Prader-Willi Syndrom
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
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