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Metabolic Syndrome In Children With Prader-Willi Syndrome: The Effect Of Obesity




Obesity is the most common cause of metabolic complications and poor quality of life in Children with Prader-Willi syndrome (PWS) . Hyperphagia and obesity develop after an initial phase of poor feeding and failure to thrive. Several mechanisms for the aetiology of obesity in PWS are proposed, which include disruption in hypothalamic pathways of satiety control resulting in hyperphagia, aberration in hormones regulating food intake, reduced energy expenditure because of hypotonia and altered behaviour with features of autism spectrum disorder. Profound muscular hypotonia prevents PWS patients from becoming physically active, causing reduced muscle movements and hence reduced energy expenditure. In this review, the metabolic syndrome associated with Prader-Willi Syndrome in Children and the effect of obesity is analyzed.



Prader–Willi syndrome (PWS), also known as Labhart–Willi syndrome, Prader’s syndrome, Prader–Labhart–Willi-Fanconi syndrome is a genetic disorder due to loss of function of specific genes.  In newborns, symptoms include weak muscles, poor feeding, and slow development. Beginning in childhood, the person becomes constantly hungry, which often leads to obesity and type 2 diabetes. Also, mild to moderate intellectual impairment and behavioral problems are typical (GHR, 2014).  Often, people have a narrow forehead, small hands and feet, short height, light skin and hair, and are unable to have children.[3]

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About 74% of cases occur when part of the father’s chromosome 15 is deleted. In another 25% of cases, the person has two copies of chromosome 15 from their mother and none from their father. As parts of the chromosome from the mother are turned off, they end up with no working copies of certain genes. PWS is not generally inherited, but instead the genetic changes happen during the formation of the egg, sperm, or in early development (GHR, 2014). No risk factors are known. Those that have one child with PWS have less than a 1% chance of the next child being affected (NICHD, 2016). A similar mechanism occurs in Angelman syndrome, except the defective chromosome 15 is from the mother or two copies are from the father (NICHD, 2016; GHR, 2015).

Prader–Willi syndrome has no cure. Treatment may improve outcomes, especially if carried out early (NICHD, 2014). In newborns, feeding difficulties may be supported with feeding tubes. Strict food supervision is typically required starting around the age of three, in combination with an exercise program. Growth hormone therapy also improves outcomes. Counseling and medications may help with some behavioral problems (NICHD, 2014).  PWS affects between 1 in 10,000 and 1 in 30,000 people (GHR, 2016). Males and females are affected equally (NICHD, 2014). The condition is named after Swiss physicians Andrea Prader, Heinrich Willi, and Alexis Labhart, who described it in detail in 1956. An earlier description was made in 1887 by British physician John Langdon Down (Mia and Mohan, 2016; Jorde et al., 2015).

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In this review, the metabolic syndrome in children with Prader-Willi Syndrome was analyzed.

Pages:  61

Category: Seminar

Format:  Word & PDF        

Chapters: 1-5

Material contains Table of Content, Abstract and References.

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