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Dietary Management for Adolescents with Prader–Willi Syndrome

Prader–Willi syndrome (PWS) is a complex, multisystem neurodevelopmental disorder affecting approximately 1 in 25,000 live births. PWS is caused by absence of expression of paternally inherited imprinted genes on chromosome 15q11-q13. The syndrome typically occurs due to one of three genetic mechani...

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Detalles Bibliográficos
Autores principales: Miller, Jennifer L, Tan, Michael
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7457755/
https://www.ncbi.nlm.nih.gov/pubmed/32922110
http://dx.doi.org/10.2147/AHMT.S214893
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author Miller, Jennifer L
Tan, Michael
author_facet Miller, Jennifer L
Tan, Michael
author_sort Miller, Jennifer L
collection PubMed
description Prader–Willi syndrome (PWS) is a complex, multisystem neurodevelopmental disorder affecting approximately 1 in 25,000 live births. PWS is caused by absence of expression of paternally inherited imprinted genes on chromosome 15q11-q13. The syndrome typically occurs due to one of three genetic mechanisms: paternal deletion of involved genes, maternal uniparental disomy, or imprinting center defects. These genetic anomalies lead to well-described clinical phenotype that includes hypotonia, hypothalamic dysfunction, social and behavioral issues, life-threatening hyperphagia, and elevated probability of obesity. Adolescents with PWS are at the highest risk for development of life-threatening obesity due to increased access to food, decreased physical activity, and hyperphagia. Currently, the only treatment for the hyperphagia is environmental control, including locked kitchens and continuous supervision of the affected individual. Caloric intake must be restricted to prevent obesity, which subsequently increases the hunger drive even more. Research and clinical practice have demonstrated that increasing physical activity along with insuring a well-balanced, nutritionally dense diet can improve overall weight control in adolescents with PWS.
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spelling pubmed-74577552020-09-11 Dietary Management for Adolescents with Prader–Willi Syndrome Miller, Jennifer L Tan, Michael Adolesc Health Med Ther Review Prader–Willi syndrome (PWS) is a complex, multisystem neurodevelopmental disorder affecting approximately 1 in 25,000 live births. PWS is caused by absence of expression of paternally inherited imprinted genes on chromosome 15q11-q13. The syndrome typically occurs due to one of three genetic mechanisms: paternal deletion of involved genes, maternal uniparental disomy, or imprinting center defects. These genetic anomalies lead to well-described clinical phenotype that includes hypotonia, hypothalamic dysfunction, social and behavioral issues, life-threatening hyperphagia, and elevated probability of obesity. Adolescents with PWS are at the highest risk for development of life-threatening obesity due to increased access to food, decreased physical activity, and hyperphagia. Currently, the only treatment for the hyperphagia is environmental control, including locked kitchens and continuous supervision of the affected individual. Caloric intake must be restricted to prevent obesity, which subsequently increases the hunger drive even more. Research and clinical practice have demonstrated that increasing physical activity along with insuring a well-balanced, nutritionally dense diet can improve overall weight control in adolescents with PWS. Dove 2020-08-25 /pmc/articles/PMC7457755/ /pubmed/32922110 http://dx.doi.org/10.2147/AHMT.S214893 Text en © 2020 Miller and Tan. http://creativecommons.org/licenses/by-nc/3.0/ This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php).
spellingShingle Review
Miller, Jennifer L
Tan, Michael
Dietary Management for Adolescents with Prader–Willi Syndrome
title Dietary Management for Adolescents with Prader–Willi Syndrome
title_full Dietary Management for Adolescents with Prader–Willi Syndrome
title_fullStr Dietary Management for Adolescents with Prader–Willi Syndrome
title_full_unstemmed Dietary Management for Adolescents with Prader–Willi Syndrome
title_short Dietary Management for Adolescents with Prader–Willi Syndrome
title_sort dietary management for adolescents with prader–willi syndrome
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7457755/
https://www.ncbi.nlm.nih.gov/pubmed/32922110
http://dx.doi.org/10.2147/AHMT.S214893
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