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Review Article
[English]
Management of Hyperphagia and Obesity in Prader–Willi Syndrome
JiHoon Hwang, Sung Yoon Cho
Ewha Med J 2023;46(s1):e32.   Published online December 31, 2023
DOI: https://doi.org/10.12771/emj.2023.e32
ABSTRACT

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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  • Emerging Infectious Diseases at the End of the Fourth Year of the COVID-19 Pandemic and Recent Updates on Colorectal and Pediatric Endocrine Diseases
    Sun Huh
    The Ewha Medical Journal.2023;[Epub]     CrossRef
  • 116 View
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  • 1 Crossref
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