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Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report

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Bibliographic Details
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.
Main Authors:de Albuquerque Monteiro, Inês
Other Authors:Fernandes Moura, Pedro; Pinho, Filipa; Gonçalves Pinto, Rita; Silveira, Pedro
Subject: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
Year:2024
Country:Portugal
Document type:article
Access type:unknown
Associated institution:Sociedade Portuguesa de Medicina Interna
Language:Portuguese
Origin:Revista Portuguesa de Medicina Interna
Description
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.