Table 1.
Baseline demographics and disease characteristics.
| Variables | Values |
|---|---|
| Beginning year of follow-up,an (%) | |
| 1993-2006 (period A) | 129 (29.8) |
| 2007-2018 (period B) | 290 (67.0) |
| Unknown | 14 (3.2) |
| Sex, n (%) | |
| Male | 427 (98.6) |
| Female | 6 (1.4) |
| Age, mean ± SD | |
| 0-17 y (n = 173) | 9.7 ± 4.8 |
| 18-64 y (n = 246) | 32.6 ± 11.6 |
| ≥65 y (n = 10) | 73.6 ± 8.0 |
| Family history, n (%) | |
| Yes | 193 (44.6) |
| No | 93 (21.5) |
| Unknown | 147 (33.9) |
| Disease severity (factor IX level), n (%) | |
| Severe (<1 IU/dL) | 130 (30.0) |
| Moderate (1-5 IU/dL) | 212 (49.0) |
| Mild (>5 IU/dL) | 68 (15.7) |
| Unknown | 23 (5.3) |
| Inhibitor titer, n (%) | |
| LT <5 BU/mL | 12 (2.8) |
| HT ≥5 BU/mL | 7 (1.6) |
| No inhibitor detected | 382 (88.2) |
| Unknown | 32 (7.4) |
HT, high titer; LT, low titer.
a
Beginning year of follow-up of the patients at the reporting center was divided into 2 periods based on the accessibility to factor replacement therapy in Turkey.