Practical Recommendations for Diagnosis and Management of Respiratory Muscle Weakness in Late-Onset Pompe Disease

Practical Recommendations for Diagnosis and Management of Respiratory Muscle Weakness in Late-Onset Pompe Disease

Pompe disease is an autosomal-recessive lysosomal storage disorder characterized by progressive myopathy with proximal muscle weakness, respiratory muscle dysfunction, and cardiomyopathy (in infants only). In patients with juvenile or adult disease onset, respiratory muscle weakness may decline more...

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Bibliographic Details
Main Authors: Matthias Boentert, Hélène Prigent, Katalin Várdi, Harrison N. Jones, Uwe Mellies, Anita K. Simonds, Stephan Wenninger, Emilia Barrot Cortés, Marco Confalonieri
Format: Article
Language:English
Published: MDPI AG 2016-10-01
Series:International Journal of Molecular Sciences
Subjects:
neuromuscular disorders
Pompe disease
respiratory muscle weakness
mechanical ventilation
cough assistance
Online Access:http://www.mdpi.com/1422-0067/17/10/1735

Internet

http://www.mdpi.com/1422-0067/17/10/1735

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