Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de caso
Autor(a) principal: | |
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Data de Publicação: | 2011 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Institucional da UFBA |
Texto Completo: | http://www.repositorio.ufba.br/ri/handle/ri/5705 |
Resumo: | p. 194-197 |
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Santos, Aline Silva dosSantos Neto, Fernando Presídio dosSilva, Delson ArcanjoSouza, Delano OliveiraSantos, Aline Silva dosSantos Neto, Fernando Presídio dosSilva, Delson ArcanjoSouza, Delano Oliveira2012-04-23T13:20:06Z2012-04-23T13:20:06Z2011http://www.repositorio.ufba.br/ri/handle/ri/5705v.10, n.2p. 194-197Mucopolissacaridose VI (MPSVI), conhecida como Síndrome de Maroteaux-Lamy é uma doença hereditária, resultante da deficiência da enzima N-acetilgalactosamina-4-sulfatase, causando acúmulo do glicosaminoglicano Dermatan sulfato em todos os tecidos, resultando em alterações nos órgãos afetados. O diagnóstico é obtido através de excreção aumentada de Dermatan sulfato na urina e demonstração reduzida da enzima supracitada. O tratamento é realizado a fim de melhorar a qualidade de vida dos portadores, tratando as manifestações clínicas, ou através de transplante de medula óssea ou terapia de reposição enzimática. Esse estudo descreve o caso clínico de um paciente portador da MPS VI atendido no Ambulatório de Odontologia do Hospital Ana Neri, apresentando manifestações típicas faciais da doença.Submitted by Barcellos Bárbara (barbara_barcellos@yahoo.com.br) on 2012-04-23T13:20:06Z No. of bitstreams: 1 5250-15821-2-PB[1].pdf: 166646 bytes, checksum: 15ceceee6026120f412aaf62c06c9989 (MD5)Made available in DSpace on 2012-04-23T13:20:06Z (GMT). No. of bitstreams: 1 5250-15821-2-PB[1].pdf: 166646 bytes, checksum: 15ceceee6026120f412aaf62c06c9989 (MD5) Previous issue date: 2011SalvadorMucopolissacaridose VISíndrome de Marateaux - Lamy.Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de casoRevista de Ciências Médicas e Biológicasinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleporreponame:Repositório Institucional da UFBAinstname:Universidade Federal da Bahia (UFBA)instacron:UFBAinfo:eu-repo/semantics/openAccessORIGINAL5250-15821-2-PB[1].pdf5250-15821-2-PB[1].pdfapplication/pdf166646https://repositorio.ufba.br/bitstream/ri/5705/1/5250-15821-2-PB%5b1%5d.pdf15ceceee6026120f412aaf62c06c9989MD51LICENSElicense.txtlicense.txttext/plain1762https://repositorio.ufba.br/bitstream/ri/5705/2/license.txt1b89a9a0548218172d7c829f87a0eab9MD52TEXT5250-15821-2-PB[1].pdf.txt5250-15821-2-PB[1].pdf.txtExtracted texttext/plain18351https://repositorio.ufba.br/bitstream/ri/5705/3/5250-15821-2-PB%5b1%5d.pdf.txt7a317e1b8f14ae1e71f8a6f533a4a613MD53ri/57052022-07-05 14:03:21.179oai:repositorio.ufba.br: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Repositório InstitucionalPUBhttp://192.188.11.11:8080/oai/requestopendoar:19322022-07-05T17:03:21Repositório Institucional da UFBA - Universidade Federal da Bahia (UFBA)false |
dc.title.pt_BR.fl_str_mv |
Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de caso |
dc.title.alternative.pt_BR.fl_str_mv |
Revista de Ciências Médicas e Biológicas |
title |
Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de caso |
spellingShingle |
Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de caso Santos, Aline Silva dos Mucopolissacaridose VI Síndrome de Marateaux - Lamy. |
title_short |
Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de caso |
title_full |
Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de caso |
title_fullStr |
Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de caso |
title_full_unstemmed |
Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de caso |
title_sort |
Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de caso |
author |
Santos, Aline Silva dos |
author_facet |
Santos, Aline Silva dos Santos Neto, Fernando Presídio dos Silva, Delson Arcanjo Souza, Delano Oliveira |
author_role |
author |
author2 |
Santos Neto, Fernando Presídio dos Silva, Delson Arcanjo Souza, Delano Oliveira |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Santos, Aline Silva dos Santos Neto, Fernando Presídio dos Silva, Delson Arcanjo Souza, Delano Oliveira Santos, Aline Silva dos Santos Neto, Fernando Presídio dos Silva, Delson Arcanjo Souza, Delano Oliveira |
dc.subject.por.fl_str_mv |
Mucopolissacaridose VI Síndrome de Marateaux - Lamy. |
topic |
Mucopolissacaridose VI Síndrome de Marateaux - Lamy. |
description |
p. 194-197 |
publishDate |
2011 |
dc.date.issued.fl_str_mv |
2011 |
dc.date.accessioned.fl_str_mv |
2012-04-23T13:20:06Z |
dc.date.available.fl_str_mv |
2012-04-23T13:20:06Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
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publishedVersion |
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http://www.repositorio.ufba.br/ri/handle/ri/5705 |
dc.identifier.number.pt_BR.fl_str_mv |
v.10, n.2 |
url |
http://www.repositorio.ufba.br/ri/handle/ri/5705 |
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v.10, n.2 |
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por |
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