Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report

Bibliographic Details
Main Author: de Albuquerque Monteiro, Inês
Publication Date: 2024
Other Authors: Fernandes Moura, Pedro, Pinho, Filipa, Gonçalves Pinto, Rita, Silveira, Pedro
Format: Article
Language: por
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: https://doi.org/10.24950/rspmi.1927
Summary: Complement-mediated hemolytic uremic syndrome (cHUS)is a rare syndrome that predominantly affects children andyoung adults and arises from defects in the regulation of the complement cascade. We present the case of a patient who presented with a clinical picture of nausea, vomiting, diffuse abdominal pain, diarrhea, and dark urine with three days of evolution. Analytically, there were signs of microangiopathic hemolytic anemia, thrombocytopenia, and renal injury, and the patient was admitted for study. Initially treated with plasmapheresis and corticosteroids, without clinical or analytical improvement. After an exhaustive study, and with more likely diagnosesexcluded, cHUS was assumed and treatment with eculizumab was initiated with sustained recovery.
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spelling Hemolytic-Uremic Syndrome Mediated by Complement: A Case ReportSíndrome Hemolítica-Urémica Mediada por Complemento: A Propósito de um Caso ClínicoAtivação do ComplementoInativadores do Complemento/uso terapêuticoProteínas do Sistema ComplementoSíndrome Hemolítica-Urémica AtípicaAtypical Hemolytic Uremic SyndromeComplement ActivationComplement Inactivating Agents/ therapeutic useComplement System ProteinsComplement-mediated hemolytic uremic syndrome (cHUS)is a rare syndrome that predominantly affects children andyoung adults and arises from defects in the regulation of the complement cascade. We present the case of a patient who presented with a clinical picture of nausea, vomiting, diffuse abdominal pain, diarrhea, and dark urine with three days of evolution. Analytically, there were signs of microangiopathic hemolytic anemia, thrombocytopenia, and renal injury, and the patient was admitted for study. Initially treated with plasmapheresis and corticosteroids, without clinical or analytical improvement. After an exhaustive study, and with more likely diagnosesexcluded, cHUS was assumed and treatment with eculizumab was initiated with sustained recovery.A síndrome hemolítica-urémica mediada por complemento(SHUc) é uma síndrome rara, que atinge predominantemente crianças e jovens adultos e surge por defeitos na regulação da cascata do complemento. Apresentamos o caso de uma doente que se apresenta com quadro de náuseas, vómitos, dor abdominal difusa, dejeções e colúria com três dias de evolução. Analiticamente com sinais de anemia hemolíticamicroangiopática, trombocitopenia e lesão renal, foi internada para estudo. Inicialmente sob terapêutica com permuta plasmática e corticoterapia, sem melhoria clínica ou analítica. Após estudo exaustivo, e excluídos diagnósticos mais prováveis, assumido diagnóstico de SHUc tendo iniciado terapêutica dirigida com eculizumab com recuperação sustentada.Sociedade Portuguesa de Medicina Interna2024-03-20info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://doi.org/10.24950/rspmi.1927https://doi.org/10.24950/rspmi.1927Internal Medicine; Vol. 31 No. 1 (2024): Janeiro/Março ; 23-26Medicina Interna; Vol. 31 N.º 1 (2024): Janeiro/Março ; 23-262183-99800872-671Xreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAPporhttps://revista.spmi.pt/index.php/rpmi/article/view/1927https://revista.spmi.pt/index.php/rpmi/article/view/1927/1857Direitos de Autor (c) 2024 Medicina Internainfo:eu-repo/semantics/openAccessde Albuquerque Monteiro, InêsFernandes Moura, PedroPinho, FilipaGonçalves Pinto, RitaSilveira, Pedro2024-05-18T07:28:54Zoai:oai.revista.spmi.pt:article/1927Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T11:13:34.604238Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report
Síndrome Hemolítica-Urémica Mediada por Complemento: A Propósito de um Caso Clínico
title Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report
spellingShingle Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report
de Albuquerque Monteiro, Inês
Ativação do Complemento
Inativadores do Complemento/uso terapêutico
Proteínas do Sistema Complemento
Síndrome Hemolítica-Urémica Atípica
Atypical Hemolytic Uremic Syndrome
Complement Activation
Complement Inactivating Agents/ therapeutic use
Complement System Proteins
title_short Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report
title_full Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report
title_fullStr Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report
title_full_unstemmed Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report
title_sort Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report
author de Albuquerque Monteiro, Inês
author_facet de Albuquerque Monteiro, Inês
Fernandes Moura, Pedro
Pinho, Filipa
Gonçalves Pinto, Rita
Silveira, Pedro
author_role author
author2 Fernandes Moura, Pedro
Pinho, Filipa
Gonçalves Pinto, Rita
Silveira, Pedro
author2_role author
author
author
author
dc.contributor.author.fl_str_mv de Albuquerque Monteiro, Inês
Fernandes Moura, Pedro
Pinho, Filipa
Gonçalves Pinto, Rita
Silveira, Pedro
dc.subject.por.fl_str_mv Ativação do Complemento
Inativadores do Complemento/uso terapêutico
Proteínas do Sistema Complemento
Síndrome Hemolítica-Urémica Atípica
Atypical Hemolytic Uremic Syndrome
Complement Activation
Complement Inactivating Agents/ therapeutic use
Complement System Proteins
topic Ativação do Complemento
Inativadores do Complemento/uso terapêutico
Proteínas do Sistema Complemento
Síndrome Hemolítica-Urémica Atípica
Atypical Hemolytic Uremic Syndrome
Complement Activation
Complement Inactivating Agents/ therapeutic use
Complement System Proteins
description Complement-mediated hemolytic uremic syndrome (cHUS)is a rare syndrome that predominantly affects children andyoung adults and arises from defects in the regulation of the complement cascade. We present the case of a patient who presented with a clinical picture of nausea, vomiting, diffuse abdominal pain, diarrhea, and dark urine with three days of evolution. Analytically, there were signs of microangiopathic hemolytic anemia, thrombocytopenia, and renal injury, and the patient was admitted for study. Initially treated with plasmapheresis and corticosteroids, without clinical or analytical improvement. After an exhaustive study, and with more likely diagnosesexcluded, cHUS was assumed and treatment with eculizumab was initiated with sustained recovery.
publishDate 2024
dc.date.none.fl_str_mv 2024-03-20
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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dc.identifier.uri.fl_str_mv https://doi.org/10.24950/rspmi.1927
https://doi.org/10.24950/rspmi.1927
url https://doi.org/10.24950/rspmi.1927
dc.language.iso.fl_str_mv por
language por
dc.relation.none.fl_str_mv https://revista.spmi.pt/index.php/rpmi/article/view/1927
https://revista.spmi.pt/index.php/rpmi/article/view/1927/1857
dc.rights.driver.fl_str_mv Direitos de Autor (c) 2024 Medicina Interna
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Direitos de Autor (c) 2024 Medicina Interna
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Sociedade Portuguesa de Medicina Interna
publisher.none.fl_str_mv Sociedade Portuguesa de Medicina Interna
dc.source.none.fl_str_mv Internal Medicine; Vol. 31 No. 1 (2024): Janeiro/Março ; 23-26
Medicina Interna; Vol. 31 N.º 1 (2024): Janeiro/Março ; 23-26
2183-9980
0872-671X
reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
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instname_str FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
instacron_str RCAAP
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reponame_str Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
collection Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
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repository.mail.fl_str_mv info@rcaap.pt
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