Prader-Willi

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 * Prader-Willi Syndrome **


 * __Definition:__** An extremely rare disease present from birth that involves obesity, decreased mucsle tone, decreased mental capacity, and sex glands that produce little or no hormones.

Prader-Willi syndrome is caused by a missing gene on chromosome 15. Most people who have this disease are either missing the genetic material on part of the chromosome that was passed down by the father, or they have two copies of chromosome 15 that was passed down by the mother. This sex-linked, genetic disorder occurs randomly. The patients do not usually have family history of the condition.




 * A KARYOTYPE AND PEDIGREE OF PRADER-WILLI SYNDROME IS SHOWN ABOVE*


 * This disease is most common in male children, however, it does occur in females. It is rare for African Americans to have Prader-Willi disease.

~ Almond-shaped eyes ~ Food cravings ~ Rapid weight gain ~ Slow mental development ~ Abnormally small feet and hands in comparison to the body size ~ Areas of skin the look like stripes, bands, or lines ~ Decreased muscle tone. Because of the weakness in the baby's muscles, they are not able to drink from a bottle, and will need specific treatment that suits the needs of the baby. The most common form of treatment is feeding tubes
 * __Symptoms May Include:__**

One of the most unfortunate symptoms is the extreme craving for food. This craving developes between ages one to six. The child will never feel full after eating, which leads to morbid obesity. The affected patient, usually a child, will do almost anything to fufil their craving and eat their food uncontrolably.


 * For children suffering from Prader-Willi syndrome, there is a slightly modified food pyramid.
 * To learn more about the modified diet, you can go to: []

As your child gets older, there are certain signs that are related to the chance of your child being morbidly obese.

~ Excessive carbon dioxide levels ~ Lack of oxygen supply ~ An abnormal tolerance to glucose
 * __These signs may include:__**


 * Other possible complications can be diabetes, heart failure (most common on the right side), and severe orthopedic problems.

To control one of the greatest health problems, obesity, the easiest most effective thing to do would be to limit the patient's calorie intake and increase excercise. To insure that these changes take effect properly, the family of the patient, as well as the school, neighbors, and other institutions, should attempt to restrict the patient's attempt to obtain food, and may have to lock the kitchen cabinets. Hormone problems can also be corrected with a hormone replacement at the patient's stage of puberty.
 * __Treatment:__**

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 * __Sources:

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