Article
Impact of elexacaftor/tezacaftor/ivacaftor therapy on lung clearance index and magnetic resonance imaging in children with cystic fibrosis and one or two F508del alleles.
The European respiratory journal - 1 Sept 2024
Stahl Mirjam, Dohna Martha, Graeber Simon Y, Sommerburg Olaf, Renz Diane M, Pallenberg Sophia T, Voskrebenzev Andreas, Schütz Katharina, Hansen Gesine, Doellinger Felix, Steinke Eva, Thee Stephanie, Röhmel Jobst, Barth Sandra, Rückes-Nilges Claudia, Berges Julian, Hämmerling Susanne, Wielpütz Mark O, Naehrlich Lutz, Vogel-Claussen Jens, Tümmler Burkhard, Mall Marcus A, Dittrich Anna-Maria
Abstract excerpt
BACKGROUND: We recently demonstrated that elexacaftor/tezacaftor/ivacaftor (ETI) improves the lung clearance index (LCI) and abnormalities in lung morphology detected by magnetic resonance imaging (MRI) in adolescent and adult patients with cystic fibrosis (CF). However, real-world data on the effect of ETI on these sensitive outcomes of lung structure and function in school-age children with CF have not been...
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