Diaphragm pacing and independent breathing in individuals with severe Pompe disease

Diaphragm pacing and independent breathing in individuals with severe Pompe disease

IntroductionPompe 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 hypoth...

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Bibliographic Details
Main Authors: Cristina Liberati, Barry J. Byrne, David D. Fuller, Chasen Croft, Teresa Pitts, Jessica Ehrbar, Carmen Leon-Astudillo, Barbara K. Smith
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-07-01
Series:Frontiers in Rehabilitation Sciences
Subjects:
Pompe disease
diaphragm
mechanical ventilation
breathing
phrenic stimulation
Online Access:https://www.frontiersin.org/articles/10.3389/fresc.2023.1184031/full

Internet

https://www.frontiersin.org/articles/10.3389/fresc.2023.1184031/full

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