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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Bibliographic Details
Published in:Childhood kidney diseases 2020, 24(2), , pp.138-142
Main Authors: Kim, Min Seung, Lim, Seon Hee, Kim, Ji Hyun, Ha, Il-Soo, Cheong, Hae Il, Kang, Hee Gyung
Format: Article
Language:English
Subjects:
atypical hemolytic uremic syndrome
complement factor h
eculizumab
소아과학
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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.
ISSN:2384-0242
2384-0250
DOI:10.3339/jkspn.2020.24.2.138