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 Hagemans, M. L.C. Articles by Van der Ploeg, A. T. Search for Related Content PubMed PubMed Citation Articles by Hagemans, M. L.C. Articles by Van der Ploeg, A. T. Related Collections Quality of life Glycogenoses Muscle disease

Late-onset Pompe disease primarily affects quality of life in physical health domains

From the Department of Pediatrics (M.L.C. Hagemans, and Drs. Winkel and Van der Ploeg), Division of Metabolic Diseases and Genetics, Erasmus MC-Sophia, Rotterdam; and Departments of Public Health (Dr. Janssens), Neurology (K.A. Sieradzan and Dr. Van Doorn), and Clinical Genetics (Dr. Reuser), Erasmus MC Rotterdam, The Netherlands.

Address correspondence and reprint requests to Dr. A.T. Van der Ploeg, Department of Pediatrics, Division of Metabolic Diseases and Genetics, Erasmus MC-Sophia, Dr. Molewaterplein 60, 3015 GJ Rotterdam, the Netherlands; e-mail: a.vanderploeg{at}erasmusmc.nl

Objective: To investigate quality of life in an international population of patients with late-onset Pompe disease.

Methods: Data on quality of life (SF-36), age, sex, disease duration, wheelchair use, and use of artificial ventilation were collected for 210 adults with Pompe disease from Australia, Germany, the Netherlands, the United Kingdom, and the United States. SF-36 scores were compared between countries and related to patient characteristics. In addition, for the Dutch subgroup (n = 51), comparisons with the general population and 1-year follow-up assessments were performed.

Results: No significant differences between countries were found for the four physical health scales. Mean scores on the vitality, role functioning-emotional, and mental health scale differed between countries, but these differences were not consistent. Wheelchair use was associated with lower physical and social functioning scores (B = –23.6 and –15.1, p < 0.001), and the use of artificial ventilation with lower physical functioning scores (B = –8.4, p = 0.004). Patients reported significantly poorer quality of life than the general population on the physical functioning, role functioning-physical, general health, vitality, and social functioning scales. No significant differences in SF-36 scores were found between the baseline and 1-year follow-up measurement.

Conclusions: Patients with late-onset Pompe disease are, on average, markedly affected on the physical health domains of quality of life, but score only slightly lower than the general population on the mental health domains.

Received March 23, 2004. Accepted in final form July 2, 2004.

This article has been cited by other articles:

				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]

				M.L.C. Hagemans, L. P.F. Winkel, W. C.J. Hop, A. J.J. Reuser, P. A. Van Doorn, and A. T. Van der Ploeg Disease severity in children and adults with Pompe disease related to age and disease duration Neurology, June 28, 2005; 64(12): 2139 - 2141. [Abstract] [Full Text] [PDF]