Mortality in Prader-Willi syndrome.
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.
Einfeld
Stewart L
SL
University of New South Wales, Sydney, New South Wales. s.einfeld@unsw.edu.au
Kavanagh
Sophie J
SJ
Smith
Arabella
A
Evans
Elizabeth J
EJ
Tonge
Bruce J
BJ
Taffe
John
J
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