Effect of CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor on pulmonary ventilation derived by 3D phase-resolved functional lung MRI in cystic fibrosis patients.
Filip KlimešAndreas VoskrebenzevMarcel GutberletMilan SpethRobert GrimmMartha DohnaGesine HansenFrank WackerDiane Miriam RenzAnna-Maria DittrichJens Vogel-ClaussenPublished in: European radiology (2023)
%pred, and global MRI score suggest that 3D PREFUL MRI is sensitive to measure improved regional ventilation of the lung parenchyma due to reduced inflammation induced by ETI therapy in CF patients. • 3D PREFUL MRI-derived improved ventilation volume (IVV) correlated with MRI mucus plugging score changes suggesting that reduced endobronchial mucus is predominantly responsible for regional ventilation improvement 8-16 weeks after ETI therapy.
Keyphrases
- cystic fibrosis
- contrast enhanced
- magnetic resonance imaging
- end stage renal disease
- newly diagnosed
- diffusion weighted imaging
- ejection fraction
- pseudomonas aeruginosa
- respiratory failure
- peritoneal dialysis
- computed tomography
- mechanical ventilation
- lung function
- mesenchymal stem cells
- patient reported outcomes
- extracorporeal membrane oxygenation
- replacement therapy