Comparison of phase-resolved functional lung (PREFUL) and hyperpolarized ¹²⁹Xe MRI for longitudinal monitoring of lung function in pediatric cystic fibrosis following elexacaftor/tezacaftor/ivacaftor.

IF 2.3 3区医学 Q2 PEDIATRICS

Pediatric Radiology Pub Date : 2025-09-12 DOI:10.1007/s00247-025-06390-4

Samal Munidasa, Faiyza Alam, Brandon Zanette, Daniel Li, Wallace Wee, Sharon Braganza, Jason Woods, Felix Ratjen, Giles Santyr

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引用次数: 0

Abstract

Background: Phase-resolved functional lung (PREFUL) MRI offers a more accessible alternative to hyperpolarized ¹²⁹Xe MRI (Xe-MRI) for monitoring treatment response in pediatric cystic fibrosis (CF), but longitudinal comparisons are limited.

Objective: To assess longitudinal lung function changes following elexacaftor/tezacaftor/ivacaftor (ETI) treatment initiation in CF children using PREFUL MRI, in comparison with Xe-MRI and pulmonary function tests (PFTs).

Materials and methods: PREFUL MRI, Xe-MRI, and PFTs were performed in 14 CF patients (median [IQR] age 15 [14-16.5] years old) at baseline and 1 month, 6 months, 12 months, and 24 months following initiation of ETI treatment. Ventilation and defect percentage (VDP) was derived from PREFUL MRI (regional ventilation VDP, VDP_RVent; regional-flow volume loop cross-correlation VDP, VDP_CC; and the combination of VDP_RVent and VDP_CC, VDP_combined) and Xe-MRI (VDP_Xe) maps. Perfusion defect percentage (QDP) was derived from normalized perfusion maps and, with VDP_combined, determined the percentage of healthy ventilation-perfusion matching (VQM). Significance of 1-month treatment changes was determined using the Wilcoxon-signed rank test and was correlated between metrics using Spearman ranked correlation.

Results: All PREFUL measures significantly improved (P < 0.01) 1-month post-treatment in agreement with changes in Xe-MRI VDP and PFTs (P < 0.03). The absolute change in VDP_CC and VDP_combined significantly correlated with VDP_Xe (r ≥ 0.62, P < 0.02), unlike VDP_RVent (P = 0.35). The change in QDP did not correlate with any metric (P > 0.10). PREFUL MRI and Xe-MRI measures showed minimal changes 1 to 24 months post-treatment (median changes = -2.3% to 1.4%), in agreement with PFTs.

Conclusion: PREFUL MRI detects longitudinal treatment-related changes in pulmonary ventilation and perfusion in CF children post ETI.

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相分辨功能肺（PREFUL）和超极化129Xe MRI纵向监测儿童囊性纤维化患者肺功能的比较，在elexaftor /tezacaftor/ivacaftor后。

背景：相分辨功能肺（PREFUL） MRI为监测儿童囊性纤维化（CF）的治疗反应提供了比超极化129Xe MRI （Xe-MRI）更容易获得的替代方案，但纵向比较有限。目的：利用PREFUL MRI评估CF儿童开始使用elexaftor /tezacaftor/ivacaftor （ETI）治疗后纵向肺功能的变化，并与Xe-MRI和肺功能测试（PFTs）进行比较。材料和方法：对14例CF患者（中位[IQR]年龄15[14-16.5]岁）在基线和开始ETI治疗后1个月、6个月、12个月和24个月进行了PREFUL MRI、x -MRI和PFTs检查。通气和缺陷百分比（VDP）来自PREFUL MRI（区域通气VDP， VDPRVent；区域-流量环相互关联VDP， VDPCC；以及VDPRVent和VDPCC的组合，VDPcombined）和Xe-MRI （VDPXe）图。灌注缺陷百分率（QDP）由归一化灌注图得出，并与vdp相结合，确定健康通气-灌注匹配百分率（VQM）。1个月治疗变化的显著性采用wilcoxon符号秩检验，指标间的相关性采用Spearman秩相关。结果：PREFUL各项指标均有显著改善(pcc和vdpx联合与vdpx显著相关（r≥0.62,P = 0.35）。QDP的变化与任何指标均无相关性（P < 0.10）。PREFUL MRI和x -MRI测量显示治疗后1至24个月的最小变化（中位变化= -2.3%至1.4%），与pft一致。结论：PREFUL MRI可检测CF患儿ETI后肺通气和灌注的纵向治疗相关改变。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Radiology 医学-核医学

CiteScore

4.40

自引率

17.40%

发文量

300

审稿时长

3-6 weeks

期刊介绍： Official Journal of the European Society of Pediatric Radiology, the Society for Pediatric Radiology and the Asian and Oceanic Society for Pediatric Radiology Pediatric Radiology informs its readers of new findings and progress in all areas of pediatric imaging and in related fields. This is achieved by a blend of original papers, complemented by reviews that set out the present state of knowledge in a particular area of the specialty or summarize specific topics in which discussion has led to clear conclusions. Advances in technology, methodology, apparatus and auxiliary equipment are presented, and modifications of standard techniques are described. Manuscripts submitted for publication must contain a statement to the effect that all human studies have been reviewed by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in an appropriate version of the 1964 Declaration of Helsinki. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study. Details that might disclose the identity of the subjects under study should be omitted.