Prader-Willie's syndrome

 
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PRADERS WILLIE SYNDROME

THE DISORDER OF THE APPETITE

 A brain dysfunction in the level of the hypothalamus is the reason of this disorder in the center of the appetite and satiety, which is typical of PWS.

 To eat compulsively and the obsession with the food normally begins before 6 years old.

 The impulse of eating is physiological and very big, it is very difficult to control and requires constant alertness.

 A balanced diet, low in calories with vitamin supplementation and calcium supplement is advisable. To be weighed regularly and a periodic review of the diet is necessary.

 The education to eat small morsels, morsel by morsel and healthy NON FAT food is the plan to apply routinely and in a consistent form.

 The weight control depends on the restriction of the food out of home or out of alertness and it may be needed to close with key the kitchen and areas of food storage.

 The daily exercise (at least 30 minutes) also is essential for the control of weight and health.

 Up to now no medication or surgical intervention has been defined that could helps to eliminate the need of the strict control of the nourishment and the alertness about the food.

 The treatment with growth hormone, which increases the muscular mass, can allow a level of higher caloric daily ingestion.

BEHAVIOR PROBLEMS

 Infants and young children with PWS are typically satisfied and loving, and exhibit few problems of conduct.

 The majority of older children and adult with PWS, nevertheless, have difficulties with their behavior. The assault of behavioral symptoms normally coincides with the assault of hyperphagia (though not all the conducts of the problem are related to the food), and the difficulties reach the maximum in the adolescence.

 Daily routines and a rigid structure, firm rules and well marked limits, as well as the positive prizes are the best attribute for the direction of the conduct.

PHYSICAL DEVELOPMENT PROBLEMS

 The physical and professional therapies help promoting the development of skills and the appropriate functions.

 Feet may need orthopedic devices.

 The treatment with the growth hormone, increasing the mass of the muscle, can improve the motion skills. Exercise and physical activities are necessary.

 The need must be evaluated for language therapy in the childhood. The products to increase the saliva can help to the problems of joining words when speaking. The skills trained socially can improve the use of the pragmatic language.

 Even with the delay, the verbal skill becomes often an area of strenght for the children with PWS.

 The IQs goes of 40 to 105, with an average of 70.

 Those with normal IQs have the typical troubles of learning. The areas of problem can include attention, short term auditory memory, and the abstract thought. The common strengths include the memory in the long term, while reading the skill, and the receptive language.

 Early stimulation should be done and the need for special education is necessary.

SEXUAL DEVELOPMENT

 The sexual hormones (the testosterone and the estrogen) are typically low. Both sexes can have a good response to the treatment for the deficiencies of the hormone, although there exist reports of colateral effects. Early pubic hair is common, but the puberty comes normally late. The fertility has not been documented in any sex.

PROBLEMS IN THE QUALITY OF LIFE

 Health normally is good in the individuals with PWS. If the weight is controlled, the life expectancy can be normal, and the health of the individual can increase to the maximum. The constant need for the restriction of food and direction of conduct can be the most stressful and difficult matter for the relatives.

 CONSULT YOUR DOCTOR AND A PSYCHOLOGIST.

CONTINUES: BARDET-BIEDL'S SYNDROME


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Medical supervision by Dr. Arieh Goldberg Kalik.
Bariatric physician, certified medical nutrition specialist. Certificate # 1010084

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