Table 2.
Demographic and clinical characteristics
| Characteristics | All patients (N = 49) | Adult patients (n = 40) | Pediatric patients (n = 9) |
|---|---|---|---|
| Sex, female, n (%) | 36 (73) | 29 (73) | 7 (78) |
| Age at ravulizumab initiation, years, median (range) | 35 (2–72) | 37 (19–72) | 8 (2–15) |
| Geographic location, n (%) | |||
| Germany | 26 (53) | 22 (55) | 4 (44) |
| USA | 7 (14) | 4 (10) | 3 (33) |
| Denmark | 6 (12) | 5 (13) | 1 (11) |
| UK | 5 (10) | 5 (13) | 0 |
| Spain | 3 (6) | 3 (8) | 0 |
| Israel | 2 (4) | 1 (3) | 1 (11) |
| Extrarenal manifestations at any time, n (%) | |||
| Gastrointestinal | 22 (45) | 19 (48) | 3 (33) |
| Central nervous system | 17 (35) | 14 (35) | 3 (33) |
| Cardiovascular | 15 (31) | 11 (28) | 4 (44) |
| Pulmonary | 6 (12) | 4 (10) | 2 (22) |
| Family history of aHUS, n (%) | |||
| Yes | 9 (18) | 6 (15) | 3 (33) |
| Unknown | 7 (14) | 7 (18) | 0 |
| Missing | 1 (2) | 1 (3) | 0 |
| Time from aHUS onset to eculizumab initiation, median (range), mo | 1 (0–304) | 2 (0–304) | <1 (<1–28) |
| Eculizumab treatment duration, median (range), mo | 66 (11–155) | 66 (11–155) | 62 (15–89) |
| Ravulizumab treatment duration, median (range), mo | 23 (3–41) | 23 (3–40) | 20 (7–41) |
aHUS, atypical hemolytic uremic syndrome.