Treatment
Behavioral
Prader-Willi Syndrome is also frequently associated with an extreme and
insatiable appetite, often resulting in morbid, and in some cases
life-threatening, obesity. There is currently no consensus as to the cause for
this particular symptom.
Endocrine
There are several aspects of PWS that support the concept of growth hormone
deficiency in individuals with PWS. Specifically, individuals with PWS have
short stature, are obese with abnormal body composition, have reduced fat free
mass (FFM), have reduced LBM and total energy expenditure, and have decreased
bone density.
PWS is characterized by hypogonadism. This is manifested as undescended testes
in males and benign premature adrenarche in females. Testes may descend with
time or can be managed with surgery or testosterone replacement. Adrenarche may
be treated with hormone replacement therapy.
Treatment
There is no cure for Prader-Willi syndrome. However, the physical problems
caused by the syndrome can be managed. During infancy, special feeding
techniques and formulas can help the infant grow. Physical therapy and exercise
helps improve strength and coordination. Administering human growth hormone (Genotropin,
Humatrope, Norditropin) improves muscle mass and growth.
Speech therapy can help children who are behind in their language skills to
catch up to their peers. Special education services and supports help children
with Prader-Willi achieve their full capacity.
As the child grows, eating and weight problems can by controlled through a
balanced, low-calorie diet, weight monitoring, external food restriction, and
daily exercise. During the school years, children benefit from a highly
structured learning environment as well as extra help. Throughout their lives,
the subject's food should literally be kept under lock and key, since the
largest problem associated with the syndrome is severe obesity.
Hypotonia, hypomentia, hypogonadism,obesity syndrome
