Medication Trials for Hyperphagia and Food-Related Behaviors in Prader-Willi Syndrome

Overview

Journal Diseases

Date 2017 Sep 26

PMID 28943610

Citations 8

Authors

Jennifer L Miller

Theresa V Strong

Janalee Heinemann

Affiliations

Soon will be listed here.

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 characteristically have poor feeding and lack of appetite in infancy, followed by the development of weight gain and then uncontrolled appetite and lack of satiety, sometime after the age of two. The overwhelming drive to eat is coupled with reduced energy expenditure and decreased caloric requirements, thus, individuals with PWS will become severely obese unless their food intake is strictly controlled. The mechanisms underlying hyperphagia in PWS remain incompletely understood, and to date no drugs have proven effective in controlling appetite. However, clinical trials have started for several medications, which may provide therapeutic options for those with PWS. These medication trials may also provide insight into potential treatments for obesity in the general population. Ideally, these treatments will help alleviate the complex metabolic issues that are part of this syndrome.

Citing Articles

Prader-Willi syndrome: guidance for children and transition into adulthood.

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Measuring Meaningful Benefit-Risk Tradeoffs to Promote Patient-Focused Drug Development in Prader-Willi Syndrome: A Discrete-Choice Experiment.

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Variability and change over time of weight and BMI among adolescents and adults with Prader-Willi syndrome: a 6-month text-based observational study.

Vrana-Diaz C, Balasubramanian P, Kayadjanian N, Bohonowych J, Strong T Orphanet J Rare Dis. 2020; 15(1):233.

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Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome.

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The Global Prader-Willi Syndrome Registry: Development, Launch, and Early Demographics.

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