Mucopolissacaridose tipo VI (Síndrome de Maroteaux Lamy): relato de caso

Detalhes bibliográficos
Autor(a) principal: Santos, Aline Silva dos
Data de Publicação: 2011
Outros Autores: Santos Neto, Fernando Presídio dos, Silva, Delson Arcanjo, Souza, Delano Oliveira
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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spelling 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
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