Respiratory failure in Pompe disease: Treatment with noninvasive ventilation
- 26 April 2005
- journal article
- clinical trial
- Published by Wolters Kluwer Health in Neurology
- Vol. 64 (8) , 1465-1467
- https://doi.org/10.1212/01.wnl.0000158682.85052.c0
Abstract
In this study, noninvasive ventilation (NIV) was prospectively applied to eight patients (35.8 ± 11.4 years) with late-onset Pompe disease and respiratory failure apparent from severe restrictive lung disease, nocturnal hypoxemia (83 ± 8%), and daytime hypercapnia (66.7 ± 17.9 mm Hg). The impact of NIV on respiratory function was followed for 34 ± 17 months. Despite further decrease of vital capacity and inspiratory muscle strength, NIV normalized oxygen saturation during sleep (96 ± 1%), daytime carbon dioxide tensions (44.1 ± 3.6 mm Hg), and symptoms.Keywords
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