Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome.
Diana Carolina Bello-MarquezJohn Fredy Nieto-RiosLina María Serna HiguitaAlfonso Jose Gonzalez-VergaraPublished in: Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia (2021)
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.