Abstract
BackgroundAtypical hemolytic uremic syndrome (aHUS) is a rare genetic disorder caused by chronic uncontrolled complement activation.Case-diagnosis/treatmentWe present a 4-year-old girl with aHUS who had multiple severe clinical manifestations of thrombotic microangiopathy (TMA) including acute kidney injury, dilated cardiomyopathy, and cardiorespiratory arrest. She was managed with intensive plasma exchange and hemodialysis, which could not halt the progression of TMA. The initial single dose of eculizumab only temporarily improved the clinical symptoms of TMA. Sustained improvement of renal, hematological, and cardiac values were only achieved upon institution of chronic treatment with eculizumab. During long-term treatment with eculizumab (>2.5 years), she has had no further clinical manifestations of TMA, and required neither plasma exchange nor hemodialysis.ConclusionChronic eculizumab treatment was associated with control of complement-mediated TMA and sustained long-term improvement in renal and cardiac function.
Highlights
Atypical hemolytic uremic syndrome is a rare genetic disorder caused by chronic uncontrolled complement activation [1, 2]
We report on a severely ill child with Atypical hemolytic uremic syndrome (aHUS) treated with eculizumab
This patient maintained an elevated lactate dehydrogenase (LDH), the level stabilized during eculizumab treatment
Summary
Atypical hemolytic uremic syndrome (aHUS) is a rare genetic disorder caused by chronic uncontrolled complement activation [1, 2].
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