ERIC Number: EJ723019
Record Type: Journal
Publication Date: 2006-Jan
Reference Count: 45
A Long-Term Population-Based Clinical and Morbidity Review of Prader-Willi Syndrome in Western Australia
Thomson, A. K.; Glasson, E. J.; Bittles, A. H.
Journal of Intellectual Disability Research, v50 n1 p69-78 Jan 2006
Background: An investigation of the clinical morbidity and genetic profiles of individuals with Prader?Willi syndrome (PWS) in Western Australia (WA) was undertaken as part of a wider study into the effects of intellectual disability (ID) on the life course of individuals. Methods: All persons with a diagnosis of PWS were identified from the records of the Disability Services Commission of WA (DSC). The DSC client files formed the main data source, and were supplemented by information from other state health data sets. The analysis was retrospective and quantitative in nature. Results: A total of 56 individuals were identified, 10 of whom exhibited normal methylation patterns and so were analysed separately (PWS-like). The ages of the PWS group ranged from 0.9 to 48.3 years, with six persons deceased. Most people with PWS (76%) had mild or moderate ID, and 70% lived in their family home. The birth prevalence of the disorder was 1 in 29 500 births. Respiratory disorders, dentistry and gastrointestinal disorders were common reasons for hospital admission, with epilepsy or convulsions also reported at moderate frequency. The PWS-like group shared many clinical features in common with PWS patients, the principal exceptions being hypotonia and feeding difficulties in infancy. Conclusions: The estimated birth prevalence of PWS was lower than expected; however, the case ascertainment method may have excluded some individuals. Older people with PWS were generally living in sheltered accommodation. As the cohort ages, demand for places in similar accommodation will increase, adding to the existing burden on service providers. Substantial future increases in the use of medical services and hospital-based care also are predicted with the onset of age-associated disorders.
Descriptors: Congenital Impairments, Mental Retardation, Disease Incidence, Patients, Medical Services, Residential Care, Foreign Countries
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Publication Type: Journal Articles; Reports - Research
Education Level: N/A
Authoring Institution: N/A
Identifiers - Location: Australia