Table 2.

Complement assessment of all subjects of the family with hybrid gene

Complement ParametersII:1 (on Dialysis)III:1II:2III:2
Before EculizumabAfter Eculizumab
C3 (83–177 mg/dl)1117991n.a.n.a.
C4 (15–45 mg/dl)2619n.a.n.a.n.a.
CH50 (79–187 units Eq/ml)n.a.2265n.a.n.a.
sC5b-9 (127–303 ng/ml)236329543194242
FH (120–560 mg/L)164242156136133
Ab anti-FH (AU/ml)AbsentAbsentAbsentAbsentAbsent
  • The normal ranges were set as mean±2 SD of the values recorded in healthy subjects. The proband is subject II:1, his affected daughter is III:1, his wife is II:2, and the unaffected son is III:2. n.a., not available.