Oral and craniofacial manifestations of mucopolysaccharidoses
Autor(a) principal: | |
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Data de Publicação: | 2020 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10284/8992 |
Resumo: | Mucopolysaccharidoses (MPS) are a group of inherited metabolic disorders caused by the deficiency of lysosomal enzymes necessary for the degradation of glycosaminoglycans (GAGs). Non-degraded GAGs accumulate inside the lysosomes and compromise cell function in different tissues and organs. This accumulation causes progressive and multisystemic damage, leading to a wide spectrum of clinical manifestations, including oral and craniofacial manifestations. This work aims, therefore, to conduct a literature review that promotes specific knowledge regarding the oral and craniofacial manifestations of MPS. The results converged to a variability of alterations, among them facial dysmorphism, macroglossia, lingual protrusion, anterior open bite, dental caries, gingival inflammation, enamel hypoplasia, taurodontism, condylar hypoplasia and presence of dentigerouscysts. Preventive and interceptive actions in oral health are used as a means of improving oral hygiene and reducing oral problems. Finally, there is a need for improvement in comprehensive care for patients with MPS. |
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Oral and craniofacial manifestations of mucopolysaccharidosesMucopolysaccharidosesOral manifestationsCraniofacial manifestationsOral healthMucopolysaccharidoses (MPS) are a group of inherited metabolic disorders caused by the deficiency of lysosomal enzymes necessary for the degradation of glycosaminoglycans (GAGs). Non-degraded GAGs accumulate inside the lysosomes and compromise cell function in different tissues and organs. This accumulation causes progressive and multisystemic damage, leading to a wide spectrum of clinical manifestations, including oral and craniofacial manifestations. This work aims, therefore, to conduct a literature review that promotes specific knowledge regarding the oral and craniofacial manifestations of MPS. The results converged to a variability of alterations, among them facial dysmorphism, macroglossia, lingual protrusion, anterior open bite, dental caries, gingival inflammation, enamel hypoplasia, taurodontism, condylar hypoplasia and presence of dentigerouscysts. Preventive and interceptive actions in oral health are used as a means of improving oral hygiene and reducing oral problems. Finally, there is a need for improvement in comprehensive care for patients with MPS.Repositório Institucional da Universidade Fernando PessoaLeal, FernandaCardoso, Inês LopesDias, Renata2020-09-21T15:25:17Z2020-06-29T07:47:36Z2020-06-29T00:00:00Z2020-06-29T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10284/8992eng2394-7489cv-prod-1961612info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2022-09-06T02:08:09Zoai:bdigital.ufp.pt:10284/8992Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T15:45:39.108394Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Oral and craniofacial manifestations of mucopolysaccharidoses |
title |
Oral and craniofacial manifestations of mucopolysaccharidoses |
spellingShingle |
Oral and craniofacial manifestations of mucopolysaccharidoses Leal, Fernanda Mucopolysaccharidoses Oral manifestations Craniofacial manifestations Oral health |
title_short |
Oral and craniofacial manifestations of mucopolysaccharidoses |
title_full |
Oral and craniofacial manifestations of mucopolysaccharidoses |
title_fullStr |
Oral and craniofacial manifestations of mucopolysaccharidoses |
title_full_unstemmed |
Oral and craniofacial manifestations of mucopolysaccharidoses |
title_sort |
Oral and craniofacial manifestations of mucopolysaccharidoses |
author |
Leal, Fernanda |
author_facet |
Leal, Fernanda Cardoso, Inês Lopes Dias, Renata |
author_role |
author |
author2 |
Cardoso, Inês Lopes Dias, Renata |
author2_role |
author author |
dc.contributor.none.fl_str_mv |
Repositório Institucional da Universidade Fernando Pessoa |
dc.contributor.author.fl_str_mv |
Leal, Fernanda Cardoso, Inês Lopes Dias, Renata |
dc.subject.por.fl_str_mv |
Mucopolysaccharidoses Oral manifestations Craniofacial manifestations Oral health |
topic |
Mucopolysaccharidoses Oral manifestations Craniofacial manifestations Oral health |
description |
Mucopolysaccharidoses (MPS) are a group of inherited metabolic disorders caused by the deficiency of lysosomal enzymes necessary for the degradation of glycosaminoglycans (GAGs). Non-degraded GAGs accumulate inside the lysosomes and compromise cell function in different tissues and organs. This accumulation causes progressive and multisystemic damage, leading to a wide spectrum of clinical manifestations, including oral and craniofacial manifestations. This work aims, therefore, to conduct a literature review that promotes specific knowledge regarding the oral and craniofacial manifestations of MPS. The results converged to a variability of alterations, among them facial dysmorphism, macroglossia, lingual protrusion, anterior open bite, dental caries, gingival inflammation, enamel hypoplasia, taurodontism, condylar hypoplasia and presence of dentigerouscysts. Preventive and interceptive actions in oral health are used as a means of improving oral hygiene and reducing oral problems. Finally, there is a need for improvement in comprehensive care for patients with MPS. |
publishDate |
2020 |
dc.date.none.fl_str_mv |
2020-09-21T15:25:17Z 2020-06-29T07:47:36Z 2020-06-29T00:00:00Z 2020-06-29T00:00:00Z |
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/10284/8992 |
url |
http://hdl.handle.net/10284/8992 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
2394-7489 cv-prod-1961612 |
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.source.none.fl_str_mv |
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
instname_str |
Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
instacron_str |
RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
collection |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799130322964578304 |