Context: To date, several studies have been done regarding the treatment of atypical hemolytic uremic syndrome (aHUS) which discussed eculizumab as a potential treatment for this syndrome. However, the safety and efficacy of eculizumab were not fully assessed. This study aims to do a systematic review about the efficacy and safety of eculizumab in treatment of aHUS. Evidence Acquisitions: An electronic literature search was conducted to identify appropriate studies. We included all randomized

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... ials and observational studies about using eculizumab in aHUS. Two independent reviewers extracted data from the articles according to the selection criteria. Results: Eligible studies were included in this systematic review. The literature search and reference mining yielded 571 potential relevant articles. We removed 173 articles because of duplication. We also excluded 245 articles after reviewing the titles and abstracts, and removed 61 studies because the topics were not relevant to the subject. Finally, five studies were included in the systematic review. Conclusions: Acknowledging the limitations of the study due to the size and nature of the included studies, our systematic review shows that eculizumab was effective in the treatment of aHUS. However, further large randomized trials are suggested.