Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease.
Letizia MacconiValeria GaliciMarco Di MaurizioEnrica RossiGiovanni TaccettiVito TerlizziPublished in: Journal of clinical medicine (2022)
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.
Keyphrases
- cystic fibrosis
- magnetic resonance imaging
- contrast enhanced
- diffusion weighted
- end stage renal disease
- ejection fraction
- newly diagnosed
- pseudomonas aeruginosa
- diffusion weighted imaging
- chronic kidney disease
- computed tomography
- prognostic factors
- peritoneal dialysis
- bone marrow
- machine learning
- big data
- electronic health record
- deep learning
- artificial intelligence
- data analysis