| M694V/M694V (n = 17) | M694V/Other (n = 72) | Other/Other (n = 232) | P-value |
Age of onset (years), mean ± SD | 3.9 ± 2.7 | 3.7 ± 3.5 | 4.9 ± 3.7 | 0.041 |
Age at diagnosis (years), mean ± SD | 6.4 ± 3.7 | 6.618 ± 4.4 | 7.2 ± 4.2 | 0.426 |
Delay in diagnosis (years), mean ± SD | 2.5 ± 2.3 | 2.9 ± 2 | 2.5 ± 2 | 0.592 |
Abdominal pain | 15 (88.2%) | 61 (84.7%) | 196 (84.5%) | 0.917 |
Fever | 15 (88.2%) | 64 (88.9%) | 172 (74.1%) | 0.018 |
Chest pain | 7 (41.2%) | 29 (40.3%) | 62 (26.7%) | 0.057 |
Arthralgia | 12 (70.6%) | 34 (47.2%) | 92 (39.7%) | 0.032 |
>3 hospitalizations prior to diagnosis | 3 (17.6%) | 1 (1.4%) | 13 (5.6%) | 0.015 |
Response to colchicine |
|
|
| 0.069 |
No response | 2 (11.8%) | 7 (9.7%) | 47 (20.3%) |
|
Incomplete response | 11 (64.7%) | 36 (50%) | 90 (38.8%) |
|
Complete response | 4 (23.5%) | 9 (40.3%) | 95 (40.9%) |
|
Severity score |
|
|
| 0.008 |
Mild | 3 (17.6%) | 22 (30.5%) | 107 (46.1%) |
|
Moderate and severe | 14 (82.4%) | 50 (69.5%) | 125 (53.9%) |
|