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2020•Journal of the Korean Society of Pediatric NephrologyOpen access

Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab Treatment

Minseung Kim, Seon Hee Lim, Ji Hyun Kim, Il Soo Ha, Hae Il Cheong, Hee Gyung Kang

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Abstract

Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab TreatmentAtypical 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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Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab TreatmentAtypical 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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Available abstract

Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab TreatmentAtypical 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.

Key concepts: Eculizumab, Atypical hemolytic uremic syndrome, Complement system, Medicine, Immunology, Alternative complement pathway, Autoantibody, Shiga toxin

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