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Table 3 Genetic and autoimmune complement abnormalities in patients in the study

From: Outcomes in patients with atypical hemolytic uremic syndrome treated with eculizumab in a long-term observational study

Complement Abnormality by Risk Level,a n (%) Never Discontinued (n = 51) Discontinued (n = 42)
Reinitiated (n = 21) Not Reinitiated (n = 21)
High risk 25 (49) 8 (38) 6 (29)
CFH 14 (27)b 6 (29) 3 (14)c
C3 6 (12) 1 (5) 1 (5)d
CFH autoantibodies 5 (10) 1 (5) 1 (5)
CFB 0 (0) 0 (0) 1 (5)
Low/moderate risk 6 (12) 6 (29) 3 (14)
CD46 (MCP) 3 (6)e 3 (14) 2 (10)
CFI 3 (6)f 3 (14) 1 (5)
Deletions 0 (0) 0 (0) 1 (5)
 CFHR1, CHFR3 0 (0) 0 (0) 1 (5)
No identified abnormality 20 (39) 7 (33) 11 (52)
  1. CFB complement factor B; CFH complement factor H; CFHR1, CFHR3 complement factor H–related protein 1, complement factor H–related protein 3; CFI complement factor I; MCP membrane cofactor protein
  2. aRisk stratification as proposed by Goodship et al. [17]
  3. bIncludes one patient who also had a CFI mutation, and one patient who also had a CD46 (MCP) mutation
  4. cIncludes one patient who also had a C3 mutation
  5. dExcludes one patient who also had a CFH mutation
  6. eIncludes one patient who also had a CFI mutation. Excludes one patient who also had a CFH mutation
  7. fExcludes two patients who also had CFH or CD46 mutations