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Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome
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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Sociedade Brasileira de Nefrologia
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8428631/ https://www.ncbi.nlm.nih.gov/pubmed/32779691 http://dx.doi.org/10.1590/2175-8239-JBN-2020-0050 |
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author | Bello-Marquez, Diana Carolina Nieto-Rios, John Fredy Serna-Higuita, Lina Maria Gonzalez-Vergara, Alfonso Jose |
author_facet | Bello-Marquez, Diana Carolina Nieto-Rios, John Fredy Serna-Higuita, Lina Maria Gonzalez-Vergara, Alfonso Jose |
author_sort | Bello-Marquez, Diana Carolina |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-8428631 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Sociedade Brasileira de Nefrologia |
record_format | MEDLINE/PubMed |
spelling | pubmed-84286312021-09-16 Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome Bello-Marquez, Diana Carolina Nieto-Rios, John Fredy Serna-Higuita, Lina Maria Gonzalez-Vergara, Alfonso Jose J Bras Nefrol Case Report 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. Sociedade Brasileira de Nefrologia 2020-08-10 2021 /pmc/articles/PMC8428631/ /pubmed/32779691 http://dx.doi.org/10.1590/2175-8239-JBN-2020-0050 Text en https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Bello-Marquez, Diana Carolina Nieto-Rios, John Fredy Serna-Higuita, Lina Maria Gonzalez-Vergara, Alfonso Jose Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome |
title | Nephrotic syndrome associated with primary atypical hemolytic uremic
syndrome |
title_full | Nephrotic syndrome associated with primary atypical hemolytic uremic
syndrome |
title_fullStr | Nephrotic syndrome associated with primary atypical hemolytic uremic
syndrome |
title_full_unstemmed | Nephrotic syndrome associated with primary atypical hemolytic uremic
syndrome |
title_short | Nephrotic syndrome associated with primary atypical hemolytic uremic
syndrome |
title_sort | nephrotic syndrome associated with primary atypical hemolytic uremic
syndrome |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8428631/ https://www.ncbi.nlm.nih.gov/pubmed/32779691 http://dx.doi.org/10.1590/2175-8239-JBN-2020-0050 |
work_keys_str_mv | AT bellomarquezdianacarolina nephroticsyndromeassociatedwithprimaryatypicalhemolyticuremicsyndrome AT nietoriosjohnfredy nephroticsyndromeassociatedwithprimaryatypicalhemolyticuremicsyndrome AT sernahiguitalinamaria nephroticsyndromeassociatedwithprimaryatypicalhemolyticuremicsyndrome AT gonzalezvergaraalfonsojose nephroticsyndromeassociatedwithprimaryatypicalhemolyticuremicsyndrome |