ABSTRACT: To the Editor: The hemolytic-uremic syndrome (HUS), a thrombotic microangiopathy, most commonly occurs secondary to infection with Shiga-toxin-producing Escherichia coli (STEC-HUS), although rare, atypical forms are associated with abnormalities in complement-regulating proteins. The inhibition of terminal complement complex formation by the monoclonal C5 antibody eculizumab has recently been reported as a treatment for atypical HUS.(1) We report on three 3-year-old patients with severe STEC-HUS that required hemodialysis. In Patient 1, plasma exchanges were performed because of low C3 and elevated C3d serum concentrations, which suggested complement activation. Plasma exchange was also performed in Patient 2 because of severe central nervous . . .
New England Journal of Medicine 05/2011; 364(26):2561-3. · 53.30 Impact Factor