Table 2.
Complement assessment of all subjects of the family with hybrid gene
| Complement Parameters | II:1 (on Dialysis) | III:1 | II:2 | III:2 | |
|---|---|---|---|---|---|
| Before Eculizumab | After Eculizumab | ||||
| C3 (83–177 mg/dl) | 111 | 79 | 91 | n.a. | n.a. |
| C4 (15–45 mg/dl) | 26 | 19 | n.a. | n.a. | n.a. |
| CH50 (79–187 units Eq/ml) | n.a. | 226 | 5 | n.a. | n.a. |
| sC5b-9 (127–303 ng/ml) | 236 | 329 | 543 | 194 | 242 |
| FH (120–560 mg/L) | 164 | 242 | 156 | 136 | 133 |
| Ab anti-FH (AU/ml) | Absent | Absent | Absent | Absent | Absent |
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.