% vs. 0 %, p<0.001 and 26% vs. 8 %, p < 0.05, respectively). This leads to a reduction in mucopyocele subscore (-3.4 ± 1.9, p < 0.001), and sinus subscores in MRI3 (maxillary sinus: -5.3 ± 3.1, p < 0.001, frontal sinus: -1.0 ± 1.9, p < 0.01, sphenoid subscore: -2.8 ± 3.5, p < 0.001, ethmoid sinus: -1.7 ± 1.9, p < 0.001). The CRS-MRI sum score decreased after therapy initiation with ELX/TEZ/IVA by -9.6 ± 5.5 score points (p < 0.001). The strength in reduction of mucopyoceles subscore and CRS-MRI sum score was independent of a pretreatment with LUM/IVA from MRI1-MRI2 (p = 0.275–0.999).

Conclusions: ELX/TEZ/IVA therapy leads to improvement of CRS in eligible children with CF. Our data support the role of MRI for comprehensive monitoring of CRS disease severity and response to therapy in children with CF.

简介：之前使用磁共振成像（MRI）的研究表明，慢性鼻窦炎（CRS）从婴儿期到学龄期的早期发病和进展，以及囊性纤维化（CF）儿童对lumacaftor/ivacaftor（LUM/IVA）治疗的反应。然而，elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) 对患有 CF 和至少一种 F508del 突变的儿童 MRI 检测到的 CRS 的影响，以及与 F508del 中的 LUM/IVA 相比，ELX/TEZ/IVA 的潜在增量效应尚未对纯合子儿童进行研究。

方法： 30 名患有至少一种 F508del 突变的 CF 儿童在术前接受了 3 次纵向鼻旁窦 MRI (MRI1)、未接受 LUM/IVA 治疗( n = 16) 或接受 LUM/IVA 治疗 ( n  = 14, MRI2) 以及接受 ELX/TEZ/IVA治疗（MRI3，开始治疗时的平均年龄 11.1 ± 3.4 岁，范围 6-16 岁）。使用 CRS-MRI 评分评估 MRI。

结果：开始使用 ELX/TEZ/IVA 治疗后，上颌窦和蝶窦中粘膜膨出的患病率和优势均下降（分别为 35% vs. 0 %，p <0.001 和 26% vs. 8 %，p  < 0.05） 。这导致 MRI3 中 mucopyocele 子评分 (-3.4 ± 1.9, p  < 0.001) 和窦子评分的降低（上颌窦：-5.3 ± 3.1，p  < 0.001，额窦：-1.0 ± 1.9，p  < 0.01，蝶骨子评分：-2.8 ± 3.5，p  < 0.001，筛窦：-1.7 ± 1.9，p  < 0.001）。开始使用 ELX/TEZ/IVA 治疗后，CRS-MRI 总分下降了 -9.6 ± 5.5 分 ( p  < 0.001)。mucopyoceles 子评分和 CRS-MRI 总评分的减少强度与 MRI1-MRI2 中 LUM/IVA 预处理无关（p  = 0.275–0.999）。

结论：ELX/TEZ/IVA 治疗可改善符合条件的 CF 儿童的 CRS。我们的数据支持 MRI 在全面监测 CF 儿童 CRS 疾病严重程度和治疗反应方面的作用。