A new study of Prader-Willi Syndrome may cast some light on a baffling and disabling disorder that affects one in 10,000 people.
- Prader-Willi Syndrome is a genetic disorder in which people have four to five times the amount of ghrelin, a hormone produced by the stomach that helps regulate the appetite.
- Those who have the syndrome are born hungry and remain that way 24 hours a day. Parents say they often have to keep food under lock and key.
- There is no cure for Prader-Willi Syndrome, and many people with the disorder can never function independently as adults.
"It is really a biological drive that they have and it reminds us that the regulation of body weight is very complex," said Dr. Andrea Haqq, a professor of pediatrics at the University of Alberta in Canada, and the lead author of the new study. "A lot of people with Prader-Willi will end up living in group homes that are tailored to constant supervision around food. There has to be very strict control of their food environment both at home and at school."
- Dr. Haqq has recruited eight children with Prader-Willi Syndrome to take part in her study of the syndrome, but she hopes to recruit 50 more.
- Her research will look at links between obesity and the syndrome by comparing children in the study with obese children who do not have Prader-Willi.
- She will also research whether levels of ghrelin can be manipulated by controlling protein in the children's diets, and investigate proteins in the brain that are connected with the syndrome.
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