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Atypical hemolytic uremic syndrome and eculizumab therapy in children

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating...

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Autores principales: Kim, Seong Heon, Kim, Hye Young, Kim, Su Young
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Pediatric Society 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5854840/
https://www.ncbi.nlm.nih.gov/pubmed/29563942
http://dx.doi.org/10.3345/kjp.2018.61.2.37
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author Kim, Seong Heon
Kim, Hye Young
Kim, Su Young
author_facet Kim, Seong Heon
Kim, Hye Young
Kim, Su Young
author_sort Kim, Seong Heon
collection PubMed
description Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy. Eculizumab is a newly developed biologic that blocks the terminal complement pathway and has been successfully used in the treatment of aHUS. Currently, several guidelines for aHUS, including the Korean guideline, recommend eculizumab as the first-line therapy in children with aHUS. Moreover, life-long eculizumab therapy is generally recommended. Further studies on discontinuation of eculizumab are needed.
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spelling pubmed-58548402018-03-21 Atypical hemolytic uremic syndrome and eculizumab therapy in children Kim, Seong Heon Kim, Hye Young Kim, Su Young Korean J Pediatr Review Article Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy. Eculizumab is a newly developed biologic that blocks the terminal complement pathway and has been successfully used in the treatment of aHUS. Currently, several guidelines for aHUS, including the Korean guideline, recommend eculizumab as the first-line therapy in children with aHUS. Moreover, life-long eculizumab therapy is generally recommended. Further studies on discontinuation of eculizumab are needed. The Korean Pediatric Society 2018-02 2018-02-28 /pmc/articles/PMC5854840/ /pubmed/29563942 http://dx.doi.org/10.3345/kjp.2018.61.2.37 Text en Copyright © 2018 by The Korean Pediatric Society 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 unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Kim, Seong Heon
Kim, Hye Young
Kim, Su Young
Atypical hemolytic uremic syndrome and eculizumab therapy in children
title Atypical hemolytic uremic syndrome and eculizumab therapy in children
title_full Atypical hemolytic uremic syndrome and eculizumab therapy in children
title_fullStr Atypical hemolytic uremic syndrome and eculizumab therapy in children
title_full_unstemmed Atypical hemolytic uremic syndrome and eculizumab therapy in children
title_short Atypical hemolytic uremic syndrome and eculizumab therapy in children
title_sort atypical hemolytic uremic syndrome and eculizumab therapy in children
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5854840/
https://www.ncbi.nlm.nih.gov/pubmed/29563942
http://dx.doi.org/10.3345/kjp.2018.61.2.37
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