Pain interference and fatigue in limb-girdle muscular dystrophy R9.
10-meter walk/run
6-minute walk test
FKRP
Motor function
PROMIS short form
Pulmonary function
Journal
Neuromuscular disorders : NMD
ISSN: 1873-2364
Titre abrégé: Neuromuscul Disord
Pays: England
ID NLM: 9111470
Informations de publication
Date de publication:
06 2023
06 2023
Historique:
received:
04
03
2023
revised:
15
05
2023
accepted:
18
05
2023
pmc-release:
01
06
2024
medline:
31
7
2023
pubmed:
30
5
2023
entrez:
29
5
2023
Statut:
ppublish
Résumé
Pain is prevalent in individuals with limb-girdle muscular dystrophy (LGMD) R9, but impact on daily living and correlation with fatigue remain unknown. Patient-Reported Outcomes Measurement Information System (PROMIS) pain interference and fatigue short forms were completed annually by 23 children and 54 adults with biallelic fukutin-related protein (FKRP) variants for up to six years. Concurrent motor and pulmonary function were evaluated. Pain interference T-scores were near the normal mean of 50 by linear mixed model analysis (48.5 in children, 51.6 in adults). 58% of participants experienced pain interference levels greater than the general population on at least one assessment. Fatigue T-scores were elevated in adults but not children (49.0 in children, 55.1 in adults), and 75% had at least one elevated fatigue score. Of participants with at least two visits, serial scores were not consistent across visits, without a clear pattern. Pain interference and fatigue were positively correlated (r = 0.55). Both increased with older age (r = 0.21 and 0.41 respectively). Neither differed by sex or ambulation status. Motor (r=-0.32) and pulmonary (r=-0.25) function correlated with fatigue in adults, not children. Results suggest that pain in those with LGMDR9 is variable and episodic, limiting impact on daily life, while fatigue increases over time.
Identifiants
pubmed: 37247532
pii: S0960-8966(23)00142-6
doi: 10.1016/j.nmd.2023.05.005
pmc: PMC10527028
mid: NIHMS1906392
pii:
doi:
Substances chimiques
FKRP protein, human
EC 2.4.2.-
Pentosyltransferases
EC 2.4.2.-
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
523-530Subventions
Organisme : NIAMS NIH HHS
ID : UH3 AR076387
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR002537
Pays : United States
Organisme : NIAMS NIH HHS
ID : UG3 AR076387
Pays : United States
Organisme : NINDS NIH HHS
ID : U54 NS053672
Pays : United States
Organisme : NINR NIH HHS
ID : R01 NR017610
Pays : United States
Organisme : NINDS NIH HHS
ID : P50 NS053672
Pays : United States
Organisme : NIAMS NIH HHS
ID : R01 AR077418
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL153095
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA193249
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA246540
Pays : United States
Informations de copyright
Copyright © 2023. Published by Elsevier B.V.
Déclaration de conflit d'intérêts
Declaration of Competing Interest None.
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