Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles.

doi:10.1164/rccm.202201-0219OC

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Article

Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles.

American journal of respiratory and critical care medicine - 1 Aug 2022

Graeber Simon Y, Renz Diane M, Stahl Mirjam, Pallenberg Sophia T, Sommerburg Olaf, Naehrlich Lutz, Berges Julian, Dohna Martha, Ringshausen Felix C, Doellinger Felix, Vitzthum Constanze, Röhmel Jobst, Allomba Christine, Hämmerling Susanne, Barth Sandra, Rückes-Nilges Claudia, Wielpütz Mark O, Hansen Gesine, Vogel-Claussen Jens, Tümmler Burkhard, Mall Marcus A, Dittrich Anna-Maria

Abstract excerpt

Rationale: We recently demonstrated that triple-combination CFTR (cystic fibrosis transmembrane conductance regulator) modulator therapy with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) improves CFTR function in airway and intestinal epithelia to 40-50% of normal in patients with cystic fibrosis (CF) with one or two F508del alleles. In previous studies, this improvement of CFTR function was shown to improve...

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PMID: 35536314
DOI: 10.1164/rccm.202201-0219OC

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