Open Access

Clinical profile and outcome of children with haemophilia A: The Royal Hospital, Oman’s experience

, , , ,  and   
Mar 19, 2025

Cite
Download Cover

A study of over 40 Omani children with haemophilia A notes a milder phenotype but similar clinical outcomes to other studies
A study of over 40 Omani children with haemophilia A notes a milder phenotype but similar clinical outcomes to other studies

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