HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Figures Only Full Text Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mellies, U. Articles by Voit, T. Search for Related Content PubMed PubMed Citation Articles by Mellies, U. Articles by Voit, T.

Respiratory failure in Pompe disease: Treatment with noninvasive ventilation

From the Department of Pediatrics and Pediatric Neurology (Drs. Mellies, Stehling, Dohna-Schwake, and Voit), University of Essen, and Department of Pneumology and Sleep Medicine (Drs. Ragette and Teschler), Ruhrlandklinik, Essen, Germany.

Address correspondence and reprint requests to Dr. U. Mellies, Department of Pediatrics and Pediatric Neurology, University of Essen, Hufelandstr. 55, D-45122 Essen, Germany; e-mail: uwe.mellies{at}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.

Supported by grants from the University of Essen (grant 107505-0/IFORES) and the Alfried Krupp von Bohlen and Halbach Foundation.

Received July 27, 2004. Accepted in final form December 23, 2004.

This article has been cited by other articles:

				B. Bembi, E. Cerini, C. Danesino, M. A. Donati, S. Gasperini, L. Morandi, O. Musumeci, G. Parenti, S. Ravaglia, F. Seidita, et al. Management and treatment of glycogenosis type II Neurology, December 2, 2008; 71(23_suppl_2): S12 - S36. [Abstract] [Full Text] [PDF]

				A. T. van der Ploeg Monitoring of pulmonary function in Pompe disease: a muscle disease with new therapeutic perspectives Eur. Respir. J., December 1, 2005; 26(6): 984 - 985. [Full Text] [PDF]