Clinical details of the aHUS patients with factor H autoantibodies
Patient ID . | Age at presentation, y . | Sex . | Clinical outcome . | Length of follow-up, y . | Transplanted . | C3, g/L . | C4, g/L . | Factor H, g/L . | Factor I, mg/L . |
---|---|---|---|---|---|---|---|---|---|
1 | 5 | M | Recovered renal function. 2 relapses. On PE 2×/week | 2 | No | 0.61 | 0.1 | 0.37 | 39 |
2 | 11 | M | Recovered renal function | 5 | No | 0.57 | 0.15 | 0.44 | 70 |
3 | 8 | F | ESRF | 9 | No | 0.86 | 0.25 | 0.49 | 58 |
4 | 4 | F | ESRF | 1 | No | 0.89 | 0.3 | 0.52 | 41 |
5 | 10 | F | ESRF | 5 | Yes, 3 y no recurrence | 0.92 | 0.2 | 0.29 | n/a |
6 | 8 | M | ESRF | 6 | No | 1.08 | 0.36 | 0.5 | 74 |
7 | 6 | M | ESRF | 7 | Yes, 2 years no recurrence | 0.77 | 0.28 | 0.56 | 77 |
8 | 1 | M | Recovered renal function | 4 | No | 1.59 | 0.38 | 0.67 | 75 |
9 | 9 | F | Recovered renal function | 11 | No | n/a | n/a | 0.63 | n/a |
10 | 5 | M | Recovered renal function; multiple relapses; PE 1×/2 weeks | 3 | No | 1.06 | 0.13 | 0.45 | 68 |
11 | 4 | F | Recovered renal function; 3 relapses treated with PE | 6 | No | 0.85 | 0.24 | n/a | n/a |
12 | 10 | F | ESRF | 8 | Yes, 6 y no recurrence | 0.51 | 0.23 | 0.57 | 62 |
13 | 9 | F | Recovered renal function | 8 | No | n/a | n/a | n/a | n/a |
Patient ID . | Age at presentation, y . | Sex . | Clinical outcome . | Length of follow-up, y . | Transplanted . | C3, g/L . | C4, g/L . | Factor H, g/L . | Factor I, mg/L . |
---|---|---|---|---|---|---|---|---|---|
1 | 5 | M | Recovered renal function. 2 relapses. On PE 2×/week | 2 | No | 0.61 | 0.1 | 0.37 | 39 |
2 | 11 | M | Recovered renal function | 5 | No | 0.57 | 0.15 | 0.44 | 70 |
3 | 8 | F | ESRF | 9 | No | 0.86 | 0.25 | 0.49 | 58 |
4 | 4 | F | ESRF | 1 | No | 0.89 | 0.3 | 0.52 | 41 |
5 | 10 | F | ESRF | 5 | Yes, 3 y no recurrence | 0.92 | 0.2 | 0.29 | n/a |
6 | 8 | M | ESRF | 6 | No | 1.08 | 0.36 | 0.5 | 74 |
7 | 6 | M | ESRF | 7 | Yes, 2 years no recurrence | 0.77 | 0.28 | 0.56 | 77 |
8 | 1 | M | Recovered renal function | 4 | No | 1.59 | 0.38 | 0.67 | 75 |
9 | 9 | F | Recovered renal function | 11 | No | n/a | n/a | 0.63 | n/a |
10 | 5 | M | Recovered renal function; multiple relapses; PE 1×/2 weeks | 3 | No | 1.06 | 0.13 | 0.45 | 68 |
11 | 4 | F | Recovered renal function; 3 relapses treated with PE | 6 | No | 0.85 | 0.24 | n/a | n/a |
12 | 10 | F | ESRF | 8 | Yes, 6 y no recurrence | 0.51 | 0.23 | 0.57 | 62 |
13 | 9 | F | Recovered renal function | 8 | No | n/a | n/a | n/a | n/a |
aHUS indicates atypical hemolytic uremic syndrome; ESRF, end-stage renal failure; F, female; M, male; n/a, not available; and PE, plasma exchange.