Síndrome hemolítico urémico atípico en tratamiento con eculizumab. Casos clínicos

Hemolytic uremic syndrome (HUS) is a type of thrombotic microangiopathy where organic damage predominates in the kidney. Atypical HUS (aHUS) is a rare disease that affects young adults and causes terminal chronic renal failure ending in dialysis, in most cases. It also recurs after kidney transplant...

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Autores principales:	Tagle,Rodrigo, Rivera,Gustavo, Walbaum,Benjamín, Sepúlveda,Rodrigo A.
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2018
Materias:	Antibodies Monoclonal Humanized Atypical Hemolytic Uremic Syndrome Thrombotic Microangiopathies
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000200254
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spelling	oai:scielo:S0034-988720180002002542018-06-20Síndrome hemolítico urémico atípico en tratamiento con eculizumab. Casos clínicosTagle,RodrigoRivera,GustavoWalbaum,BenjamínSepúlveda,Rodrigo A. Antibodies Monoclonal Humanized Atypical Hemolytic Uremic Syndrome Thrombotic Microangiopathies Hemolytic uremic syndrome (HUS) is a type of thrombotic microangiopathy where organic damage predominates in the kidney. Atypical HUS (aHUS) is a rare disease that affects young adults and causes terminal chronic renal failure ending in dialysis, in most cases. It also recurs after kidney transplantation. aHUS is associated with genetic defects of the alternative complement pathway or its activation by other factors such as drugs, autoimmune diseases, infections, malignant hypertension and ischemia-reperfusion. We report two women aged 17 and 25 years old with catastrophic aHUS. In both cases, complement amplifying factors (drugs and infections) were added and acted on a genetic vulnerability to precipitate complement activation and produce aHUS. Both patients developed terminal renal failure and had to undergo hemodialysis. Fortunately, after a broad etiological study, it was possible to make the diagnosis of aHUS and start treatment with Eculizumab, a monoclonal antibody that changed the natural history of aHUS. It inhibits complement activity controlling microangiopathy and preventing the development of end-stage renal disease. It also improves the success rate in kidney transplantation. In the case of our patients, both discontinued dialysis after chronic treatment with Eculizumab.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.146 n.2 20182018-02-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000200254es10.4067/s0034-98872018000200254
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Antibodies Monoclonal Humanized Atypical Hemolytic Uremic Syndrome Thrombotic Microangiopathies
spellingShingle	Antibodies Monoclonal Humanized Atypical Hemolytic Uremic Syndrome Thrombotic Microangiopathies Tagle,Rodrigo Rivera,Gustavo Walbaum,Benjamín Sepúlveda,Rodrigo A. Síndrome hemolítico urémico atípico en tratamiento con eculizumab. Casos clínicos
description	Hemolytic uremic syndrome (HUS) is a type of thrombotic microangiopathy where organic damage predominates in the kidney. Atypical HUS (aHUS) is a rare disease that affects young adults and causes terminal chronic renal failure ending in dialysis, in most cases. It also recurs after kidney transplantation. aHUS is associated with genetic defects of the alternative complement pathway or its activation by other factors such as drugs, autoimmune diseases, infections, malignant hypertension and ischemia-reperfusion. We report two women aged 17 and 25 years old with catastrophic aHUS. In both cases, complement amplifying factors (drugs and infections) were added and acted on a genetic vulnerability to precipitate complement activation and produce aHUS. Both patients developed terminal renal failure and had to undergo hemodialysis. Fortunately, after a broad etiological study, it was possible to make the diagnosis of aHUS and start treatment with Eculizumab, a monoclonal antibody that changed the natural history of aHUS. It inhibits complement activity controlling microangiopathy and preventing the development of end-stage renal disease. It also improves the success rate in kidney transplantation. In the case of our patients, both discontinued dialysis after chronic treatment with Eculizumab.
author	Tagle,Rodrigo Rivera,Gustavo Walbaum,Benjamín Sepúlveda,Rodrigo A.
author_facet	Tagle,Rodrigo Rivera,Gustavo Walbaum,Benjamín Sepúlveda,Rodrigo A.
author_sort	Tagle,Rodrigo
title	Síndrome hemolítico urémico atípico en tratamiento con eculizumab. Casos clínicos
title_short	Síndrome hemolítico urémico atípico en tratamiento con eculizumab. Casos clínicos
title_full	Síndrome hemolítico urémico atípico en tratamiento con eculizumab. Casos clínicos
title_fullStr	Síndrome hemolítico urémico atípico en tratamiento con eculizumab. Casos clínicos
title_full_unstemmed	Síndrome hemolítico urémico atípico en tratamiento con eculizumab. Casos clínicos
title_sort	síndrome hemolítico urémico atípico en tratamiento con eculizumab. casos clínicos
publisher	Sociedad Médica de Santiago
publishDate	2018
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000200254
work_keys_str_mv	AT taglerodrigo sindromehemoliticouremicoatipicoentratamientoconeculizumabcasosclinicos AT riveragustavo sindromehemoliticouremicoatipicoentratamientoconeculizumabcasosclinicos AT walbaumbenjamin sindromehemoliticouremicoatipicoentratamientoconeculizumabcasosclinicos AT sepulvedarodrigoa sindromehemoliticouremicoatipicoentratamientoconeculizumabcasosclinicos
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Síndrome hemolítico urémico atípico en tratamiento con eculizumab. Casos clínicos

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