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Neurology. 2005 Apr 26;64(8):1465-7.

Respiratory failure in Pompe disease: treatment with noninvasive ventilation.

Author information

  • 1Department of Pediatrics and Pediatric Neurology, University of Essen, Essen, Germany. uwe.mellies@uni-essen.de


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.

[PubMed - indexed for MEDLINE]
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