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Table 1 Percentage of patients with decreased C3 plasma concentration in the various subgroups of atypical hemolytic uremic syndrome

From: Atypical hemolytic uremic syndrome

  CFH mutation CFI mutation MCP mutation C3 mutation CFB mutation THBD mutation Anti -CFH Ab None
Decreased C3 concentration
(< 2SD)
(% patients) 		30-50% 20-30% 0-27% 70-80% 100% 50% 40-60% up to 20%
  1. Normal C3 plasma concentration does not eliminate the presence of a mutation in the complement system or of anti- CFH antibodies. Conversely, decreased C3 level signs the presence of a complement abnormality.
  2. CFH: factor H; CFI: factor I; MCP: membrane cofactor protein; CFB: factor B; THBD: thrombomodulin; Ab, antibodies.
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172