Prader-Willie's syndrome

 
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PRADER WILLIE SYNDROME (PWS)

WHAT IS IT?

 It is an eating disorder of the 15th chromosome and appears in 1 of 12,000 to 15,000 births, appears equally in both sexes and all races. Its more important characteristics are:

Hypotonicity, (flabbiness of the muscles).

Hypogonadism (insufficient development of the genital).

Hyperphagia (to eat without stopping).

Psychomotor delay.

Abnormal behaviors.

Morbid obesity (very obese).

WHICH IS THE CAUSE?

 The cause is genetic from genes not identified, attributed in 70% to the Father.

DIAGNOSTIC

 The individuals who present clinical suggestive history should be referred for the genetic test. The analysis of methylation of the DNA confirms the diagnostic of the PWS.

 The patients who had negative or slightly conclusive tests with old methods should be reexamined.

 All families must receive genetic advice.

CLINICAL PICTURE:

MAJOR SIGNS:

 Hypotonicity central neonatal and infantile, improving with the age.

 Problems of nourishment.

 Excessive or quick increase of weight between 1 and 6 years of age.

 Central obesity.

 Facial distinctive characteristics.

 Narrow diameter of the forehead.

 Eyes in shape of almond.

 Small mouth with superior thin lip and corners of the mouth drops.

 Hypogonadism, even the testicles do not descend and the penis is small, the gonad maturation is incomplete and puberty gets late(after 16 years of age), whereas the women do not menstruate or they menstruate very belatedly.

 The delay in the global development is seen before the 6 years of age; and to moderate their mental delay or problems, they learn from older children.

 The hyperphagia consists of obsession for the food and to eat continually without control.

MINOR SIGNS:

 Low fetal movement.

 Infantile lethargy.

 Weak weeping.

 Violent conduct.

 Obsessive - compulsive conduct.

 Trend to be argumentative, rigid, manipulative, possessive, and stubborn.

 Disturbance of sleeping.

 Small stature for same age.

 Low pigmentation of the skin.

 Small hands and/or feet for same age.

 Thick, viscous saliva with scabs in the corners of the mouth.

 Disorders of the language.

 Retiring skin.

FOLLOWING: THE DISORDER OF THE APPETITE IN THE PWS


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Dear visitor: The object of this web site is to inform and educate. Therefore, the information contained herein does not substitute professional medical attention.
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Medical supervision by Dr. Arieh Goldberg Kalik.
Bariatric physician, certified medical nutrition specialist. Certificate # 1010084

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