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http://dx.doi.org/10.3339/jkspn.2020.24.2.138

Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab Treatment  

Kim, Min Seung (Departments of Pediatrics, Seoul National University Children's Hospital)
Lim, Seon Hee (Departments of Pediatrics, Seoul National University Children's Hospital)
Kim, Ji Hyun (Department of Pediatrics, Seoul National University Bundang Hospital)
Ha, Il-Soo (Departments of Pediatrics, Seoul National University Children's Hospital)
Cheong, Hae Il (Departments of Pediatrics, Seoul National University Children's Hospital)
Kang, Hee Gyung (Departments of Pediatrics, Seoul National University Children's Hospital)
Publication Information
Childhood Kidney Diseases / v.24, no.2, 2020 , pp. 138-142 More about this Journal
Abstract
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare and life-threatening disorder. Typical HUS is often caused by Shiga toxin-positive Escherichia coli, while aHUS is caused by dysregulation of the alternative pathway of the complement system in association with genetic abnormalities or development of autoantibodies. Eculizumab, a humanized anti-complement 5 monoclonal antibody, is recommended for the treatment of aHUS, but its long-term safety and efficacy in pediatric patients remain under review. In this paper, we report a pediatric case of aHUS with anti-complement factor H autoantibodies, who was treated successfully with eculizumab.
Keywords
Atypical hemolytic uremic syndrome; Eculizumab; Complement factor H;
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Times Cited By KSCI : 2  (Citation Analysis)
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