Atypical hemolytic uremic syndrome responsive to steroids and intravenous immune globulin.

Atypical hemolytic uremic syndrome remains a challenge to diagnose and treat, with significant acute morbidity and risk for progression to end stage renal disease. Treatment strategies center on plasma exchange but do not necessarily affect the progression of disease. We report the case of a patient with atypical HUS resulting from a mutation in the complement pathway who responded to treatment with steroids and IVIG, therefore avoiding transfusion or plasma exchange. Copyright 2009 Wiley-Liss, Inc.