What question did this study set out to answer?

To evaluate the short-term repeatability and treatment sensitivity of low signal volume using UTE MRI in pediatric cystic fibrosis.

May 6, 2026

Automated Ultrashort Echo Time ( UTE ) MRI Low Signal Volume Analysis in Stable Pediatric Cystic Fibrosis and After Elexacaftor/Tezacaftor/Ivacaftor Therapy

Key Points

To evaluate the short-term repeatability and treatment sensitivity of low signal volume using UTE MRI in pediatric cystic fibrosis.
Conducted longitudinal analysis in two groups of pediatric cystic fibrosis participants (N=27), scanned at baseline and after therapy.
Utilized 3D UTE MRI, along with spirometry and Xe-MRI, to assess lung function and gas trapping.
Applied Wilcoxon matched-pairs signed-rank test and Bland-Altman analysis to evaluate repeatability and treatment effects.
LSV was significantly reduced 1 month after initiating elexacaftor/tezacaftor/ivacaftor therapy (p < 0.05).
Observed strong correlations between LSV analysis and spirometry measures (|ρ| ≥ 0.59).
Bland-Altman analysis indicated that LSV measurements were repeatable in stable cystic fibrosis over the 1-month interval.

Abstract

BACKGROUND: * of pulmonary tissues, improving image quality. Abnormally low UTE signal intensities are associated with hallmarks of cystic fibrosis (CF), including gas trapping and lung hyperexpansion. PURPOSE: To explore short-term repeatability and sensitivity to treatment of low signal volume (LSV) from UTE MRI in pediatric CF. STUDY-TYPE: Single-site, retrospective, longitudinal. SUBJECTS: Thirteen participants with stable CF (6M/7F, median age = 15 years old) were scanned at baseline and 1-month to evaluate short-term repeatability. Subsequently, 14 CF participants (7M/7F median age = 16 years old) were scanned pre- and 1-month post-initiation of elexacaftor/tezacaftor/ivacaftor (ETI). FIELD STRENGTH/SEQUENCE: Three-dimensional stack-of-spirals for UTE, 2-dimensional gradient-echo for hyperpolarized xenon (Xe-MRI), 3-dimensional gradient-echo for thoracic cavity estimation at 3 T. ASSESSMENT: LSV was analyzed from UTE MRI. Same-day spirometry, multiple-breath washout, and Xe-MRI were also performed to compare to LSV. STATISTICAL TESTS: Differences were assessed with the Wilcoxon matched-pairs signed-rank test. Bland-Altman analysis and the Intraclass Correlation Coefficient (ICC) were used to assess 1-month repeatability in stable CF. Relationships between measures were assessed with Spearman correlation. p < 0.05 was considered significant. RESULTS: /FVC, LCI, and VDP (all |ρ| ≥ 0.59). DATA CONCLUSION: LSV analysis was feasible and repeatable in pediatric CF over a 1-month period. LSV was significantly reduced 1-month after ETI treatment, indicating sensitivity to reduced gas trapping, hyperexpansion, and obstruction. EVIDENCE LEVEL: 4. TECHNICAL EFFICACY: 1.

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Cite This Study

Zanette et al. (Mon,) studied this question.

synapsesocial.com/papers/69fadaab03f892aec9b1e5b8 https://doi.org/https://doi.org/10.1002/jmri.70333

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