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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