Article
Effect of elexacaftor/tezacaftor/ivacaftor on mucus and mucociliary clearance in cystic fibrosis.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jan 2024
Donaldson Scott H, Corcoran Timothy E, Pilewski Joseph M, Mogayzel Peter, Laube Beth L, Boitet Evan R, Harris Elex S, Ceppe Agathe, Edwards Lloyd J, Zeman Kirby, Wu Jihong, Esther Charles R, Nichols David P, Bennett William D, Rowe Steven M
Abstract excerpt
BACKGROUND: The cystic fibrosis transmembrane conductance regulator (CFTR) modulator elexacaftor/tezacaftor/ivacaftor (E/T/I) is highly effective clinically for those with at least one F508del-CFTR allele. The effects of E/T/I on mucociliary clearance (MCC) and sputum properties are unknown. We, therefore, sought to characterize the effects of E/T/I on in vivo MCC and sputum characteristics hypothesized to impact...
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