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Phenotypic Features and Salivary Parameters in Patients with Ectodermal Dysplasia: Report of Three Cases

Ectodermal dysplasia (ED) is a rare hereditary disorder affecting the development of ectoderm-derived organs and tissues. The aim of this study was to describe phenotypic features and the therapeutic approach in dentistry among three patients with ED, correlating their data with the literature. Addi...

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Autores principales: Gomes, Mônica Fernandes, Sichi, Luigi Giovanni Bernardo, Giannasi, Lilian Chrystiane, Amorim, José Benedito Oliveira, da Rocha, João Carlos, Koga-Ito, Cristiane Yumi, Salgado, Miguel Angel Castillo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5941722/
https://www.ncbi.nlm.nih.gov/pubmed/29854479
http://dx.doi.org/10.1155/2018/2409212
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author Gomes, Mônica Fernandes
Sichi, Luigi Giovanni Bernardo
Giannasi, Lilian Chrystiane
Amorim, José Benedito Oliveira
da Rocha, João Carlos
Koga-Ito, Cristiane Yumi
Salgado, Miguel Angel Castillo
author_facet Gomes, Mônica Fernandes
Sichi, Luigi Giovanni Bernardo
Giannasi, Lilian Chrystiane
Amorim, José Benedito Oliveira
da Rocha, João Carlos
Koga-Ito, Cristiane Yumi
Salgado, Miguel Angel Castillo
author_sort Gomes, Mônica Fernandes
collection PubMed
description Ectodermal dysplasia (ED) is a rare hereditary disorder affecting the development of ectoderm-derived organs and tissues. The aim of this study was to describe phenotypic features and the therapeutic approach in dentistry among three patients with ED, correlating their data with the literature. Additionally, to investigate the salivary gland disorders and their impacts on oral microbiota, we performed salivary tests, including salivary flow rate, salivary buffering capacity, and concentration levels of mutans streptococci, lactobacilli, and yeasts. All patients presented oligodontia, resulting in a significant masticatory dysfunction and aesthetic impairment. The counts of mutans streptococci (n=3) and yeasts (n=2) were high; on the other hand, the count of lactobacilli (n=3) was low. Therefore, salivary and microbiological tests showed that the patients with ED, particularly the hypohidrotic type, presented a high risk of enamel caries and susceptibility to oral infections, which may be likely triggered by reduction of salivary flow and/or possible immunological disorders.
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spelling pubmed-59417222018-05-31 Phenotypic Features and Salivary Parameters in Patients with Ectodermal Dysplasia: Report of Three Cases Gomes, Mônica Fernandes Sichi, Luigi Giovanni Bernardo Giannasi, Lilian Chrystiane Amorim, José Benedito Oliveira da Rocha, João Carlos Koga-Ito, Cristiane Yumi Salgado, Miguel Angel Castillo Case Rep Dent Case Report Ectodermal dysplasia (ED) is a rare hereditary disorder affecting the development of ectoderm-derived organs and tissues. The aim of this study was to describe phenotypic features and the therapeutic approach in dentistry among three patients with ED, correlating their data with the literature. Additionally, to investigate the salivary gland disorders and their impacts on oral microbiota, we performed salivary tests, including salivary flow rate, salivary buffering capacity, and concentration levels of mutans streptococci, lactobacilli, and yeasts. All patients presented oligodontia, resulting in a significant masticatory dysfunction and aesthetic impairment. The counts of mutans streptococci (n=3) and yeasts (n=2) were high; on the other hand, the count of lactobacilli (n=3) was low. Therefore, salivary and microbiological tests showed that the patients with ED, particularly the hypohidrotic type, presented a high risk of enamel caries and susceptibility to oral infections, which may be likely triggered by reduction of salivary flow and/or possible immunological disorders. Hindawi 2018-03-20 /pmc/articles/PMC5941722/ /pubmed/29854479 http://dx.doi.org/10.1155/2018/2409212 Text en Copyright © 2018 Mônica Fernandes Gomes et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Gomes, Mônica Fernandes
Sichi, Luigi Giovanni Bernardo
Giannasi, Lilian Chrystiane
Amorim, José Benedito Oliveira
da Rocha, João Carlos
Koga-Ito, Cristiane Yumi
Salgado, Miguel Angel Castillo
Phenotypic Features and Salivary Parameters in Patients with Ectodermal Dysplasia: Report of Three Cases
title Phenotypic Features and Salivary Parameters in Patients with Ectodermal Dysplasia: Report of Three Cases
title_full Phenotypic Features and Salivary Parameters in Patients with Ectodermal Dysplasia: Report of Three Cases
title_fullStr Phenotypic Features and Salivary Parameters in Patients with Ectodermal Dysplasia: Report of Three Cases
title_full_unstemmed Phenotypic Features and Salivary Parameters in Patients with Ectodermal Dysplasia: Report of Three Cases
title_short Phenotypic Features and Salivary Parameters in Patients with Ectodermal Dysplasia: Report of Three Cases
title_sort phenotypic features and salivary parameters in patients with ectodermal dysplasia: report of three cases
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5941722/
https://www.ncbi.nlm.nih.gov/pubmed/29854479
http://dx.doi.org/10.1155/2018/2409212
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