Summary of cohorts
| . | HGDS (n = 265) . | HIGS (n = 448) . | MIBS (n = 120) . |
|---|---|---|---|
| With inhibitor ≥ 1 BU, n (%) | 52 (20%) | 360 (80%) | 45 (38%) |
| Race, n (%)* | |||
| Asian | 0 (0%) | 10 (2%) | 0 (0%) |
| Black | 24 (9%) | 21 (5%) | 3 (3%) |
| Hispanic | 42 (16%) | 50 (11%) | 0 (0%) |
| White | 195 (74%) | 347 (77%) | 114 (95%) |
| Other | 4 (2%) | 20 (4%) | 2 (2%) |
| Region, n (%) | |||
| Europe | 0 (0%) | 276 (62%) | 112 (93%) |
| North America | 265 (100%) | 134 (30%) | 8 (7%) |
| Latin America | 0 (0%) | 26 (6%) | 0 (0%) |
| South Africa | 0 (0%) | 12 (3%) | 0 (0%) |
| Hemophilia severity, n (%) | |||
| Mild | 16 (6%) | 0 (0%) | 14 (12%) |
| Moderate | 50 (19%) | 0 (0%) | 19 (16%) |
| Severe | 199 (75%) | 448 (100%) | 87 (72%) |
| Mutation type, n (%)† | |||
| Inversion | 98 (39%) | 253 (59%) | 52 (43%) |
| Large deletion | 5 (2%) | 31 (7%) | 3 (3%) |
| Missense mutation | 41 (16%) | 23 (5%) | 38 (32%) |
| Nonsense mutation | 9 (4%) | 52 (12%) | 1 (1%) |
| Small deletion/insertion | 10 (4%) | 57 (13%) | 20 (17%) |
| Splice site mutation | 3 (1%) | 8 (2%) | 2 (2%) |
| No mutation found | 3 (1%) | 4 (3%) | |
| Other | 3 (1%) | 2 (0%) | |
| No inversion | 84 (33%) | ||
| Year of birth‡ | 1977 (1974-1980) | 1996 (1988-2001) | 1981 (1964-1990) |
| Discordant brother pairs, n | 4 | 77 | 23 |
| . | HGDS (n = 265) . | HIGS (n = 448) . | MIBS (n = 120) . |
|---|---|---|---|
| With inhibitor ≥ 1 BU, n (%) | 52 (20%) | 360 (80%) | 45 (38%) |
| Race, n (%)* | |||
| Asian | 0 (0%) | 10 (2%) | 0 (0%) |
| Black | 24 (9%) | 21 (5%) | 3 (3%) |
| Hispanic | 42 (16%) | 50 (11%) | 0 (0%) |
| White | 195 (74%) | 347 (77%) | 114 (95%) |
| Other | 4 (2%) | 20 (4%) | 2 (2%) |
| Region, n (%) | |||
| Europe | 0 (0%) | 276 (62%) | 112 (93%) |
| North America | 265 (100%) | 134 (30%) | 8 (7%) |
| Latin America | 0 (0%) | 26 (6%) | 0 (0%) |
| South Africa | 0 (0%) | 12 (3%) | 0 (0%) |
| Hemophilia severity, n (%) | |||
| Mild | 16 (6%) | 0 (0%) | 14 (12%) |
| Moderate | 50 (19%) | 0 (0%) | 19 (16%) |
| Severe | 199 (75%) | 448 (100%) | 87 (72%) |
| Mutation type, n (%)† | |||
| Inversion | 98 (39%) | 253 (59%) | 52 (43%) |
| Large deletion | 5 (2%) | 31 (7%) | 3 (3%) |
| Missense mutation | 41 (16%) | 23 (5%) | 38 (32%) |
| Nonsense mutation | 9 (4%) | 52 (12%) | 1 (1%) |
| Small deletion/insertion | 10 (4%) | 57 (13%) | 20 (17%) |
| Splice site mutation | 3 (1%) | 8 (2%) | 2 (2%) |
| No mutation found | 3 (1%) | 4 (3%) | |
| Other | 3 (1%) | 2 (0%) | |
| No inversion | 84 (33%) | ||
| Year of birth‡ | 1977 (1974-1980) | 1996 (1988-2001) | 1981 (1964-1990) |
| Discordant brother pairs, n | 4 | 77 | 23 |