Hemolytic-Uremic Syndrome Mediated by Complement: A Case Report

• Autor(a) principal: de Albuquerque Monteiro, Inês
• Data de Publicação: 2023
• Outros Autores: Fernandes Moura, Pedro, Pinho, Filipa, Gonçalves Pinto, Rita, Silveira, Pedro
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: https://doi.org/10.24950/rspmi.1927
• 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

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 diagnoses excluded, cHUS was assumed and treatment with eculizumab was initiated with sustained recovery.