Mortality in Prader-Willi Syndrome
- 1 January 2006
- journal article
- Published by American Association on Intellectual and Developmental Disabilities (AAIDD) in American Journal on Mental Retardation
- Vol. 111 (3), 193-8
- https://doi.org/10.1352/0895-8017(2006)111[193:MIPS]2.0.CO;2
Abstract
Persons with Prader-Willi syndrome have been known to have a high mortality rate. However, intellectual disability, which usually accompanies Prader-Willi syndrome, is also associated with a higher mortality rate than in the general population. In this study, the death rates in a longitudinal cohort of people with Prader-Willi syndrome are compared with those for an epidemiologically derived control sample of people with intellectual disability from other causes. We found that those with Prader-Willi syndrome had a higher mortality rate than did controls. After the protective effect of mild intellectual disability or average intellectual function was accounted for, the hazard ratio for Prader-Willi syndrome versus controls was 6.07. Obesity and its complications were factors contributing to the mortality identified in this study.Keywords
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