Prader-Willi syndrome is a complex, multidimensional disease affecting approximately 1 in 8000 to 1 in 50 000 individuals. Although there is an abundance of data available regarding Prader-Willi syndrome, there are few sources of compiled, nutritionally pertinent information. Nutrition intervention is currently the only treatment for Prader-Willi syndrome, and therefore it is critical that nutrition professionals have an understanding of the disease. This review provides background information regarding Prader-Willi syndrome and the causes of obesity and hyperphagia, as well as outlines current nutritional recommendations and strategies for working with individuals with Prader-Willi syndrome. It is recommended that the diet of these children be strictly controlled and structure provided, yet it is important to allow for some flexibility to enhance quality of life. Behavioral issues also need to be considered as they render it a challenge for parents to impose restrictions on their children’s eating patterns.
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