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Table 3 Main clinical characteristics of patients with atypical hemolytic uremic syndrome according to complement abnormality

From: Atypical hemolytic uremic syndrome

Gene or subgroup Frequency in aHUS Minimal age at onset Risk of death or ESRD at 1st episode or within < 1 y Risk of relapses Risk of recurrence after renal transplantation Plasma therapy indicated
   Children Adults     
CFH 20-30% Birth any age 50-70% 50% 75-90% Yes
CFI 4 -10% Birth any age 50% 10-30% 45-80% Yes
MCP 5 -15% > 1 y any age 0-6% 70-90% < 20% Questionable
C3 2 -10% 7 m any age 60% 50% 40-70% Yes
CFB 1-4% 1 m any age 50% 3/3 not in ESRD 100% Yes
THBD 3 -5% 6 m rare 50% 30% 1 patient Yes
Anti-CFH Ab 6% Mostly 7-11 y 30-40% 40-60% Yes if high Ab titer Yes (+ IS)
  1. CFH: factor H; CFI: factor I; MCP: membrane cofactor protein; CFB: factor B; THBD: thrombomodulin; Ab, antibodies; ESRD: end stage renal disease; IS: immunosuppressive treatment.