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Diaphragm pacing and independent breathing in individuals with severe Pompe disease

INTRODUCTION: Pompe disease is an inherited disease characterized by a deficit in acid-α-glucosidase (GAA), an enzyme which degrades lysosomal glycogen. The phrenic-diaphragm motor system is affected preferentially, and respiratory failure often occurs despite GAA enzyme replacement therapy. We hypo...

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Detalles Bibliográficos
Autores principales: Liberati, Cristina, Byrne, Barry J., Fuller, David D., Croft, Chasen, Pitts, Teresa, Ehrbar, Jessica, Leon-Astudillo, Carmen, Smith, Barbara K.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10423945/
https://www.ncbi.nlm.nih.gov/pubmed/37583873
http://dx.doi.org/10.3389/fresc.2023.1184031