Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome

Abstract Primary atypical hemolytic-uremic syndrome is a rare disease characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction; it is related to alterations in the regulation of the alternative pathway of complement due to genetic mutations. The associat...

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Bibliographic Details
Published inBrazilian Journal of Nephrology Vol. 43; no. 3; pp. 440 - 444
Main Authors Bello-Marquez, Diana Carolina, Nieto-Rios, John Fredy, Serna-Higuita, Lina Maria, Gonzalez-Vergara, Alfonso Jose
Format Journal Article
LanguageEnglish
Published Sociedade Brasileira de Nefrologia 01.01.2021
Subjects
Acute Renal Injury
Atypical Hemolytic Uremic Syndrome
Case Report
Complement System Proteins
Hypertension
Nephrotic Syndrome
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Summary:Abstract Primary atypical hemolytic-uremic syndrome is a rare disease characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction; it is related to alterations in the regulation of the alternative pathway of complement due to genetic mutations. The association with nephrotic syndrome is unusual. We present here a pediatric patient diagnosed with primary atypical hemolytic-uremic syndrome associated with nephrotic syndrome who responded to eculizumab treatment. Resumo A síndrome hemolítico-urêmica atípica primária é uma doença rara, caracterizada por anemia hemolítica microangiopática não-imune, trombocitopenia e disfunção renal; está relacionado a alterações na regulação da via alternativa do complemento devido a mutações genéticas. A associação com a síndrome nefrótica é incomum. Apresentamos aqui um paciente pediátrico com diagnóstico de síndrome hemolítico-urêmica atípica primária associada à síndrome nefrótica que respondeu ao tratamento com eculizumab.
Bibliography:Author’s Contribution
Diana Carolina Bello-Marquez, John Fredy Nieto-Rios, Lina Maria Serna-Higuita, Alfonso Jose Gonzalez-Vergara contributed substantially to the conception or design of the study; collection, analysis, or interpretation of data; writing or critical review of the manuscript; and final approval of the version to be published.
Conflict of Interest
The authors John Fredy Nieto-Ríos and Diana Carolina Bello-Marquez declare that they have given talks about thrombotic microangiopathies sponsored by Alexion Pharma. All other authors declare that they have no conflicts of interest.
ISSN:0101-2800
2175-8239
DOI:10.1590/2175-8239-jbn-2020-0050