title: Mortality in Prader-Willi syndrome.
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.
authors.affiliation: University of New South Wales, Sydney, New South Wales. s.einfeld@unsw.edu.au
authors.forename: Stewart L
authors.initials: SL
authors.lastname: Einfeld
authors.forename: Sophie J
authors.initials: SJ
authors.lastname: Kavanagh
authors.forename: Arabella
authors.initials: A
authors.lastname: Smith
authors.forename: Elizabeth J
authors.initials: EJ
authors.lastname: Evans
authors.forename: Bruce J
authors.initials: BJ
authors.lastname: Tonge
authors.forename: John
authors.initials: J
authors.lastname: Taffe
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000453:epidemiology; D008297:Male; D011218:Prader-Willi Syndrome / Q000401:mortality*; D015996:Survival Rate