Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab Treatment

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 d...

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Main Authors: Min Seung Kim (Author), Seon Hee Lim (Author), Ji Hyun Kim (Author), Il-Soo Ha (Author), Hae Il Cheong (Author), Hee Gyung Kang (Author)
Format: Book
Published: Korean Society of Pediatric Nephrology, 2020-10-01T00:00:00Z.
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Summary: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.
Item Description:2384-0242
2384-0250
10.3339/jkspn.2020.24.2.138