[HTML][HTML] Peripheral muscle abnormalities in cystic fibrosis: Etiology, clinical implications and response to therapeutic interventions

M Gruet, T Troosters, S Verges - Journal of Cystic Fibrosis, 2017 - Elsevier
Peripheral muscle dysfunction is an important systemic consequence of cystic fibrosis (CF)
with major clinical implications, such as exercise intolerance and reduced quality of life …

[HTML][HTML] Exercise intolerance in cystic fibrosis-the role of CFTR modulator therapies

JE Caterini, F Ratjen, AR Barker, CA Williams… - Journal of Cystic …, 2022 - Elsevier
Exercise intolerance is common in people with CF (pwCF), but not universal among all
individuals. While associated with disease prognosis, exercise intolerance is not simply a …

A comparison of respiratory and peripheral muscle strength, functional exercise capacity, activities of daily living and physical fitness in patients with cystic fibrosis and …

H Arikan, İ Yatar, E Calik-Kutukcu, Z Aribas… - Research in …, 2015 - Elsevier
There are limited reports that compare muscle strength, functional exercise capacity,
activities of daily living (ADL) and parameters of physical fitness of cystic fibrosis (CF) …

Improvement in exercise duration, lung function and well-being in G551D-cystic fibrosis patients: a double-blind, placebo-controlled, randomized, cross-over study …

D Edgeworth, D Keating, M Ellis, B Button… - Clinical …, 2017 - portlandpress.com
G551D, a mutation of the cystic fibrosis transmembrane conductance regulator (CFTR)
gene, results in impaired chloride channel function in cystic fibrosis (CF) with multiple end …

[HTML][HTML] Effects of inspiratory muscle training on postural stability, pulmonary function and functional capacity in children with cystic fibrosis: A randomised controlled …

M Zeren, E Cakir, HN Gurses - Respiratory medicine, 2019 - Elsevier
Background Previous research has found conflictive results regarding the benefits of
inspiratory muscle training (IMT) for cystic fibrosis (CF) patients. Also, involvement of …

CFTR Genotype and Maximal Exercise Capacity in Cystic Fibrosis. A Cross-Sectional Study

T Radtke, H Hebestreit, S Gallati… - Annals of the …, 2018 - atsjournals.org
Rationale: Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in
human skeletal muscle cells. Variations of CFTR dysfunction among patients with cystic …

Evidence of microvascular dysfunction in patients with cystic fibrosis

P Rodriguez-Miguelez, J Thomas… - American Journal …, 2016 - journals.physiology.org
Cystic fibrosis (CF) is a genetic, multisystemic disorder with broad clinical manifestations
apart from the well-characterized pulmonary dysfunction. Recent findings have described …

Exercise physiology across the lifespan in cystic fibrosis

RJ Shei, KA Mackintosh, JE Peabody Lever… - Frontiers in …, 2019 - frontiersin.org
Cystic fibrosis (CF), a severe life-limiting disease, is associated with multi-organ pathologies
that contribute to a reduced exercise capacity. At present, the impact of, and interaction …

Prediction of peak oxygen uptake using the modified shuttle test in children and adolescents with cystic fibrosis

FM Vendrusculo, JP Heinzmann‐Filho… - Pediatric …, 2019 - Wiley Online Library
Background Several tests may be used to assess exercise intolerance in cystic fibrosis (CF),
including the gold standard cardiopulmonary exercise test (CPET) and the Modified Shuttle …

Preterm birth and exercise capacity: what do we currently know?

CA O'Dea, ML Beaven, AC Wilson, EF Smith… - Frontiers in …, 2023 - frontiersin.org
Objectives The long-term cardiopulmonary outcomes following preterm birth during the
surfactant era remain unclear. Respiratory symptoms, particularly exertional symptoms, are …