Respiratory muscle training with enzyme replacement therapy improves muscle strength in late - onset Pompe disease

Respiratory muscle training with enzyme replacement therapy improves muscle strength in late - onset Pompe disease

Background: Pompe disease is an autosomal recessive metabolic disorder caused by the deficiency of the lysosomal enzyme acid α-glucosidase. This deficiency leads to glycogen accumulation in the lysosomes of muscle tissue causing progressive muscular weakness particularly of the respiratory system. E...

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Bibliographic Details
Main Authors: Jevnikar Mitja, Kodric Metka, Cantarutti Fabiana, Rossella Cifaldi, Cinzia Longo, Della Porta Rossana, Bembi Bruno, Confalonieri Marco
Format: Article
Language:English
Published: Elsevier 2015-12-01
Series:Molecular Genetics and Metabolism Reports
Subjects:
Pompe disease
Late-onset type II glycogenosis
Respiratory muscles
Muscle training
Enzyme replacement therapy
Online Access:http://www.sciencedirect.com/science/article/pii/S2214426915300355

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http://www.sciencedirect.com/science/article/pii/S2214426915300355

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