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Early treatment with eculizumab in atypical haemolytic uraemic syndrome
Atypical haemolytic uraemic syndrome (aHUS) is a rare and life-threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy. We report the case of an adult patient with plasmaphaeresis-resistant aHUS and hypertension treat...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4400458/ https://www.ncbi.nlm.nih.gov/pubmed/26069743 http://dx.doi.org/10.1093/ndtplus/sfr157 |
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author | Garjau, Maria Azancot, María Ramos, Rosa Sánchez-Corral, Pilar Montero, Maria Angeles Serón, Daniel |
author_facet | Garjau, Maria Azancot, María Ramos, Rosa Sánchez-Corral, Pilar Montero, Maria Angeles Serón, Daniel |
author_sort | Garjau, Maria |
collection | PubMed |
description | Atypical haemolytic uraemic syndrome (aHUS) is a rare and life-threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy. We report the case of an adult patient with plasmaphaeresis-resistant aHUS and hypertension treated with the complement inhibitor eculizumab. Eculizumab was shown to completely inhibit haemolysis, normalize thrombocyte levels and increase diuresis. Full recovery of renal function was not possible due to irreversible renal damage prior to eculizumab initiation. These findings highlight the importance of early treatment with eculizumab in patients with poor response to standard therapy, in order to avoid irreversible renal damage. |
format | Online Article Text |
id | pubmed-4400458 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-44004582015-06-11 Early treatment with eculizumab in atypical haemolytic uraemic syndrome Garjau, Maria Azancot, María Ramos, Rosa Sánchez-Corral, Pilar Montero, Maria Angeles Serón, Daniel Clin Kidney J Clinical Cases Atypical haemolytic uraemic syndrome (aHUS) is a rare and life-threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy. We report the case of an adult patient with plasmaphaeresis-resistant aHUS and hypertension treated with the complement inhibitor eculizumab. Eculizumab was shown to completely inhibit haemolysis, normalize thrombocyte levels and increase diuresis. Full recovery of renal function was not possible due to irreversible renal damage prior to eculizumab initiation. These findings highlight the importance of early treatment with eculizumab in patients with poor response to standard therapy, in order to avoid irreversible renal damage. Oxford University Press 2012-02 2012-01-31 /pmc/articles/PMC4400458/ /pubmed/26069743 http://dx.doi.org/10.1093/ndtplus/sfr157 Text en © The Author 2012. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | Clinical Cases Garjau, Maria Azancot, María Ramos, Rosa Sánchez-Corral, Pilar Montero, Maria Angeles Serón, Daniel Early treatment with eculizumab in atypical haemolytic uraemic syndrome |
title | Early treatment with eculizumab in atypical haemolytic uraemic syndrome |
title_full | Early treatment with eculizumab in atypical haemolytic uraemic syndrome |
title_fullStr | Early treatment with eculizumab in atypical haemolytic uraemic syndrome |
title_full_unstemmed | Early treatment with eculizumab in atypical haemolytic uraemic syndrome |
title_short | Early treatment with eculizumab in atypical haemolytic uraemic syndrome |
title_sort | early treatment with eculizumab in atypical haemolytic uraemic syndrome |
topic | Clinical Cases |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4400458/ https://www.ncbi.nlm.nih.gov/pubmed/26069743 http://dx.doi.org/10.1093/ndtplus/sfr157 |
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