Bioimpedance Phase Angle as a Prognostic Tool in Late-Onset Pompe Disease: A Single-Centre Prospective Study With a 15-year Follow-Up

Background: Late-onset Pompe disease (LOPD) is an autosomal-recessive metabolic myopathy caused by deficiency of the lysosomal enzyme Acid Alpha—Glucosidase (GAA), leading to glycogen accumulation in proximal and axial muscles, and in the diaphragm. Enzyme Replacement Therapy (ERT) with recombinant...

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Detalles Bibliográficos
Autores principales: Ravaglia, Sabrina, de Giuseppe, Rachele, Carlucci, Annalisa, Jehne, Susan, Crescimanno, Grazia, Ahmad, Lara, Paoletti, Matteo, Clemente, Gabriele, Pichiecchio, Anna, Bazzano, Rosella, Cirio, Serena, Valente, Enza Maria, Danesino, Cesare, De Filippi, Paola, Tartara, Alice, Cena, Hellas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Cell and Developmental Biology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8896115/
https://www.ncbi.nlm.nih.gov/pubmed/35252175
http://dx.doi.org/10.3389/fcell.2022.793566

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