Comprehensive approach to weaning in difficult-to-wean infantile and juvenile-onset glycogen-storage disease type II patients: a case series

Comprehensive approach to weaning in difficult-to-wean infantile and juvenile-onset glycogen-storage disease type II patients: a case series

Abstract Background Glycogen storage disease type II (GSD II) is caused by acid alpha-glucosidase (GAA) deficiency. Both infantile-onset and juvenile-onset GSD II lead to proximal muscle weakness and respiratory insufficiency and require mechanical ventilation. However, GSD II is also independently...

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Bibliographic Details
Main Authors: Lingling Xu, Hongjun Ba, Yuxin Pei, Xueqiong Huang, Yujian Liang, Lidan Zhang, Huimin Huang, Cheng Zhang, Wen Tang
Format: Article
Language:English
Published: BMC 2019-08-01
Series:Italian Journal of Pediatrics
Subjects:
Enzyme replacement therapy
Glycogen storage disease type II
Respiratory failure
Sequential invasive-noninvasive ventilation
Weaning
Online Access:http://link.springer.com/article/10.1186/s13052-019-0692-0

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http://link.springer.com/article/10.1186/s13052-019-0692-0

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