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Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome
Haemolytic–uraemic syndrome is a clinical syndrome characterized by thrombocytopaenia, non-autoimmune haemolytic anaemia and renal impairment. Pathological alterations in kidney samples show thrombotic microangiopathy. The underlying pathogenesis is endothelial cell injury with thrombotic occlusion...
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/PMC4400444/ https://www.ncbi.nlm.nih.gov/pubmed/26069742 http://dx.doi.org/10.1093/ndtplus/sfr107 |
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author | Durán, Carlos E. Blasco, Miquel Maduell, Francisco Campistol, Josep M. |
author_facet | Durán, Carlos E. Blasco, Miquel Maduell, Francisco Campistol, Josep M. |
author_sort | Durán, Carlos E. |
collection | PubMed |
description | Haemolytic–uraemic syndrome is a clinical syndrome characterized by thrombocytopaenia, non-autoimmune haemolytic anaemia and renal impairment. Pathological alterations in kidney samples show thrombotic microangiopathy. The underlying pathogenesis is endothelial cell injury with thrombotic occlusion of the arterioles and capillaries. A variety of causes have been identified, associated with infection of Escherichia coli O157:H7, environmental factors as immunosuppressive drugs and genetic deficiencies in complement regulatory factors. The latter is called atypical haemolytic–uraemic syndrome (aHUS). Here, we present a patient with severe aHUS with complement factor H deficiency triggered by cocaine use and recurrence after kidney transplantation. The patient restarted haemodialysis for severe renal insufficiency and anti-C5 antibody eculizumab was used as salvage treatment with progressive recovery of graft function and suppression of dialysis. |
format | Online Article Text |
id | pubmed-4400444 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-44004442015-06-11 Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome Durán, Carlos E. Blasco, Miquel Maduell, Francisco Campistol, Josep M. Clin Kidney J Clinical Cases Haemolytic–uraemic syndrome is a clinical syndrome characterized by thrombocytopaenia, non-autoimmune haemolytic anaemia and renal impairment. Pathological alterations in kidney samples show thrombotic microangiopathy. The underlying pathogenesis is endothelial cell injury with thrombotic occlusion of the arterioles and capillaries. A variety of causes have been identified, associated with infection of Escherichia coli O157:H7, environmental factors as immunosuppressive drugs and genetic deficiencies in complement regulatory factors. The latter is called atypical haemolytic–uraemic syndrome (aHUS). Here, we present a patient with severe aHUS with complement factor H deficiency triggered by cocaine use and recurrence after kidney transplantation. The patient restarted haemodialysis for severe renal insufficiency and anti-C5 antibody eculizumab was used as salvage treatment with progressive recovery of graft function and suppression of dialysis. Oxford University Press 2012-02 2012-01-28 /pmc/articles/PMC4400444/ /pubmed/26069742 http://dx.doi.org/10.1093/ndtplus/sfr107 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 Durán, Carlos E. Blasco, Miquel Maduell, Francisco Campistol, Josep M. Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome |
title | Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome |
title_full | Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome |
title_fullStr | Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome |
title_full_unstemmed | Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome |
title_short | Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome |
title_sort | rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome |
topic | Clinical Cases |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4400444/ https://www.ncbi.nlm.nih.gov/pubmed/26069742 http://dx.doi.org/10.1093/ndtplus/sfr107 |
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