Table 2.
Patient characteristics
| Characteristics | Data completeness, n (%) | |
|---|---|---|
| Sex, n (%) | 2700 (100%) | |
| Male | 1294 (47.9) | |
| Female | 1406 (52.1) | |
| Age at diagnosis, years, | 2700 (100%) | |
| Median (Min; Max) | 31.0 (4; 89) | |
| Mean (SD) | 31.4 (19.6) | |
| N (%) | ||
| 4–10 | 460 (17.0) | |
| 11–20 | 475 (17.6) | |
| 21–40 | 939 (34.8) | |
| 41–60 | 565 (21.0) | |
| > 60 | 261 (9.7) | |
| Age at onset, years, | 2236 (82.8%) | |
| Median (Min; Max) | 18.0 (0; 81) | |
| Mean (SD) | 22.4 (19.0) | |
| N (%) | ||
| ≤ 10 | 829 (37.1) | |
| 11–20 | 401 (17.9) | |
| 21–40 | 601 (26.9) | |
| 41–60 | 309 (13.8) | |
| > 60 | 96 (4.3) | |
| Diagnostic delay, years | 2236 (82.8%) | |
| Median (Min; Max) | 4.0 (0; 69) | |
| Mean (SD) | 8.8 (11.4) | |
| N (%) | ||
| 0 (diagnosis in the year of symptoms onset) | 357 (16.0) | |
| 1–4 | 769 (34.4) | |
| 5–9 | 443 (19.8) | |
| 10–20 | 380 (17.0) | |
| ≥ 21 | 287 (12.8) | |
| Period of diagnosis, n (%) | 2700 (100%) | |
| ≤ 1980 | 101 (3.7) | |
| 1980–1999 | 734 (27.2) | |
| ≥ 2000 | 1865 (69.1) | |
| Consanguinity, n (%) | 1488 (55.1%) | |
| With consanguinity | 68 (4.6) | |
| With records on immunoglobulin replacement therapy, n (%) | 2290 (84.8%) | |