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Prader-Willi_Syndro 內(nèi)容簡(jiǎn)介：

By Bergen Holzworth
CONCORDIA COLLEGE – MOORHEAD, MN

Objectives
Be able to describe Prader-Willi Syndrome
Be familiar with the health effects associated with Prader-Willi Syndrome
To gain an understanding of the nutrition care process for patients with Prader-Willi Syndrome throughout their lifespan
Be able to identify ethical issues involved with nutritional care methods used in managing Prader-Willi Syndrome

Background Information
Prader-Willi Syndrome (PWS) is a Endocrine Hypothalamic Disorder
It affects 1 in 10,000 to 25,000 persons born
There are approximately 400, 000 people living with PWS worldwide
PWS is a congenital chromosomal disorder affecting chromosome 15
There is no cure to the syndrome but it can be managed through strict diet restriction, behavior management, and exercise regimenting
Most common genetically identified cause of obesity

Characteristics of PWS
Compulsive Hyperphagia
Abnormally increased appetite
Polyphagia
Excessive eating
Short stature with small hands and feet
Mild retardation
Hypogonadism
production of sex hormones and germ cells (sperm and eggs) is below normal which means they are sexually sterile

Characteristics Continued
Muscular Hypotonia
Low muscle tone
Decreased limb movement
Poor reflexes
Exhibit non-ambulatory behavior
Exercise and movement are avoided
Sedentary lifestyles are sought due to comfort
Reduced energy needs
Metabolism is slower than average

Characteristics Continued
Dental caries
Bizarre eating behaviors (gorging, hoarding, food stealing, eating inappropriate foods, etc.)
Poor sucking ability and failure to thrive in infancy
Varying degrees of obsessive compulsive behavior
Slow motor development
Obesity related diabetes in later childhood
Lack of emotional control
Drowsiness

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