Comparison of Health-Related Quality of Life Between Heterozygous Women with Fabry Disease, the General Population, and Patients with Chronic Disease

Autor: Jansen, Natalie R.
Jazyk: angličtina
Rok vydání: 2005
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Druh dokumentu: Text
Popis: Introduction: Fabry disease is an X-linked lysosomal storage disorder that affects both men and women. Manifestations of the disease have been well-documented in hemizygous males. This study assessed the health-related quality of life (HRQoL) of obligate heterozygotes compared to a healthy control population, and to populations with multiple sclerosis (MS) and rheumatoid arthritis (RA). Methods: Study subjects were identified through lysosomal disease centers and the Fabry Support and Information Group (FSIG). Mean scores in each HRQoL subscales from the RAND-36 were compared between study subjects and previously published data from the Women’s Health Initiative (WHI). Comparisons were also made between studies on MS and RA. Results: Comparisons between the study population (202 women) and WHI indicated that all eight domains of HRQoL were significantly lower for women with Fabry disease with p
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