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Prader-Willi Syndrome is
caused by a rare birth defect centered on chromosome 15. Characteristics
of the syndrome include developmental delays and mental retardation,
behavioral problems, and uncontrolled appetite leading to obesity. Affected individuals also experience incomplete sexual development, poor
muscle tone, and short stature as adults. Newborns with PWS have low
birth weight, poor muscle tone, are lethargic, do not feed well and
generally fail to thrive. At about two to four years of age, children
with PWS develop an uncontrollable, insatiable appetite with excessive
or rapid weight gain between the ages of 1-6 without intervention. The
urge to eat is physiological and overwhelming. It is difficult to
control and requires constant vigilance. Motor development is delayed
1-2 years; speech and language problems are common. Mental retardation
is usually present to varying degrees. Children with PWS often develop
behavior problems, ranging from stubbornness to temper tantrums, and are
easily upset by unexpected changes. Other common characteristics include
a high pain threshold, obsessive/compulsive behavior, dental problems
and breathing difficulties. PWS occurs in 1 in 12,000 to 15,000 births
and is regarded as the most common cause of genetic obesity. It affects
both genders and all races. Treatment involves speech and language
therapy and special education. Stringent control of food intake is vital
to prevent obesity related disease and death. Life expectancy may be
normal if weight can be controlled. Individuals with PWS typically do
best in settings that offer a stable routine and restricted access to
food.
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