Juvenile Dermatomyositis Forty Years On: Case Report
Autor(a) principal: | |
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Data de Publicação: | 2018 |
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/10400.17/3086 |
Resumo: | We present a case report of a 42 year old female, diagnosed at the age of 3 with Juvenile Dermatomyositis. The clinical course was severe and refractory to immunosuppressive therapy. Currently, she is mostly affected by severe muscle atrophy, large joint contractures, calcinosis, and a lipodystrophy associated metabolic syndrome with hypertriglyceridemia, insulin resistance, high total testosterone and hepatic steatosis. She developed Hodgkin´s lymphoma in the course of her disease. Personalized therapeutic choices are discussed as regards juvenile dermatomyositis complications. |
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Juvenile Dermatomyositis Forty Years On: Case ReportCalcinosis/diagnosisCalcinosis/pathologyDermatomyositis/diagnosisDermatomyositis/pathologyHCC DAUTOIMHDE IMAHCC ENDDisease ProgressionInsulin ResistanceLipodystrophy/diagnosisLipodystrophy/pathologyMetabolic Syndrome/diagnosisMetabolic Syndrome/pathologyMuscular Atrophy/diagnosisMuscular Atrophy/pathologySeverity of Illness IndexWe present a case report of a 42 year old female, diagnosed at the age of 3 with Juvenile Dermatomyositis. The clinical course was severe and refractory to immunosuppressive therapy. Currently, she is mostly affected by severe muscle atrophy, large joint contractures, calcinosis, and a lipodystrophy associated metabolic syndrome with hypertriglyceridemia, insulin resistance, high total testosterone and hepatic steatosis. She developed Hodgkin´s lymphoma in the course of her disease. Personalized therapeutic choices are discussed as regards juvenile dermatomyositis complications.ElsevierRepositório do Centro Hospitalar Universitário de Lisboa Central, EPERego de Figueiredo, IGuerreiro Castro, SBernardino, VSilva Nunes, JAlves, PMoraes-Fontes, MF2018-10-22T12:16:08Z20182018-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.17/3086engNeuromuscul Disord. 2018 Sep;28(9):791-79710.1016/j.nmd.2018.06.011info: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:RCAAP2023-03-10T09:41:11Zoai:repositorio.chlc.min-saude.pt:10400.17/3086Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T17:20:25.531444Repositório 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 |
Juvenile Dermatomyositis Forty Years On: Case Report |
title |
Juvenile Dermatomyositis Forty Years On: Case Report |
spellingShingle |
Juvenile Dermatomyositis Forty Years On: Case Report Rego de Figueiredo, I Calcinosis/diagnosis Calcinosis/pathology Dermatomyositis/diagnosis Dermatomyositis/pathology HCC DAUTOIM HDE IMA HCC END Disease Progression Insulin Resistance Lipodystrophy/diagnosis Lipodystrophy/pathology Metabolic Syndrome/diagnosis Metabolic Syndrome/pathology Muscular Atrophy/diagnosis Muscular Atrophy/pathology Severity of Illness Index |
title_short |
Juvenile Dermatomyositis Forty Years On: Case Report |
title_full |
Juvenile Dermatomyositis Forty Years On: Case Report |
title_fullStr |
Juvenile Dermatomyositis Forty Years On: Case Report |
title_full_unstemmed |
Juvenile Dermatomyositis Forty Years On: Case Report |
title_sort |
Juvenile Dermatomyositis Forty Years On: Case Report |
author |
Rego de Figueiredo, I |
author_facet |
Rego de Figueiredo, I Guerreiro Castro, S Bernardino, V Silva Nunes, J Alves, P Moraes-Fontes, MF |
author_role |
author |
author2 |
Guerreiro Castro, S Bernardino, V Silva Nunes, J Alves, P Moraes-Fontes, MF |
author2_role |
author author author author author |
dc.contributor.none.fl_str_mv |
Repositório do Centro Hospitalar Universitário de Lisboa Central, EPE |
dc.contributor.author.fl_str_mv |
Rego de Figueiredo, I Guerreiro Castro, S Bernardino, V Silva Nunes, J Alves, P Moraes-Fontes, MF |
dc.subject.por.fl_str_mv |
Calcinosis/diagnosis Calcinosis/pathology Dermatomyositis/diagnosis Dermatomyositis/pathology HCC DAUTOIM HDE IMA HCC END Disease Progression Insulin Resistance Lipodystrophy/diagnosis Lipodystrophy/pathology Metabolic Syndrome/diagnosis Metabolic Syndrome/pathology Muscular Atrophy/diagnosis Muscular Atrophy/pathology Severity of Illness Index |
topic |
Calcinosis/diagnosis Calcinosis/pathology Dermatomyositis/diagnosis Dermatomyositis/pathology HCC DAUTOIM HDE IMA HCC END Disease Progression Insulin Resistance Lipodystrophy/diagnosis Lipodystrophy/pathology Metabolic Syndrome/diagnosis Metabolic Syndrome/pathology Muscular Atrophy/diagnosis Muscular Atrophy/pathology Severity of Illness Index |
description |
We present a case report of a 42 year old female, diagnosed at the age of 3 with Juvenile Dermatomyositis. The clinical course was severe and refractory to immunosuppressive therapy. Currently, she is mostly affected by severe muscle atrophy, large joint contractures, calcinosis, and a lipodystrophy associated metabolic syndrome with hypertriglyceridemia, insulin resistance, high total testosterone and hepatic steatosis. She developed Hodgkin´s lymphoma in the course of her disease. Personalized therapeutic choices are discussed as regards juvenile dermatomyositis complications. |
publishDate |
2018 |
dc.date.none.fl_str_mv |
2018-10-22T12:16:08Z 2018 2018-01-01T00:00:00Z |
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/10400.17/3086 |
url |
http://hdl.handle.net/10400.17/3086 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Neuromuscul Disord. 2018 Sep;28(9):791-797 10.1016/j.nmd.2018.06.011 |
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 |
Elsevier |
publisher.none.fl_str_mv |
Elsevier |
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 |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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