Atypical hemolytic uremic syndrome (aHUS) is a genetic, life-threatening, chronic disease that can affect patients of all ages. aHUS is caused by uncontrolled complement activation due to genetic defects of complement regulation. Plasma exchange or infusion has been used to manage aHUS and may transiently maintain hematologic variables in some patients, but as the underlying complement dysregulation persists, end-stage renal disease or death occurs in 33% to 40% of patients during the first clinical manifestation. Here we present a pediatric case showing that first-line eculizumab treatment successfully blocked the progression of thrombotic microangiopathy in aHUS.
Eculizumab as First-Line Therapy for Atypical Hemolytic Uremic Syndrome
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
- Views Icon Views
- Share Icon Share
- Search Site
Martin Christmann, Matthias Hansen, Carsten Bergmann, Dirk Schwabe, Jörg Brand, Wilfried Schneider; Eculizumab as First-Line Therapy for Atypical Hemolytic Uremic Syndrome. Pediatrics June 2014; 133 (6): e1759–e1763. 10.1542/peds.2013-1787
Download citation file: