Clinical characteristics of children with haemophilia A treated at the Royal Hospital, Oman
| VARIABLE | TOTAL | MILD HAEMOPHILIA A | MODERATE HAEMOPHILIA A | SEVERE HAEMOPHILIA A |
|---|---|---|---|---|
| Number of persons, n (%) | 44 (100%) | 2 (4.5) | 29 (66) | 13 (29.5) |
| Presentation, n (%) | ||||
| Joint bleeding | 3 (6.8) | 0 (0) | 3 (10.3) | 0 (0) |
| Muscle bleeding | 13 (29.5) | 0 (0) | 10 (34.5) | 3 (23.1) |
| Mucosal bleeding | 4 (9.1) | 1 (50) | 3 (10.3) | 0 (0) |
| Screening with positive family history | 20 (45.5) | 0 (0) | 12 (41.4) | 8 (61.5) |
| Post-circumcision bleeding | 5 (11.4) | 1 (50) | 2 (6.9) | 2 (15.4) |
| Intracranial bleeding | 1 (2.3) | 0 (0) | 1 (3.4) | 0 (0) |
| Post-operative bleeding | 2 (4.5) | 0 (0) | 1 (3.4) | 1 (7.7) |
| Clinical profile, n (%) | ||||
| Joint involvement | 37 (84.1) | 1 (50) | 26 (89.7) | 10 (76.9) |
| At least one target joint | 10 (22.7) | 0 (0) | 7 (24) | 3 (23) |
| Muscle involvement | 17 (38.6) | 0 (0) | 12 (41.4) | 5 (38.5) |
| CNS involvement | 2 (4.5) | 0 (0) | 1 (3.4) | 1 (7.7) |
| Mucosal involvement | 5 (11.4) | 0 (0) | 5 (17.2) | 0 (0) |
| Treatment, n (%) | ||||
| On-demand | 16 (36.4) | 1 (50) | 10 (34.5) | 5 (38.5) |
| Prophylaxis | 28 (63.6) | 1 (50) | 19 (65.5) | 8 (61.5) |
| Other treatments | 5 (11.4) | 1 (50) | 2 (6.8) | 2 (15.4) |
| Complications, n (%) | ||||
| Inhibitor development | 6 (13.6) | 0 (0) | 3 (10.3) | 3 (23.1) |
| CNS complications | 1 (2.3) | 0 (0) | 1 (3.4) | 0 (0) |
| Joint arthropathy | 24 (54.5) | 0 (0) | 17 (58.6) | 7 (53.8) |
| ABR, mean ± SD | 4.4 ± 0.4 | 1.8 ± 2.3 | 4.6 ± 0.4 | 4.6 ± 8.6 |