Prader-Willi Syndrome Essay -- essays research papers

Prader-Willi Syndrom Prader-Willi Syndrome is a serious genetic disorder that begins at birth with no known cure ; causing mental retardation,short stature,low muscle tone,incomplete sexual development,and its main charecteristic,the desire to eat everything and anything in sight. Prader-Willi syndrome was first known as Prader-Labhart-Willi Syndrome after three Swiss doctors who first described the disorder in 1956. The doctors described a small group of kids with obesity, short stature and mental deficiency , neonatal hypotonia (floppiness) and a desire to constantly eat because they are always hungry. Many other features of PWS have since been described, but extreme obesity and the health problems associated with being fat are the most prominent features. Individuals with PWS have some but not all of the same features and symptoms. PWS is a birth defect. A defect in the hypothalamus, a region of the brain, is suspected to be the cause.The hypothalamus determines hunger and satiety.They can’t fell satiety,so they always have a urge to eat.Some PWS cases are so out of control thay will eat bottlecaps,glass,pencils,garbage,bugs,dogfood, and anything else they can stuff in their mouths. "The ingenuity and determination of PWS children in surreptitiously obtaining edibles is almost legendary and belies their cognitive defects. Serial weighing may be the only way to discover whether such a child is, in fact, stealing food"(Finey,1983). PWS occurs in about l in 10,000 births. It occurs in both males and females equally and is found in people of all races and all nations.It is one of the ten most common conditions seen in genetics clinics. Young people with PWS resemble each other very much.Most of the time, they look like brother and sister. Most of PWS people have almond shaped eyes, narrow foreheads, downturned mouth, thin upper lip and a small chin. Other common features are : obesity , they may be short; they have small hands and feet; have a skin picking habit, thick and sticky saliiva,incomplete sexual development, a curved spine (scoliosis),and chronic sleepiness. PWS patients also have similar personalities: talkative, friendly,extreme attempts towards getting food,arguementivness,repetitve thoughts and behavior, stubbornness, frequent temper tantrums, and sometimes sudden acts of violence. Most people with PWS have some degree of men... ...th PWS after six months of growth hormone treatment Other significant actions of growth hormone that have been reported is an improvement of muscle mass, muscle strength, energy expenditure, bone mineralization ,sexual development ,and also a decrease in fat mass ,have led to further investigations in people with PWS. Children with PWS have distinct behavioral abnormalities because of all the frustrations associated with the syndrome. These behaviors may begin as early as two years of age. They will get a variety of different eating behaviors like foraging for food, secretly eating large amounts of food, and other attempts to continue eating. Other problems include verbally and physically aggressive behaviors such as lying, stealing, scratching and skin picking. Tantrums and unprovoked outbursts are common among children and youths with PWS. People with mild cases of PWS can do many things their normal peers can do,such as go to school,get jobs,and sometimes even move away from home.However they need a lot of help.Kids going to school would need to be enrolled in special education programs(Otherwise they’d be eating their pencil and paper).They need to be constantly supervised.