Increase of liver stiffness and altered bile acid metabolism after triple CFTR modulator initiation in children and young adults with cystic fibrosis
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DOI: 10.1111/liv.15544
Abstract: Novel cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies (elexacaftor/tezacaftor/ivacaftor—ETI) promise clinically significant and sustained improvements for patients with cystic fibrosis (CF). In this study, we investigated the impact of ETI therapy on liver stiffness… read more here.
Keywords: acid metabolism; liver stiffness; cftr modulator; fibrosis ... See more keywords
S16 The influence of the CFTR modulator ivacaftor on aspergillosis in cystic fibrosis
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DOI: 10.1136/thorax-2019-btsabstracts2019.22
Abstract: Introduction Cystic fibrosis (CF) is a life limiting genetic condition which occurs due to mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). Absence of functional CFTR protein leads to progressive respiratory disease characterized… read more here.
Keywords: modulator ivacaftor; cystic fibrosis; cftr modulator;
Novel CFTR modulator combinations maximise rescue of G85E and N1303K in rectal organoids
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DOI: 10.1183/23120541.00716-2021
Abstract: Introduction Cystic fibrosis (CF) is a severe monogenic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Several types of CFTR modulators (correctors/potentiators) have been developed to overcome protein dysfunction associated… read more here.
Keywords: elexacaftor; rectal organoids; rescue; g85e n1303k ... See more keywords
CFTR modulator use and risk of nontuberculous mycobacteria positivity in cystic fibrosis, 2011–2018
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DOI: 10.1183/23120541.00724-2021
Abstract: Background People with cystic fibrosis are at increased risk of pulmonary nontuberculous mycobacteria (NTM) disease. Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are associated with reduced lung infection with pathogens like Pseudomonas aeruginosa and Staphylococcus… read more here.
Keywords: fibrosis; cftr modulator; cystic fibrosis; positivity ... See more keywords
Elexacaftor-Tezacaftor-Ivacaftor: A Life-Changing Triple Combination of CFTR Modulator Drugs for Cystic Fibrosis
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DOI: 10.3390/ph16030410
Abstract: Cystic fibrosis (CF) is a potentially fatal monogenic disease that causes a progressive multisystemic pathology. Over the last decade, the introduction of CF transmembrane conductance regulator (CFTR) modulator drugs into clinical practice has profoundly modified… read more here.
Keywords: modulator drugs; cftr; ivacaftor; cftr modulator ... See more keywords