Síndrome hemolítico urémico atípico

Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy, characterized by microangiopathic hemolytic anemia, thrombocytopenia and renal involvement. It causes end stage renal disease requiring dialysis in most affected patients. It mainly affects young adults (contrary to what...

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Autores principales: Sepúlveda,Rodrigo A., Tagle,Rodrigo, Jara,Aquiles
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2018
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000600770
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spelling oai:scielo:S0034-988720180006007702018-08-24Síndrome hemolítico urémico atípicoSepúlveda,Rodrigo A.Tagle,RodrigoJara,Aquiles Atypical Hemolytic Uremic Syndrome Complement System Proteins Hemolytic-Uremic Syndrome Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy, characterized by microangiopathic hemolytic anemia, thrombocytopenia and renal involvement. It causes end stage renal disease requiring dialysis in most affected patients. It mainly affects young adults (contrary to what was thought years ago). When aHUS is primary, the cause is a genetic mutation in the alternative complement pathway. Instead, secondary aHUS is caused by external factors that trigger the disease by themselves or in combination with a genetic vulnerability. The type of mutation determines the severity of the disease, prognosis, response to therapy and renal transplantation. Advances in the understanding of renal diseases associated with complement defects and the development of specific biologic therapies changed the course of this disease. Eculizumab is internationally approved for the treatment of primary aHUS. Its inhibitory action on the complement cascade leads to hematologic remission and restoration of renal function. We present a review of aHUS detailing its etiology, pathogenesis, clinical presentation, diagnosis and treatment.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.146 n.6 20182018-06-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000600770es10.4067/s0034-98872018000600770
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic Atypical Hemolytic Uremic Syndrome
Complement System Proteins
Hemolytic-Uremic Syndrome
spellingShingle Atypical Hemolytic Uremic Syndrome
Complement System Proteins
Hemolytic-Uremic Syndrome
Sepúlveda,Rodrigo A.
Tagle,Rodrigo
Jara,Aquiles
Síndrome hemolítico urémico atípico
description Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy, characterized by microangiopathic hemolytic anemia, thrombocytopenia and renal involvement. It causes end stage renal disease requiring dialysis in most affected patients. It mainly affects young adults (contrary to what was thought years ago). When aHUS is primary, the cause is a genetic mutation in the alternative complement pathway. Instead, secondary aHUS is caused by external factors that trigger the disease by themselves or in combination with a genetic vulnerability. The type of mutation determines the severity of the disease, prognosis, response to therapy and renal transplantation. Advances in the understanding of renal diseases associated with complement defects and the development of specific biologic therapies changed the course of this disease. Eculizumab is internationally approved for the treatment of primary aHUS. Its inhibitory action on the complement cascade leads to hematologic remission and restoration of renal function. We present a review of aHUS detailing its etiology, pathogenesis, clinical presentation, diagnosis and treatment.
author Sepúlveda,Rodrigo A.
Tagle,Rodrigo
Jara,Aquiles
author_facet Sepúlveda,Rodrigo A.
Tagle,Rodrigo
Jara,Aquiles
author_sort Sepúlveda,Rodrigo A.
title Síndrome hemolítico urémico atípico
title_short Síndrome hemolítico urémico atípico
title_full Síndrome hemolítico urémico atípico
title_fullStr Síndrome hemolítico urémico atípico
title_full_unstemmed Síndrome hemolítico urémico atípico
title_sort síndrome hemolítico urémico atípico
publisher Sociedad Médica de Santiago
publishDate 2018
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000600770
work_keys_str_mv AT sepulvedarodrigoa sindromehemoliticouremicoatipico
AT taglerodrigo sindromehemoliticouremicoatipico
AT jaraaquiles sindromehemoliticouremicoatipico
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