Management of Hyperphagia and Obesity in Prader–Willi Syndrome
Prader-Willi syndrome (PWS) is a neurodevelopmental disorder caused by the absence of paternally expressed imprinted genes on chromosome 15q11–13. Individuals with PWS typically experience feeding difficulties and a lack of appetite in infa...
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| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Ewha Womans University College of Medicine
2023-12-01
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| Series: | The Ewha Medical Journal |
| Subjects: | |
| Online Access: | http://www.e-emj.org/archive/view_article?pid=emj-46-s1-32 |
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| Summary: | Prader-Willi syndrome (PWS) is a neurodevelopmental disorder caused by the
absence of paternally expressed imprinted genes on chromosome 15q11–13.
Individuals with PWS typically experience feeding difficulties and a lack of
appetite in infancy, followed by weight gain, uncontrolled appetite, and a lack
of satiety. Hyperphagia in PWS is exacerbated by impaired satiety, low energy
expenditure, and intellectual difficulties, including obsessive-compulsive
disorder and/or autistic behaviors. Without rigorous external management of
their eating behaviors, patients with PWS become severely obese and are at a
higher risk of obesity-related morbidities, such as type 2 diabetes, obstructive
sleep apnea, and hypertension. Moreover, the main causes of death for PWS are
obesity-related comorbidities, such as renal failure, pulmonary embolism, and
respiratory and heart failure. Clinical experiences with different supplements,
diets, and other methods have not been encouraging. However, therapeutic options
for patients with PWS may be improving, based on recent clinical trials for a
number of medications. This report reviews the causes and management of
hyperphagia, as well as previous and recent clinical trials aimed at treating
hyperplasia in PWS. We are optimistic that the novel treatments currently in
development will help alleviate the complex metabolic issues associated with
PWS. |
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| ISSN: | 2234-2591 |