Disease manifestation a , n (%) | Severe (n = 36) | Attenuated (n = 16) | Total (N = 52) |
---|---|---|---|
Hearing loss | 28 (75.7) | 13 (86.7) | 41 (78.8) |
Disabling joint stiffness | 25 (69.4) | 15 (93.8) | 40 (76.9) |
Hernias | 24 (66.7) | 13 (81.3) | 37 (71.2) |
Adenoidectomy | 22 (61.1) | 13 (81.3) | 38 (67.3) |
Mental retardation | 24 (66.7) | 4 (25.0) | 28 (53.8) |
Spine deformities | 17 (45.9) | 9 (60.0) | 26 (50.0) |
Tonsillectomy | 16 (44.4) | 9 (56.3) | 25 (48.1) |
Recurrent otitis | 17 (45.9) | 7 (46.7) | 24 (46.2) |
Loss of vision | 11 (30.6) | 8 (50.0) | 19 (36.5) |
Sleep apnoea | 13 (36.1) | 5 (31.3) | 18 (34.6) |
Skin involvement | 11 (30.6) | 4 (25.0) | 15 (28.8) |
Other serious clinical eventsb | 10 (27.0) | 3 (20.0) | 13 (25.0) |
Breathing difficulties with hospitalisation | 9 (25.0) | 4 (25.0) | 13 (25.0) |
Stay in intensive care unitc | 5 (13.9) | 3 (18.8) | 8 (15.4) |
Aspiration requiring hospitalisation | 2 (5.6) | 1 (6.3) | 3 (5.8) |
Spinal cord compression | 1 (2.7) | 1 (6.3) | 2 (3.8) |
Heart failure with hospitalisation | 1 (2.7) | 1 (6.7) | 2 (3.8) |