Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report

Detalhes bibliográficos
Autor(a) principal: Reis, Sofia
Data de Publicação: 2019
Outros Autores: Ramos, Daniela, Cordinhã, Carolina, Gomes, Clara
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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spelling 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
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dc.type.driver.fl_str_mv info:eu-repo/semantics/article
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url 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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dc.relation.none.fl_str_mv 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
dc.format.none.fl_str_mv application/pdf
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dc.publisher.none.fl_str_mv Ordem dos Médicos
publisher.none.fl_str_mv 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
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