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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...

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Autores principales: Garjau, Maria, Azancot, María, Ramos, Rosa, Sánchez-Corral, Pilar, Montero, Maria Angeles, Serón, Daniel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2012
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.
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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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