Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype

M Noris, J Caprioli, E Bresin, C Mossali… - Clinical Journal of the …, 2010 - journals.lww.com
Background and objectives: Hemolytic uremic syndrome (HUS) is characterized by
microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Most
childhood cases are caused by Shiga toxin–producing bacteria. The other form, atypical
HUS (aHUS), accounts for 10% of cases and has a poor prognosis. Genetic complement
abnormalities have been found in aHUS.