Exercise intolerance in cystic fibrosis-the role of CFTR modulator therapies.
Cystic fibrosis
Exercise intolerance
Phosphorus magnetic resonance spectroscopy CFTR modulators
Journal
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
ISSN: 1873-5010
Titre abrégé: J Cyst Fibros
Pays: Netherlands
ID NLM: 101128966
Informations de publication
Date de publication:
03 2022
03 2022
Historique:
received:
01
07
2021
revised:
18
10
2021
accepted:
20
11
2021
pubmed:
28
12
2021
medline:
14
4
2022
entrez:
27
12
2021
Statut:
ppublish
Résumé
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 reflection of the degree of lung disease. In people with severe CF, respiratory limitations may contribute more significantly to impaired exercise capacity than in those with mild-moderate CF. At all levels of disease severity, there are peripheral factors e.g., abnormal macro- and micro-vascular function that impair blood flow and reduce oxygen extraction, and mitochondrial defects that diminish metabolic efficiency. We discuss advances in understanding the central and peripheral mechanisms underlying exercise intolerance in pwCF. Exploring both the central and peripheral factors that contribute to exercise intolerance in CF can help inform the development of new therapeutic targets, as well as help define prognostic criteria.
Identifiants
pubmed: 34955387
pii: S1569-1993(21)02152-4
doi: 10.1016/j.jcf.2021.11.011
pii:
doi:
Substances chimiques
CFTR protein, human
0
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
282-292Informations de copyright
Copyright © 2021. Published by Elsevier B.V.