Analysis of hyperphagia in Prader-Willi Syndrome: alternatives for medication Treatment
DOI:
https://doi.org/10.34024/rnc.2024.v32.18851Keywords:
Prader-Willi Syndrome, Hyperphagia, TreatmentAbstract
Introduction. Prader-Willi Syndrome (PWS) is a rare and complex genetic condition characterized by errors in genomic imprinting, resulting in the loss of expression of paternal origin genes in the 15q11-q13 chromosomal region. PWS presents an extensive range of clinical manifestations and behavioral challenges, including temper tantrums, anxiety, obsessive-compulsive behaviors, rigidity, and deficits in social cognition. However, a distinctive behavioral phenotype in this syndrome is severe hyperphagia, making it the most prevalent syndromic form of life-threatening obesity. Despite advances in understanding this syndrome, growth hormone therapy remains the only therapy approved by the Food and Drug Administration (FDA) for use in children with PWS. However, there is no significant impact on reducing hyperphagia, and the high levels of caregiver burden faced by individuals with PWS reinforce the need to prioritize the search for therapeutic approaches aimed at treating hyperphagia. Objectives. Therefore, this study aims to identify and discuss the possibilities of drug treatment directed at hyperphagia in PWS at present. Methods. Data retrieval was conducted in PubMed and Virtual Health Library (VHL) databases, between August 2023 and June 2024, using the combination of the following descriptors: "Prader-Willi Syndrome," "hyperphagia," and "treatment," combined with the Boolean operator "AND." Results. Seven clinical trials were analyzed, covering therapies such as Liraglutide, GLWL-01, Choline Diazoxide, Topiramate, Oxytocin, and Carbetocin. The main results revealed variations in hyperphagia levels, depending on its severity, the medication used, dose, genetic profile, sex, and age of patients in each study. This diversity can significantly influence treatment response.Conclusion. Although several clinical studies have been conducted with different therapies for PWS, the results do not provide a definitive indication of which specific therapy should be adopted. Therefore, further research is crucial to explore new interventions.
Keywords. Prader-Willi Syndrome; Hyperphagia; Treatment.
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References
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Copyright (c) 2024 Lucas Vasconcelos Leitão Moreira, Rayana Elias Maia, Patrícia Vasconcelos Leitão Moreira, Sílvia Viana Bezerra da Cunha, Cláudio Renato Renato Silva Lima, Janine Agra Padilha, Jéssica Laureano Martins
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Accepted 2024-10-08
Published 2024-10-22
