Table 2.
Characteristics of patient cohort during initial aHUS presentation, number of relapses, and renal outcome
| All patients N = 20 | Patients without long-term eculizumab N = 18 | |||
|---|---|---|---|---|
| No relapse N = 9 | Relapse N = 9 | No-relapse vs. relapse p value | ||
| Gender (male/female) | 15/5 | 5/4 | 9/0 | 0.04 |
| Age at the first episode (years) | 4.58 (2.8–7.5) | 6 (4–13.1) | 4.1 (2.5–6.0) | 0.054 |
| Follow-up time (years) | 2.6 (1.8–9.1) | 1.7 (0.4–2.6) | 9.5 (4.6–14.3) | < 0.001 |
| Age at last follow-up (years) | 12.29 (5.8–15.21) | 12.2 (5.3–14.1) | 14.0 (8.5–16.8) | 0.15 |
|
Time until first relapse Age at the first relapse (years) |
- | - |
3.75 (1.5–6.7) 7.5 (6.2–12.3) |
- |
| Number of relapses | - | |||
| 0 | 10 | 9 | 0 | |
| 1 | 3 | 0 | 2 | |
| 2 | 4 | 0 | 4 | |
| 3–6 | 1 | 0 | 1 | |
| 7–11 | 2 | 0 | 2 | |
| GFR (ml/min/1.73 m2) | ||||
| Lowest in the first episode | 22 (15–41) | 22 (10–34) | 39 (16–46) | 0.13 |
| Best after the first episode | 100 (70–114) | 106 (96–111) | 70 (69–131) | 0.50 |
| Lowest in the first relapse | - | - | 23 (15–41) | - |
| Best after the first relapse | - | - | 94 (80–120) | - |
| At last follow-up | 102 (85–124) | 105 (81–136) | 94 (84–121) | 0.25 |
| Characteristics of the first episode | ||||
| Received erythrocytes or platelets | 9 | 4 | 5 | - |
| Received plasma therapy/plasmapheresis | 4 | 2 | 1 | - |
| Received short-term eculizumab (overall) | 1 (3) | 1 | 0 (2) | - |
| Received long-term eculizumab (overall) | 2 (4) | 0 | 0 (2) | - |
| Days in hospital | 9.0 (7.2–14.5) | 9.0 (7.5–12) | 8.0 (6.0–13.0) | 0.38 |
Values are shown as n or median (interquartile range)
p values for two-sided Wilcoxon rank sum tests for continuous variables and Fisher’s exact test for categorical variables
n.a. not applicable