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http://dx.doi.org/10.3345/kjp.2018.61.2.37

Atypical hemolytic uremic syndrome and eculizumab therapy in children  

Kim, Seong Heon (Department of Pediatrics, Pusan National University Children's Hospital)
Kim, Hye Young (Department of Pediatrics, Pusan National University Hospital)
Kim, Su Young (Department of Pediatrics, Pusan National University Children's Hospital)
Publication Information
Clinical and Experimental Pediatrics / v.61, no.2, 2018 , pp. 37-42 More about this Journal
Abstract
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.
Keywords
Atypical hemolytic uremic syndrome; Child; Eculizumab; Plasma therapy; Guideline;
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