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: | , , , , , |
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Format: | Book |
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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. |
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Item Description: | 2384-0242 2384-0250 10.3339/jkspn.2020.24.2.138 |