ArticleRadiology. Cardiothoracic imaging2024
PREFUL MRI for Monitoring Perfusion and Ventilation Changes after Elexacaftor-Tezacaftor-Ivacaftor Therapy for Cystic Fibrosis: A Feasibility Study.
Open access · greenAbstract readMulticenter Study
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Article in Radiology. Cardiothoracic imaging, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.
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25citing papers in PubMed, 1 pooled it
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Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 32 citations in OpenAlex.
- Non-contrast enhanced functional lung MRI in children: systematic review.Frontiers in pediatrics · 2025Pooled it
- Adapted Linear Binning Method to Assess Pulmonary Ventilation and Perfusion in Children Using Lung Matrix Pencil Decomposition (MP)-MRI.Magnetic resonance in medicine · 2026Article
- Automated Ultrashort Echo Time (UTE) MRI Low Signal Volume Analysis in Stable Pediatric Cystic Fibrosis and After Elexacaftor/Tezacaftor/Ivacaftor Therapy.Journal of magnetic resonance imaging : JMRI · 2026Article
- Characterizing chronic obstructive pulmonary disease using quantitative MRI biomarkers.The British journal of radiology · 2026Review
- Characteristics of pulmonary perfusion and ventilation in healthy adults: a prospective observational study with phase-resolved functional lung magnetic resonance imaging.BMC medical imaging · 2026Observational
- Phase-resolved functional lung magnetic resonance imaging for evaluation of lung perfusion and ventilation in fibrosing interstitial lung diseases.Insights into imaging · 2026Article
- Analysis of Pulsatile Vessel Expansion in Healthy, COPD- and PH-Patients Using Dynamic Vessel Segmentation in Free-Breathing Lung MRI.Journal of magnetic resonance imaging : JMRI · 2026Article
- Comparison of Signal- and Volume-Based Ventilation-Weighted Assessment Using 3D FLORET UTE MRI in Patients With Various Pulmonary Disease.Magnetic resonance in medicine · 2026Article
- Accelerating PREFUL MRI: Comparison of Registration Methods and Impact of Time Series Reduction.NMR in biomedicine · 2026Article
- Structural and Functional Pulmonary MRI to Predict Pulmonary Exacerbations in Cystic Fibrosis.Chest · 2026Article
- Comparison of phase-resolved functional lung (PREFUL) and hyperpolarizedPediatric radiology · 2025Article
- [Pulmonary imaging of cystic fibrosis with a focus on children and adolescents].Radiologie (Heidelberg, Germany) · 2025Review
- Magnetic Resonance Imaging in the Clinical Evaluation of Lung Disorders: Current Status and Future Prospects.Journal of magnetic resonance imaging : JMRI · 2025Review
- Bronchial thermoplasty reduces ventilation heterogeneity measured by phase-resolved functional lung magnetic resonance imaging in severe asthma.Respiratory research · 2025Article
- Quantitative MRI detects delayed perfusion and impact of bronchial artery dilatation on pulmonary circulation in patients with cystic fibrosis.European radiology · 2025Article
- Evaluation of pulmonary perfusion and ventilation in suspected chronic thromboembolic pulmonary hypertension via phase-resolved functional lung magnetic resonance imaging: correlations with hemodynamics and cardiopulmonary function.Quantitative imaging in medicine and surgery · 2025Article
- A multicenter retrospective study protocol comparing thoracoscopic anatomical lesion resection and lobectomy in children with congenital lung malformations.Scientific reports · 2025Article
- Review
- Assessment of ventilation heterogeneity in severe asthma using phase-resolved functional lung magnetic resonance imaging.Physiological reports · 2025Article
- Progress of personalized medicine of cystic fibrosis in the times of efficient CFTR modulators.Molecular and cellular pediatrics · 2025Review
Corrections and comments
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Authors and funding
12 authors at 1 institution in 1 country.
Martha DohnaFrom the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0002-2471-8530
Andreas VoskrebenzevFrom the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0001-5699-1841
Filip KlimešFrom the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0003-2715-0757
Till F KaireitFrom the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0003-4007-4904
Julian GlandorfFrom the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0003-1927-9876
Sophia T PallenbergFrom the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0002-9948-7589
Felix C RingshausenFrom the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0001-9084-4968
Gesine HansenFrom the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0001-5185-9454
Diane Miriam RenzFrom the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0002-3702-356X
Frank WackerFrom the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0002-6285-8403
Anna-Maria Dittrich *From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0002-9582-1025
Jens Vogel-Claussen *From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).ORCID 0000-0001-5595-6948
Medizinische Hochschule Hannover · DE
Funding
No grant is acknowledged in the PubMed record.
Abstract
Purpose To assess the feasibility of monitoring the effects of elexacaftor-tezacaftor-ivacaftor (ETI) therapy on lung ventilation and perfusion in people with cystic fibrosis (CF), using phase-resolved functional lung (PREFUL) MRI. Materials and Methods This secondary analysis of a multicenter prospective study was carried out between August 2020 and March 2021 and included participants 12 years or older with CF who underwent PREFUL MRI, spirometry, sweat chloride test, and lung clearance index assessment before and 8-16 weeks after ETI therapy. For PREFUL-derived ventilation and perfusion parameter extraction, two-dimensional coronal dynamic gradient-echo MR images were evaluated with an automated quantitative pipeline. T1- and T2-weighted MR images and PREFUL perfusion maps were visually assessed for semiquantitative Eichinger scores. Wilcoxon signed rank test compared clinical parameters and PREFUL values before and after ETI therapy. Correlation of parameters was calculated as Spearman ρ correlation coefficient. Results Twenty-three participants (median age, 18 years [IQR: 14-24.5 years]; 13 female) were included. Quantitative PREFUL parameters, Eichinger score, and clinical parameters (lung clearance index = 21) showed significant improvement after ETI therapy. Ventilation defect percentage of regional ventilation decreased from 18% (IQR: 14%-25%) to 9% (IQR: 6%-17%) (
Indexed as
AminophenolsBenzodioxolesCystic FibrosisIndolesPyrazolesPyridinesPyrrolidinesQuinolonesAdolescentFeasibility StudiesFemaleHumansLungMagnetic Resonance ImagingMalePerfusionAminophenolsBenzodioxoleselexacaftorIndolesivacaftorPyrazolesPyridinesPyrrolidinesQuinolonestezacaftorCFTRComparative StudiesCystic FibrosisElexacaftor-Tezacaftor-Ivacaftor TherapyFourier DecompositionFree‐Breathing Proton MRIFunctional MRIKaftrioLungModulator TherapyMR–Functional ImagingPediatricsPerfusionPREFULPulmonaryPulmonary MRI
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