[HTML][HTML] Guidance and standard operating procedures for functional exercise testing in cystic fibrosis

ZL Saynor, M Gruet, MA McNarry… - European …, 2023 - Eur Respiratory Soc
Regular exercise testing is recommended for all people with cystic fibrosis (PwCF). A range
of validated tests, which integrate both strength and aerobic function, are available and …

Elexacaftor-Tezacaftor-Ivacaftor: a life-changing triple combination of CFTR modulator drugs for cystic fibrosis

M Bacalhau, M Camargo, GAV Magalhães-Ghiotto… - Pharmaceuticals, 2023 - mdpi.com
Cystic fibrosis (CF) is a potentially fatal monogenic disease that causes a progressive
multisystemic pathology. Over the last decade, the introduction of CF transmembrane …

Effects of elexacaftor/tezacaftor/ivacaftor on cardiorespiratory polygraphy parameters and respiratory muscle strength in cystic fibrosis patients with severe lung …

A Giallongo, GF Parisi, M Papale, S Manti, E Mulé… - Genes, 2023 - mdpi.com
Background: Cystic fibrosis transmembrane conductance regulator (CFTR) modulators
represent targeted therapies directly acting on the CFTR channel. The triple therapy …

[HTML][HTML] Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes

F Lurquin, S Gohy, MP Hermans, V Preumont - Journal of clinical & …, 2023 - Elsevier
Aims Combined CFTR modulator therapies have dramatically altered pulmonary outcomes
in patients with cystic fibrosis (CF). Their impact on glucose metabolism requires further …

[HTML][HTML] The effects of elexacaftor, tezacaftor, and ivacaftor (eti) on blood glucose in patients with cystic fibrosis: a systematic review

M Salazar-Barragan, DR Taub - Cureus, 2023 - ncbi.nlm.nih.gov
Cystic fibrosis (CF) is an autosomal recessive genetic disorder resulting from defects in the
cystic fibrosis transmembrane conductance regulator (CFTR) protein, which in turn results in …

Nasal Epithelium Transcriptomics Predict Clinical Response to Elexacaftor/Tezacaftor/Ivacaftor

M Yue, DJ Weiner, KM Gaietto, FJ Rosser… - American Journal of …, 2024 - atsjournals.org
Elexacaftor/tezacaftor/ivacaftor (ETI) has made a substantial positive impact for people living
with CF (pwCF). However, there can be substantial variability in efficacy, and we lack …

Evolving nutritional needs in cystic fibrosis

T Frantzen, S Barsky, G LaVecchia, M Marowitz… - Life, 2023 - mdpi.com
The course of cystic fibrosis (CF) as a nutritional illness is diverging since the introduction of
highly effective modulator therapy, leading to more heterogeneous phenotypes of the …

Longitudinal evaluation of physical fitness in adults with cystic fibrosis in the era of CFTR-modulating-therapies

M Welsner, W Gruber, C Blosch… - … Quarterly for Exercise …, 2024 - Taylor & Francis
Purpose: Maintaining physical fitness plays an important role in the management of people
with cystic fibrosis (pwCF). Longitudinal data on the course of physical fitness and the …

What the future holds: cystic fibrosis and aging

S Blankenship, AR Landis, E Harrison Williams… - Frontiers in …, 2024 - frontiersin.org
Cystic fibrosis (CF) is one of the most common genetic diseases with around 70,000 affected
patients worldwide. CF is a multisystem disease caused by a mutation in the CF …

Longitudinal changes in habitual physical activity in adult people with cystic fibrosis in the presence or absence of treatment with elexacaftor/tezacaftor/ivacaftor

W Gruber, F Stehling, C Blosch… - Frontiers in Sports and …, 2024 - frontiersin.org
Background Habitual physical activity (PA) and exercise training are accepted as important
aspects of care for people with cystic fibrosis (pwCF) to improve health-related measures of …