Academic Journal
Automated Ultrashort Echo Time (UTE) MRI Low Signal Volume Analysis in Stable Pediatric Cystic Fibrosis and After Elexacaftor/Tezacaftor/Ivacaftor Therapy.
| Τίτλος: | Automated Ultrashort Echo Time (UTE) MRI Low Signal Volume Analysis in Stable Pediatric Cystic Fibrosis and After Elexacaftor/Tezacaftor/Ivacaftor Therapy. |
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| Συγγραφείς: | Zanette B; Translational Medicine Program, The Hospital for Sick Children, Toronto, Ontario, Canada., Bierbrier J; Translational Medicine Program, The Hospital for Sick Children, Toronto, Ontario, Canada., Munidasa S; Translational Medicine Program, The Hospital for Sick Children, Toronto, Ontario, Canada.; Department of Medical Biophysics, University of Toronto, Toronto, Ontario, Canada., Genkin D; Department of Electrical, Computer, and Biomedical Engineering, Toronto Metropolitan University, Toronto, Ontario, Canada., Kirby M; Department of Physics, Toronto Metropolitan University, Toronto, Ontario, Canada., Ratjen F; Translational Medicine Program, The Hospital for Sick Children, Toronto, Ontario, Canada.; Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada., Santyr G; Translational Medicine Program, The Hospital for Sick Children, Toronto, Ontario, Canada.; Department of Medical Biophysics, University of Toronto, Toronto, Ontario, Canada. |
| Πηγή: | Journal of magnetic resonance imaging : JMRI [J Magn Reson Imaging] 2026 Aug; Vol. 64 (2), pp. 442-451. Date of Electronic Publication: 2026 May 04. |
| Τύπος έκδοσης: | Journal Article |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: Wiley-Liss Country of Publication: United States NLM ID: 9105850 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1522-2586 (Electronic) Linking ISSN: 10531807 NLM ISO Abbreviation: J Magn Reson Imaging Subsets: MEDLINE |
| Imprint Name(s): | Publication: <2005-> : Hoboken , N.J. : Wiley-Liss Original Publication: Chicago, IL : Society for Magnetic Resonance Imaging, c1991- |
| Ιατρικοί όροι (MeSH): | Cystic Fibrosis*/diagnostic imaging , Cystic Fibrosis*/drug therapy , Magnetic Resonance Imaging*/methods , Quinolones*/therapeutic use , Aminophenols*/therapeutic use , Indoles*/therapeutic use , Pyrrolidines*/therapeutic use , Benzodioxoles*/therapeutic use , Pyrazoles*/therapeutic use , Pyridines*/therapeutic use, Lung/diagnostic imaging ; Image Processing, Computer-Assisted/methods ; Humans ; Female ; Male ; Adolescent ; Reproducibility of Results ; Child ; Retrospective Studies ; Drug Combinations ; Longitudinal Studies |
| Περίληψη: | Background: Ultrashort echo time (UTE) MRI overcomes the low signal intensity and short T 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: Baseline LSV was correlated with forced expiratory volume in 1 s (FEV 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. (© 2026 International Society for Magnetic Resonance in Medicine.) |
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| Grant Information: | MOP 123431 Canada CAPMC CIHR; PJT 153099 Canada CAPMC CIHR; Cystic Fibrosis Foundation |
| Contributed Indexing: | Keywords: UTE; cystic fibrosis; gas trapping; lung; pediatric Local Abstract: [plain-language-summary] Ultrashort echo time MRI has advanced for detailed structural imaging of the lungs and assessment of chronic lung diseases such as cystic fibrosis (CF). This study explores the quantification of regions of abnormally low UTE MRI signals for investigation of gas trapping, hyperinflation, and loss of lung tissue density in children with CF. We demonstrate this is feasible and agrees with pulmonary function testing and Xenon MRI. Improvements after initiation of elexacaftor/tezacaftor/ivacaftor were also observed, highlighting sensitivity to treatment. This work demonstrates the potential for automated and quantitative analysis of UTE MRI to study chronic lung disease in children. |
| Substance Nomenclature: | 0 (Quinolones) 0 (Aminophenols) 0 (Indoles) 0 (Pyrrolidines) 0 (Benzodioxoles) RRN67GMB0V (elexacaftor) 0 (Drug Combinations) 0 (Pyrazoles) 8RW88Y506K (tezacaftor) 0 (Pyridines) 1Y740ILL1Z (ivacaftor) |
| Entry Date(s): | Date Created: 20260504 Date Completed: 20260712 Latest Revision: 20260726 |
| Update Code: | 20260726 |
| PubMed Central ID: | PMC13356414 |
| DOI: | 10.1002/jmri.70333 |
| PMID: | 42080522 |
| Βάση Δεδομένων: | MEDLINE |
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