Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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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)
  • Received : 2020.08.20
  • Accepted : 2020.09.29
  • Published : 2020.10.31
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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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References

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