- •The chest MRI global score showed a long-term stability in patients under standard therapy without elexacaftor/tezacaftor/ivacaftor.
- •The chest MRI global score improved in patients under Elexacaftor/Tezacaftor/Ivacaftor, mainly due to reduction of bronchiectasis/wall thickening and mucus plugging.
- •The improved chest MRI global score correlated with proportional improvements in FEV1%.
- •The chronic rhinosinusitis MRI sum score improved under elexacaftor/tezacaftor/ivacaftor therapy through a reduction of mucopyoceles.
Abbreviations:CF (cystic fibrosis), CFTRm (cystic fibrosis transmembrane conductance regulator modulator), CT (Computed tomography), CRS (chronic rhinosinusitis), ETI (Elexacaftor/Tezacaftor/Ivacaftor), FEV1% (Forced expiratory volume in 1 s percent predicted), MRI (magnetic resonance imaging)
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
- Pathophysiology and management of pulmonary infections in cystic fibrosis.Am J Respir Crit Care Med. 2003; 168: 918-951
- Cystic Fibrosis.(editors)in: Valle DL Antonarakis S Ballabio A Beaudet AL Mitchell GA The online metabolic and molecular bases of inherited disease. McGraw-Hill Education, New York, NY2019
- Concordant genotype of upper and lower airways P aeruginosa and S aureus isolates in cystic fibrosis.Thorax. 2009; 64: 535-540
- Importance of bacteriology in upper airways of patients with Cystic Fibrosis.J Cyst Fibros. 2013; 12: 525-529
- Progress in therapies for cystic fibrosis.Lancet Respir Med. 2016; 4: 662-674
- Cystic fibrosis: emergence of highly effective targeted therapeutics and potential clinical implications.Am J Respir Crit Care Med. 2020; 201: 1193-1208
- Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial.Lancet. 2019; 394: 1940-1948
- Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single Phe508del allele.N Engl J Med. 2019; 381: 1809-1819
- Clinical effectiveness of elexacaftor/tezacftor/ivacaftor in people with cystic fibrosis.Am J Respir Crit Care Med. 2021;
- Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis.J Cyst Fibros. 2021; 20: 460-463
- Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease.Am J Respir Crit Care Med. 2014; 189: 956-965
- Imaging of cystic fibrosis lung disease and clinical interpretation.Röfo. 2016; 188: 834-845
- Comparison of lung clearance index and magnetic resonance imaging for assessment of lung disease in children with cystic fibrosis.Am J Respir Crit Care Med. 2017; 195: 349-359
- Midterm reproducibility of chest magnetic resonance imaging in adults with clinically stable cystic fibrosis and chronic obstructive pulmonary disease.Am J Respir Crit Care Med. 2019; 200: 103-107
- Preventive inhalation of hypertonic saline in infants with cystic fibrosis (PRESIS). A randomized, double-blind, controlled study.Am J Respir Crit Care Med. 2019; 199: 1238-1248
- Magnetic resonance imaging detects progression of lung disease and impact of newborn screening in preschool children with cystic fibrosis.Am J Respir Crit Care Med. 2021;
- Effects of lumacaftor-ivacaftor on lung clearance index, magnetic resonance imaging, and airway microbiome in phe508del homozygous patients with cystic fibrosis.Ann Am Thorac Soc. 2021; 18: 971-980
- Long-term outcomes in real life of lumacaftor-ivacaftor treatment in adolescents with cystic fibrosis.Front Pediatr. 2021; 9744705
- Impact of cystic fibrosis transmembrane conductance regulator therapy on chronic rhinosinusitis and health status: deep learning CT analysis and patient-reported outcomes.Ann Am Thorac Soc. 2022; 19: 12-19
- Magnetic resonance imaging detects chronic rhinosinusitis in infants and preschool children with cystic fibrosis.Ann Am Thorac Soc. 2020; 17: 714-723
- The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel.J Pediatr. 1998; 132: 589-595
- Lung volumes and forced ventilatory flows. Report working party standardization of lung function tests, European community for steel and coal. Official statement of the european respiratory society.Eur Respir J Suppl. 1993; 16: 5-40
- Standardisation of spirometry.Eur Respir J. 2005; 26: 319-338
- Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial.JAMA. 2012; 307: 2269-2277
- Morphologic and functional scoring of cystic fibrosis lung disease using MRI.Eur J Radiol. 2012; 81: 1321-1329
- Echo time-dependence of observed lung t1 in patients with cystic fibrosis and correlation with clinical metrics.J Magn Reson Imaging. 2020; 52: 1645-1654
- Increased inflammatory markers detected in nasal lavage correlate with paranasal sinus abnormalities at mri in adolescent patients with cystic fibrosis.Antioxidants (Basel). 2021; : 10
- Rapid improvement after starting elexacaftor-tezacaftor-ivacaftor in patients with cystic fibrosis and advanced pulmonary disease.Am J Respir Crit Care Med. 2021; 204: 64-73
- Effects of elexacaftor/tezacaftor/ivacaftor therapy on CFTR function in patients with cystic fibrosis and one or two F508del alleles.Am J Respir Crit Care Med. 2021;
- Assessment of lung disease in children with cystic fibrosis using hyperpolarized 3-Helium MRI: comparison with Shwachman score, Chrispin-Norman score and spirometry.Eur Radiol. 2007; 17: 1018-1024
- Proposal of a CT scoring system of the paranasal sinuses in diagnosing cystic fibrosis.Eur Radiol. 2003; 13: 1451-1460