The present invention provides a biomarker protein whose alteration of its concentration or activity level is associated with clinically significant treatment of aHUS using atypical hemolytic uremic syndrome (aHUS) or complement inhibitors. Compositions and methods for examining the concentration and / or activity of one or more biomarker proteins in a biological fluid are also provided. The compositions and methods are particularly useful for evaluating the risk of aHUS and / or for diagnosing aHUS and / or for determining whether the subject is experiencing the first acute manifestation of aHUS and / or monitoring progression or alleviation of aHUS and / Are useful for monitoring the response to treatment with an inhibitor or for optimizing such treatment.
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