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Oral disorders in children with Prader-Willi syndrome: a case control study

INTRODUCTION: Prader-Willi Syndrome (PWS) is a genetic disorder caused by the lack of expression of certain paternal genes located on chromosome 15q11-q13. This anomaly causes cognitive, neurological and endocrine abnormalities, among which one of the most important is hyperphagia. The aim of this s...

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Autores principales: Munné-Miralvés, Carla, Brunet-Llobet, Lluís, Cahuana-Cárdenas, Abel, Torné-Durán, Sergi, Miranda-Rius, Jaume, Rivera-Baró, Alejandro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7011482/
https://www.ncbi.nlm.nih.gov/pubmed/32041633
http://dx.doi.org/10.1186/s13023-020-1326-8
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author Munné-Miralvés, Carla
Brunet-Llobet, Lluís
Cahuana-Cárdenas, Abel
Torné-Durán, Sergi
Miranda-Rius, Jaume
Rivera-Baró, Alejandro
author_facet Munné-Miralvés, Carla
Brunet-Llobet, Lluís
Cahuana-Cárdenas, Abel
Torné-Durán, Sergi
Miranda-Rius, Jaume
Rivera-Baró, Alejandro
author_sort Munné-Miralvés, Carla
collection PubMed
description INTRODUCTION: Prader-Willi Syndrome (PWS) is a genetic disorder caused by the lack of expression of certain paternal genes located on chromosome 15q11-q13. This anomaly causes cognitive, neurological and endocrine abnormalities, among which one of the most important is hyperphagia. The aim of this study was to assess the oral health of children with PWA and to establish preventive criteria. RESULTS: Thirty patients with PWS (mean age 10.2 years) and 30 age- and gender-matched controls were included in the study. Twenty-six patients with PWS(86.6%) followed dietary treatment prescribed by their endocrinologist. Individuals with PWS had a mean caries index of 53.3% and Decayed Missing Filled teeth (DMFT) index 2.5, and 53.3% had gingivitis, in the control group the respective figures were 43.3%, 0.93, and 60%. Only the DMFT index (p 0.017) presented significant differences. Regarding stimulated salivary secretion, patients with PWS presented a mean of 0.475 ml/min with a pH of 6.15, while controls presented a mean of 0.848 ml/min with a pH of 7.53; the differences between the groups were statistically significant in both cases (p 0.032 and p 0.0001 respectively). The population with PWS presented a higher plaque index (> 2) than their healthy peers, but the differences were not significant. CONCLUSION: Pediatric patients with Prader-Willi syndrome have an increased risk of caries and gingivitis. The children with this syndrome have a decreased salivary flow and a more acidic salivary pH. In these patients, dental care is an essential part of their multidisciplinary medical treatment.
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spelling pubmed-70114822020-02-14 Oral disorders in children with Prader-Willi syndrome: a case control study Munné-Miralvés, Carla Brunet-Llobet, Lluís Cahuana-Cárdenas, Abel Torné-Durán, Sergi Miranda-Rius, Jaume Rivera-Baró, Alejandro Orphanet J Rare Dis Research INTRODUCTION: Prader-Willi Syndrome (PWS) is a genetic disorder caused by the lack of expression of certain paternal genes located on chromosome 15q11-q13. This anomaly causes cognitive, neurological and endocrine abnormalities, among which one of the most important is hyperphagia. The aim of this study was to assess the oral health of children with PWA and to establish preventive criteria. RESULTS: Thirty patients with PWS (mean age 10.2 years) and 30 age- and gender-matched controls were included in the study. Twenty-six patients with PWS(86.6%) followed dietary treatment prescribed by their endocrinologist. Individuals with PWS had a mean caries index of 53.3% and Decayed Missing Filled teeth (DMFT) index 2.5, and 53.3% had gingivitis, in the control group the respective figures were 43.3%, 0.93, and 60%. Only the DMFT index (p 0.017) presented significant differences. Regarding stimulated salivary secretion, patients with PWS presented a mean of 0.475 ml/min with a pH of 6.15, while controls presented a mean of 0.848 ml/min with a pH of 7.53; the differences between the groups were statistically significant in both cases (p 0.032 and p 0.0001 respectively). The population with PWS presented a higher plaque index (> 2) than their healthy peers, but the differences were not significant. CONCLUSION: Pediatric patients with Prader-Willi syndrome have an increased risk of caries and gingivitis. The children with this syndrome have a decreased salivary flow and a more acidic salivary pH. In these patients, dental care is an essential part of their multidisciplinary medical treatment. BioMed Central 2020-02-10 /pmc/articles/PMC7011482/ /pubmed/32041633 http://dx.doi.org/10.1186/s13023-020-1326-8 Text en © The Author(s). 2020 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research
Munné-Miralvés, Carla
Brunet-Llobet, Lluís
Cahuana-Cárdenas, Abel
Torné-Durán, Sergi
Miranda-Rius, Jaume
Rivera-Baró, Alejandro
Oral disorders in children with Prader-Willi syndrome: a case control study
title Oral disorders in children with Prader-Willi syndrome: a case control study
title_full Oral disorders in children with Prader-Willi syndrome: a case control study
title_fullStr Oral disorders in children with Prader-Willi syndrome: a case control study
title_full_unstemmed Oral disorders in children with Prader-Willi syndrome: a case control study
title_short Oral disorders in children with Prader-Willi syndrome: a case control study
title_sort oral disorders in children with prader-willi syndrome: a case control study
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7011482/
https://www.ncbi.nlm.nih.gov/pubmed/32041633
http://dx.doi.org/10.1186/s13023-020-1326-8
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