Unlabelled: infection is a rare precipitant for patients to develop atypical hemolytic-uremic syndrome, of which the pathogenesis remains unclear. Previous reports suggest activation of cytokine storm from binding of cyotoxins A and B to colonic wall membranes.

Case Summary: We present a case of a previously healthy 21-year-old woman who developed fulminant colitis and atypical hemolytic-uremic syndrome requiring abdominal surgery and renal replacement therapy. She was ultimately treated with eculizumab without the use of plasmapheresis and remains in remission with full renal recovery.

Conclusions: Our patient's significant response to terminal complement inhibitor, without the use of plasmapheresis, suggests that the underlying pathology is significantly driven by the alternative complement pathway. We propose that -associated atypical hemolytic-uremic syndrome be defined as primary atypical hemolytic-uremic syndrome and strongly consider eculizumab as first-line therapy.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8280067PMC
http://dx.doi.org/10.1097/CCE.0000000000000475DOI Listing

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