Child Kidney Dis.
2020 Oct;24(2):138-142. 10.3339/jkspn.2020.24.2.138.
Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab Treatment
- Affiliations
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- 1Department of Pediatrics, Seoul National University Children's Hospital
- 2Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea
- 3Department of Pediatrics, Seoul National University College of Medicine
- KMID: 2508553
- DOI: http://doi.org/10.3339/jkspn.2020.24.2.138
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.
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