TABLE 1.
Demographic information
| Total | FVIII deficiency | FIX deficiency | |
|---|---|---|---|
| n | 49 (100) | 35 (71) | 14 (29) |
| Age (years) | 44 (32, 52) | 39 (29, 50) | 48 (33, 56) |
| Age groups | |||
| 18–32 years | 13 (26) | 11 (85) | 2 (15) |
| 33–52 years | 24 (49) | 18 (75) | 6 (25) |
| 53–71 years | 12 (25) | 6 (50) | 6 (50) |
| Severity | |||
| Severe | 43 (88) | 30 (70) | 13 (30) |
| Moderate | 6 (12) | 5 (83) | 1 (17) |
| Treatment history | |||
| Prophylaxis | 43 (88) | 30 (70) | 13 (30) |
| On demand | 6 (12) | 5 (83) | 1 (17) |
| Age prophylaxis commenced (years) a | 26 (13, 49) | 23 (12, 42) | 41 (17, 52) |
| Age prophylaxis commenced by age group | |||
| <3 years | 5 (13) | 4 (80) | 1 (20) |
| 3–17 years | 9 (24) | 7 (78) | 2 (22) |
| ≥18 years | 24 (63) | 15 (63) | 9 (37) |
| BMI (kg/m2) | 27.0 (24.8, 29.6) | 26.8 (24.1, 30.6) | 28.0 (24.8, 29.2) |
| HJHS b | |||
| Total | 28 (20, 36) | 29 (20, 38) | 24 (20, 35) |
| Left ankle | 5 (2, 8) | 5 (2, 7) | 6 (4, 8) |
| Right ankle | 7 (4, 8) | 6 (4, 7) | 7 (6, 9) |
| Left knee | 1 (0, 5) | 1 (0, 5) | 1 (1, 7) |
| Right knee | 1 (0, 3) | 1 (0, 3) | 1 (1, 4) |
| Left elbow | 3 (0, 7) | 4 (0, 7) | 1 (0, 4) |
| Right elbow | 4 (0, 8) | 4 (0, 8) | 4 (0, 9) |
| Global gait score | 4 (4, 4) | 4 (4, 4) | 4 (4, 4) |
| HJHS by age category b | |||
| 18–32 years | 20 (4, 23) | 21 (7, 26) | 1 c |
| 33–52 years | 27 (21, 38) | 31 (21, 41) | 22 (18, 23) |
| 53–71 years | 34 (32, 46) | 32 (32, 40) | 35 (32, 53) |
| History of inhibitors | |||
| Inactive | 6 (12) | 5 (83) | 1 (17) |
| No history | 43 (88) | 30 (70) | 13 (30) |
Data are presented as median (IQR‐ Q1, Q3); Categorical variables are presented as n (%).
Abbreviations: BMI, body mass index; HJHS, haemophilia joint health score.
a
n = 38 (FVIII = 26; FIX = 12, remaining did not answer).
b
n = 44 (no HJHS available for 5 participants with moderate haemophilia).
c
n = 1, thus raw value is reported.