Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease

This is a prospective, observational study involving three Cystic Fibrosis (CF) adult patients, evaluating the changes in chest magnetic resonance imaging (MRI) three months after the start of elexacaftor/tezacaftor and ivacaftor therapy. MRI showed a drastic reduction in mucus plugging and bronchia...

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Autores principales: Macconi, Letizia, Galici, Valeria, Di Maurizio, Marco, Rossi, Enrica, Taccetti, Giovanni, Terlizzi, Vito
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Communication
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9331995/
https://www.ncbi.nlm.nih.gov/pubmed/35893365
http://dx.doi.org/10.3390/jcm11154277
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author Macconi, Letizia
Galici, Valeria
Di Maurizio, Marco
Rossi, Enrica
Taccetti, Giovanni
Terlizzi, Vito
author_facet Macconi, Letizia
Galici, Valeria
Di Maurizio, Marco
Rossi, Enrica
Taccetti, Giovanni
Terlizzi, Vito
author_sort Macconi, Letizia
collection PubMed
description This is a prospective, observational study involving three Cystic Fibrosis (CF) adult patients, evaluating the changes in chest magnetic resonance imaging (MRI) three months after the start of elexacaftor/tezacaftor and ivacaftor therapy. MRI showed a drastic reduction in mucus plugging and bronchial wall thickening, with an improvement in the diffusion-weighted MRI score. Similarly, a marked improvement in spirometric parameters, nutritional status, and sweat chloride was observed. Our preliminary data confirm that chest MRI could be a useful tool to assess disease progression in CF patients on modulatory drug therapy.
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spelling pubmed-93319952022-07-29 Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease Macconi, Letizia Galici, Valeria Di Maurizio, Marco Rossi, Enrica Taccetti, Giovanni Terlizzi, Vito J Clin Med Communication This is a prospective, observational study involving three Cystic Fibrosis (CF) adult patients, evaluating the changes in chest magnetic resonance imaging (MRI) three months after the start of elexacaftor/tezacaftor and ivacaftor therapy. MRI showed a drastic reduction in mucus plugging and bronchial wall thickening, with an improvement in the diffusion-weighted MRI score. Similarly, a marked improvement in spirometric parameters, nutritional status, and sweat chloride was observed. Our preliminary data confirm that chest MRI could be a useful tool to assess disease progression in CF patients on modulatory drug therapy. MDPI 2022-07-22 /pmc/articles/PMC9331995/ /pubmed/35893365 http://dx.doi.org/10.3390/jcm11154277 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Communication
Macconi, Letizia
Galici, Valeria
Di Maurizio, Marco
Rossi, Enrica
Taccetti, Giovanni
Terlizzi, Vito
Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease
title Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease
title_full Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease
title_fullStr Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease
title_full_unstemmed Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease
title_short Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease
title_sort early effects of elexacaftor-tezacaftor-ivacaftor therapy on magnetic resonance imaging in patients with cystic fibrosis and advanced lung disease
topic Communication
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9331995/
https://www.ncbi.nlm.nih.gov/pubmed/35893365
http://dx.doi.org/10.3390/jcm11154277
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