Function, structure and quality of striated muscles in the lower extremities in patients with late onset Pompe Disease-an MRI study.

Clinical tests Enzyme replacement therapy Follow-up Late Onset Pompe Disease Muscle quality Quantitative magnetic resonance imaging (mri)

Journal

PeerJ
ISSN: 2167-8359
Titre abrégé: PeerJ
Pays: United States
ID NLM: 101603425

Informations de publication

Date de publication:
2021
Historique:
received: 07 09 2020
accepted: 20 01 2021
entrez: 17 5 2021
pubmed: 18 5 2021
medline: 18 5 2021
Statut: epublish

Résumé

Pompe Disease (PD) is a rare inherited metabolic myopathy, caused by lysosomal- The present study evaluates muscle quality in 10 patients with LOPD receiving treatment with enzyme replacement therapy and in 10 age and gender matched healthy controls applying T Fat fraction of knee flexors (0.15 vs 0.07,

Sections du résumé

BACKGROUND BACKGROUND
Pompe Disease (PD) is a rare inherited metabolic myopathy, caused by lysosomal-
METHODS METHODS
The present study evaluates muscle quality in 10 patients with LOPD receiving treatment with enzyme replacement therapy and in 10 age and gender matched healthy controls applying T
RESULTS RESULTS
Fat fraction of knee flexors (0.15 vs 0.07,

Identifiants

pubmed: 33996274
doi: 10.7717/peerj.10928
pii: 10928
pmc: PMC8106912
doi:

Types de publication

Journal Article

Langues

eng

Pagination

e10928

Informations de copyright

©2021 Vaeggemose et al.

Déclaration de conflit d'intérêts

Henning Andersen has received research, travel support and speaker honoraria from Octapharma, CSL Behring, Novo, Alexion and Genzyme/Sanofi, and has also served as consultant on advisory board of NMDPharma within the last 5 years. John Vissing has received research and travel support and speaker honoraria from Genzyme/Sanofi, Ultragenyx Pharmaceuticals and aTyr Pharmaceutical, and served as consultant on advisory boards of Genzyme/Sanofi, aTyr pharmaceuticals, Ultragenyx Pharmaceuticals, Sarepta, NOVO Nordisk, Alexion Pharmaceuticals and Stealth Bio Therapeutics within the last 5 years. Julie Schjødtz Hansen reports having received research support, honoraria, and travel funding from Sanofi Genzyme.

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Auteurs

Michael Vaeggemose (M)

Department of Neurology, Aarhus University Hospital, Aarhus N, Denmark.

Rosa Andersen Mencagli (RA)

Department of Neurology, Aarhus University Hospital, Aarhus N, Denmark.

Julie Schjødtz Hansen (JS)

Department of Neurology, Aarhus University Hospital, Aarhus N, Denmark.

Bianca Dräger (B)

Department of Sleep Medicine and Neuromuscular Disorders, University Hospital Muenster, Münster, Germany.

Steffen Ringgaard (S)

MR Research Centre, Aarhus University, Aarhus N, Denmark.

John Vissing (J)

Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.

Henning Andersen (H)

Department of Neurology, Aarhus University Hospital, Aarhus N, Denmark.

Classifications MeSH