Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report
Autor(a) principal: | |
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Data de Publicação: | 2019 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021 |
Resumo: | The atypical hemolytic uremic syndrome comprises a thrombotic microangiopathy resulting from the complement alternate pathway hyperactivation. Its severity requires early diagnosis and treatment. The differential diagnosis includes typical hemolytic uremic syndrome (associated with Shiga toxin) and thrombotic thrombocytopenic purpura (associated with deficient activity of ADAMTS13). The only specific treatment currently available for atypical hemolytic uremic syndrome is eculizumab. We describe the case of a child with atypical hemolytic uremic syndrome diagnosed in the context of bloody diarrhea, complicated by neurological involvement that posed several diagnostic and therapeutic challenges. |
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Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case ReportDesafios Diagnósticos e Terapêuticos na Síndrome Hemolítica Urémica Atípica: A Propósito de Um Caso ClínicoAtypical Hemolytic Uremic SyndromeEculizumabEculizumabSíndrome Hemolítica Urémica AtípicaThe atypical hemolytic uremic syndrome comprises a thrombotic microangiopathy resulting from the complement alternate pathway hyperactivation. Its severity requires early diagnosis and treatment. The differential diagnosis includes typical hemolytic uremic syndrome (associated with Shiga toxin) and thrombotic thrombocytopenic purpura (associated with deficient activity of ADAMTS13). The only specific treatment currently available for atypical hemolytic uremic syndrome is eculizumab. We describe the case of a child with atypical hemolytic uremic syndrome diagnosed in the context of bloody diarrhea, complicated by neurological involvement that posed several diagnostic and therapeutic challenges.A síndrome hemolítica urémica atípica constitui uma microangiopatia trombótica resultante da hiperativação da via alterna do complemento. A sua gravidade exige diagnóstico e terapêutica precoces. O diagnóstico diferencial inclui a síndrome hemolítica urémica típica (associada à toxina Shiga) e a púrpura trombótica trombocitopénica (associada a deficiência na atividade da ADAMTS13). A terapêutica específica da síndrome hemolítica urémica atípica, atualmente disponível, é o eculizumab. Descrevemos um caso clínico de uma criança com síndrome hemolítica urémica atípica diagnosticada em contexto de diarreia sanguinolenta, complicada de envolvimento neurológico e que colocou vários desafios no diagnóstico e decisões terapêuticas.Ordem dos Médicos2019-10-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfapplication/pdfapplication/pdfapplication/pdfhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021oai:ojs.www.actamedicaportuguesa.com:article/10021Acta Médica Portuguesa; Vol. 32 No. 10 (2019): October; 673-675Acta Médica Portuguesa; Vol. 32 N.º 10 (2019): Outubro; 673-6751646-07580870-399Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021/5775https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021/9879https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021/9880https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021/10882Direitos de Autor (c) 2019 Acta Médica Portuguesainfo:eu-repo/semantics/openAccessReis, SofiaRamos, DanielaCordinhã, CarolinaGomes, Clara2022-12-20T11:05:51Zoai:ojs.www.actamedicaportuguesa.com:article/10021Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:19:47.107855Repositório 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 |
Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report Desafios Diagnósticos e Terapêuticos na Síndrome Hemolítica Urémica Atípica: A Propósito de Um Caso Clínico |
title |
Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report |
spellingShingle |
Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report Reis, Sofia Atypical Hemolytic Uremic Syndrome Eculizumab Eculizumab Síndrome Hemolítica Urémica Atípica |
title_short |
Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report |
title_full |
Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report |
title_fullStr |
Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report |
title_full_unstemmed |
Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report |
title_sort |
Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report |
author |
Reis, Sofia |
author_facet |
Reis, Sofia Ramos, Daniela Cordinhã, Carolina Gomes, Clara |
author_role |
author |
author2 |
Ramos, Daniela Cordinhã, Carolina Gomes, Clara |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Reis, Sofia Ramos, Daniela Cordinhã, Carolina Gomes, Clara |
dc.subject.por.fl_str_mv |
Atypical Hemolytic Uremic Syndrome Eculizumab Eculizumab Síndrome Hemolítica Urémica Atípica |
topic |
Atypical Hemolytic Uremic Syndrome Eculizumab Eculizumab Síndrome Hemolítica Urémica Atípica |
description |
The atypical hemolytic uremic syndrome comprises a thrombotic microangiopathy resulting from the complement alternate pathway hyperactivation. Its severity requires early diagnosis and treatment. The differential diagnosis includes typical hemolytic uremic syndrome (associated with Shiga toxin) and thrombotic thrombocytopenic purpura (associated with deficient activity of ADAMTS13). The only specific treatment currently available for atypical hemolytic uremic syndrome is eculizumab. We describe the case of a child with atypical hemolytic uremic syndrome diagnosed in the context of bloody diarrhea, complicated by neurological involvement that posed several diagnostic and therapeutic challenges. |
publishDate |
2019 |
dc.date.none.fl_str_mv |
2019-10-01 |
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 |
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021 oai:ojs.www.actamedicaportuguesa.com:article/10021 |
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021 |
identifier_str_mv |
oai:ojs.www.actamedicaportuguesa.com:article/10021 |
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por |
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por |
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021 https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021/5775 https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021/9879 https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021/9880 https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10021/10882 |
dc.rights.driver.fl_str_mv |
Direitos de Autor (c) 2019 Acta Médica Portuguesa info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
Direitos de Autor (c) 2019 Acta Médica Portuguesa |
eu_rights_str_mv |
openAccess |
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application/pdf application/pdf application/pdf application/pdf |
dc.publisher.none.fl_str_mv |
Ordem dos Médicos |
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Ordem dos Médicos |
dc.source.none.fl_str_mv |
Acta Médica Portuguesa; Vol. 32 No. 10 (2019): October; 673-675 Acta Médica Portuguesa; Vol. 32 N.º 10 (2019): Outubro; 673-675 1646-0758 0870-399X 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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