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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...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2023
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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 |