Prader-Willi Syndrome, also known as PWS, is an uncommon genetic disorder. It was first described in 1956 by Andrea Prader, Heinrich Willi, Alexis Labhart, Andrew Ziegler, and Guido Fanconi.
People with this disorder have dysfunctional hypothalamus; part of the brain that controls feelings of fullness or hunger. Thus, these people never feel full and have a constant urge to eat, leading to severe stoutness.
PWS, in most cases, result from a spontaneous genetic mutation in genes on chromosome 15 that occurs at conception. In rare cases, the mutation is inherited.
What are the symptoms of Prader-Willi syndrome?
People with this disorder experience symptoms in two stages.
Stage 1 – Infants with PWS can have low muscle tone . Because of this, they need special feeding assistance or may have problems gaining weight. However, tThe situation gets better as they grow older.
Stage 2 – Insatiable appetite. Between the ages of 1 to 6 years, the children with PWS may feel constant hunger which, if not kept under strict control, may lead to life-threatening obesity.
Cure?
Prader–Willi Syndrome has no cure, however, several treatments are in place to lessen the condition’s symptoms. During infancy, subjects should undergo therapies to improve muscle tone. Speech and occupational therapy are also indicated. During the school years, children benefit from a highly structured learning environment as well as extra help. The largest problem associated with the syndrome is severe obesity.
Prescription of daily recombinant growth hormone injections are indicated for children with PWS. GH supports linear growth and increased muscle mass, and may lessen food preoccupation and weight gain.
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PWS