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Pediatr Int. 2015 Apr;57(2):313-7. doi: 10.1111/ped.12469.

Autoimmune-type atypical hemolytic uremic syndrome treated with eculizumab as first-line therapy.

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Department of Nephrology, Chiba Children's Hospital, Chiba, Japan.


We report a case of atypical hemolytic uremic syndrome (aHUS) in a 4-year-old boy. Although the patient had the typical triad of aHUS (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury), urgent dialysis was not indicated because he had neither oliguria nor severe electrolyte abnormality. He was given eculizumab as first-line therapy, which led to significant clinical improvement, thus avoiding any risk of complications associated with plasma exchange and central venous catheterization. Retrograde functional analysis of the patient's plasma using sheep erythrocytes indicated an increase in hemolysis, suggesting impairment of host cell protection by complement factor H. The use of eculizumab as first-line therapy in place of plasma exchange might be reasonable for pediatric patients with aHUS.


atypical hemolytic uremic syndrome; eculizumab; plasma exchange

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