Article
Measuring the effect of elexacaftor/tezacaftor/ivacaftor combination therapy on the respiratory pump in people with CF using dynamic chest radiography.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Nov 2022
FitzMaurice Thomas S, McCann Caroline, Nazareth Dilip, Shaw Matthew, McNamara Paul S, Walshaw Martin J
Abstract excerpt
BACKGROUND: The CFTR modulator elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) leads to significant improvement in the symptoms and spirometry of people with cystic fibrosis (pwCF), but little evidence exists to understand its effect on respiratory pump function. Dynamic chest radiography (DCR) is a novel cineradiographic tool that identifies and tracks the chest wall and diaphragm throughout the breathing cycle,...
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