Oral findings in williams-beuren syndrome

Detalhes bibliográficos
Autor(a) principal: Shirlene Barbosa Pimentel Ferreira
Data de Publicação: 2017
Outros Autores: Melissa Machado Viana, Naiara Gonçalves Fonseca Maia, Letícia Lima Leão, Renato Assis Machado, Ricardo Della Coletta, Marcos José Burle de Aguiar, Hercílio Martelli Júnior
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Institucional da UFMG
Texto Completo: http://hdl.handle.net/1843/40021
Resumo: Background: Williams-Beuren syndrome (WBS; OMIM #194050) is a developmental disorder characterized by congenital heart disease, intellectual disability, dysmorphic facial features and ophthalmologic abnormalities. Oral abnormalities are also described in clinical manifestations of the disease. This paper describes orofacial features in patients with WBS. Material and Methods: Seventeen patients with a confirmed molecular diagnosis of WBS were examined for oral abnormalities through clinical oral evaluations and panoramic radiography. Results: Malocclusion, specifically with dental midline deviation, and high-arched palate were the most common findings. Conclusions: The present results contribute to knowledge on the orofacial manifestations of WBS. Since such patients with WBS may develop severe oral abnormalities, early detection and treatment can help improve their quality of life.
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spelling 2022-03-11T18:53:35Z2022-03-11T18:53:35Z20172311610.4317/medoral.218341698-6946http://hdl.handle.net/1843/40021Background: Williams-Beuren syndrome (WBS; OMIM #194050) is a developmental disorder characterized by congenital heart disease, intellectual disability, dysmorphic facial features and ophthalmologic abnormalities. Oral abnormalities are also described in clinical manifestations of the disease. This paper describes orofacial features in patients with WBS. Material and Methods: Seventeen patients with a confirmed molecular diagnosis of WBS were examined for oral abnormalities through clinical oral evaluations and panoramic radiography. Results: Malocclusion, specifically with dental midline deviation, and high-arched palate were the most common findings. Conclusions: The present results contribute to knowledge on the orofacial manifestations of WBS. Since such patients with WBS may develop severe oral abnormalities, early detection and treatment can help improve their quality of life.Introdução: A síndrome de Williams-Beuren (WBS; OMIM #194050) é um transtorno do desenvolvimento caracterizado por cardiopatia congênita, deficiência intelectual, características faciais dismórficas e anormalidades oftalmológicas. Anormalidades bucais também são descritas nas manifestações clínicas da doença. Este artigo descreve orofacial características em pacientes com SWB. Material e Métodos: Dezessete pacientes com diagnóstico molecular confirmado de SWB foram examinados para anormalidades por meio de avaliações clínicas orais e radiografia panorâmica. Resultados: Má oclusão, especificamente com desvio da linha média dentária, e palato arqueado foram os mais comuns. descobertas. Conclusões: Os presentes resultados contribuem para o conhecimento das manifestações orofaciais da SWB. Uma vez que tal pacientes com SWB podem desenvolver anormalidades orais graves, a detecção precoce e o tratamento podem ajudar a melhorar sua qualidade de vida.engUniversidade Federal de Minas GeraisUFMGBrasilMEDICINA - FACULDADE DE MEDICINAMedicina oral patologia oral y cirugia bucalOdontologiaAnomalias congénitasCaracterísticas orofaciaisSíndrome de Williams-BeurenCongenital abnormalitiesoral featuresWilliams-Beuren syndromeOral findings in williams-beuren syndromeAchados orais na síndrome de williams-beureninfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5822531/#:~:text=In%20the%20oral%20cavity%2C%20wide,disease%20(8%2C9).Shirlene Barbosa Pimentel FerreiraMelissa Machado VianaNaiara Gonçalves Fonseca MaiaLetícia Lima LeãoRenato Assis MachadoRicardo Della ColettaMarcos José Burle de AguiarHercílio Martelli Júniorapplication/pdfinfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFMGinstname:Universidade Federal de Minas Gerais (UFMG)instacron:UFMGORIGINAL2017_Oral findings in williams-beuren syndrome.pdf2017_Oral findings in williams-beuren syndrome.pdfapplication/pdf1484624https://repositorio.ufmg.br/bitstream/1843/40021/2/2017_Oral%20findings%20in%20williams-beuren%20syndrome.pdf5b432f460e75b63a72997efdee5a6ce6MD52LICENSELicense.txtLicense.txttext/plain; charset=utf-82042https://repositorio.ufmg.br/bitstream/1843/40021/1/License.txtfa505098d172de0bc8864fc1287ffe22MD511843/400212022-03-11 15:53:35.708oai:repositorio.ufmg.br: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Repositório de PublicaçõesPUBhttps://repositorio.ufmg.br/oaiopendoar:2022-03-11T18:53:35Repositório Institucional da UFMG - Universidade Federal de Minas Gerais (UFMG)false
dc.title.pt_BR.fl_str_mv Oral findings in williams-beuren syndrome
dc.title.alternative.pt_BR.fl_str_mv Achados orais na síndrome de williams-beuren
title Oral findings in williams-beuren syndrome
spellingShingle Oral findings in williams-beuren syndrome
Shirlene Barbosa Pimentel Ferreira
Congenital abnormalities
oral features
Williams-Beuren syndrome
Odontologia
Anomalias congénitas
Características orofaciais
Síndrome de Williams-Beuren
title_short Oral findings in williams-beuren syndrome
title_full Oral findings in williams-beuren syndrome
title_fullStr Oral findings in williams-beuren syndrome
title_full_unstemmed Oral findings in williams-beuren syndrome
title_sort Oral findings in williams-beuren syndrome
author Shirlene Barbosa Pimentel Ferreira
author_facet Shirlene Barbosa Pimentel Ferreira
Melissa Machado Viana
Naiara Gonçalves Fonseca Maia
Letícia Lima Leão
Renato Assis Machado
Ricardo Della Coletta
Marcos José Burle de Aguiar
Hercílio Martelli Júnior
author_role author
author2 Melissa Machado Viana
Naiara Gonçalves Fonseca Maia
Letícia Lima Leão
Renato Assis Machado
Ricardo Della Coletta
Marcos José Burle de Aguiar
Hercílio Martelli Júnior
author2_role author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv Shirlene Barbosa Pimentel Ferreira
Melissa Machado Viana
Naiara Gonçalves Fonseca Maia
Letícia Lima Leão
Renato Assis Machado
Ricardo Della Coletta
Marcos José Burle de Aguiar
Hercílio Martelli Júnior
dc.subject.por.fl_str_mv Congenital abnormalities
oral features
Williams-Beuren syndrome
topic Congenital abnormalities
oral features
Williams-Beuren syndrome
Odontologia
Anomalias congénitas
Características orofaciais
Síndrome de Williams-Beuren
dc.subject.other.pt_BR.fl_str_mv Odontologia
Anomalias congénitas
Características orofaciais
Síndrome de Williams-Beuren
description Background: Williams-Beuren syndrome (WBS; OMIM #194050) is a developmental disorder characterized by congenital heart disease, intellectual disability, dysmorphic facial features and ophthalmologic abnormalities. Oral abnormalities are also described in clinical manifestations of the disease. This paper describes orofacial features in patients with WBS. Material and Methods: Seventeen patients with a confirmed molecular diagnosis of WBS were examined for oral abnormalities through clinical oral evaluations and panoramic radiography. Results: Malocclusion, specifically with dental midline deviation, and high-arched palate were the most common findings. Conclusions: The present results contribute to knowledge on the orofacial manifestations of WBS. Since such patients with WBS may develop severe oral abnormalities, early detection and treatment can help improve their quality of life.
publishDate 2017
dc.date.issued.fl_str_mv 2017
dc.date.accessioned.fl_str_mv 2022-03-11T18:53:35Z
dc.date.available.fl_str_mv 2022-03-11T18:53:35Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/1843/40021
dc.identifier.doi.pt_BR.fl_str_mv 10.4317/medoral.21834
dc.identifier.issn.pt_BR.fl_str_mv 1698-6946
identifier_str_mv 10.4317/medoral.21834
1698-6946
url http://hdl.handle.net/1843/40021
dc.language.iso.fl_str_mv eng
language eng
dc.relation.ispartof.pt_BR.fl_str_mv Medicina oral patologia oral y cirugia bucal
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Universidade Federal de Minas Gerais
dc.publisher.initials.fl_str_mv UFMG
dc.publisher.country.fl_str_mv Brasil
dc.publisher.department.fl_str_mv MEDICINA - FACULDADE DE MEDICINA
publisher.none.fl_str_mv Universidade Federal de Minas Gerais
dc.source.none.fl_str_mv reponame:Repositório Institucional da UFMG
instname:Universidade Federal de Minas Gerais (UFMG)
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instname_str Universidade Federal de Minas Gerais (UFMG)
instacron_str UFMG
institution UFMG
reponame_str Repositório Institucional da UFMG
collection Repositório Institucional da UFMG
bitstream.url.fl_str_mv https://repositorio.ufmg.br/bitstream/1843/40021/2/2017_Oral%20findings%20in%20williams-beuren%20syndrome.pdf
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