Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab Treatment
10.3339/jkspn.2020.24.2.138
- Author:
Min Seung KIM
1
;
Seon Hee LIM
;
Ji Hyun KIM
;
Il-Soo HA
;
Hae Il CHEONG
;
Hee Gyung KANG
Author Information
1. Department of Pediatrics, Seoul National University Children's Hospital
- Publication Type:3
- From:Childhood Kidney Diseases
2020;24(2):138-142
- CountryRepublic of Korea
- Language:English
-
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.
