From: Growth patterns in children with spinal muscular atrophy
 | SMA1 | SMA2 | ||||
---|---|---|---|---|---|---|
Male | Female | Total | Male | Female | Total | |
N = 58 (43.6%) | N = 75 (56.4%) | N = 133 | N = 42 (51.2%) | N = 40 (48.8%) | N = 82 | |
Demographic variables | Â | Â | Â | Â | Â | Â |
Age (years) | 0.7 (0.4; 2.1) | 0.6 (0.4; 1.4) | 0.6 (0.4; 1.6) | 4.3 (2.2; 7.0) | 3.9 (1.9; 6.7) | 4.1 (2.1; 6.7) |
Age classes | Â | Â | Â | Â | Â | Â |
 0–1 year | 35 (60.3%) | 51 (68.0%) | 86 (64.7%) | 1 (2.4%) | 0 (0.0%) | 1 (1.2%) |
 1–3 years | 14 (24.1%) | 10 (13.3%) | 24 (18.0%) | 12 (28.6%) | 15 (37.5%) | 27 (32.9%) |
 3–6 years | 4 (6.9%) | 8 (10.7%) | 12 (9.0%) | 15 (35.7%) | 13 (32.5%) | 28 (34.1%) |
 6–10 years | 3 (5.2%) | 4 (5.3%) | 7 (5.3%) | 9 (21.4%) | 10 (25.0%) | 18 (22.0%) |
 > 10 years | 2 (3.5%) | 2 (2.7%) | 4 (3.0%) | 5 (11.9%) | 2 (5.0%) | 8 (9.8%) |
Clinical variables | Â | Â | Â | Â | Â | Â |
Ventilation | Â | Â | Â | Â | Â | Â |
 Spontaneous breathing | 58 (63.8%) | 47 (62.7%) | 85 (63.3%) | 31 (73.8%) | 26 (65.0%) | 57 (69.5%) |
 Non invasive-ventilation | 13 (22.4%) | 23 (30.7%) | 36 (27.1%) | 11 (26.2%) | 14 (35.0%) | 25 (30.5%) |
 Tracheostomy | 8 (13.8%) | 5 (6.6%) | 13 (9.6%) | 0 (0.0%) | 0 (0.0%) | 0 (0.0%) |
 Total mechanical ventilation | 21 (36.2%) | 28 (37.3%) | 49 (36.7%) | 11 (26.2%) | 14 (35.0%) | 25 (30.5%) |
Feeding | Â | Â | Â | Â | Â | Â |
 By mouth | 48 (82.8%) | 57 (76.0%) | 105 (79.0%) | 42 (100.0%) | 40 (100.0%) | 82 (100.0%) |
 Nasogastric tube | 1 (1.7%) | 5 (6.7%) | 6 (4.5%) | 0 (0.0%) | 0 (0.0%) | 0 (0.0%) |
 Percutaneous endoscopic gastrostomy | 9 (15.5%) | 13 (17.3%) | 22 (16.5%) | 0 (0.0%) | 0 (0.0%) | 0 (0.0%) |
 Total artificial feeding | 10 (17.2%) | 18 (24.0%) | 28 (21.0%) | 0 (0.0%) | 0 (0.0%) | 0 (0.0%) |
Anthropometric variables | Â | Â | Â | Â | Â | Â |
Weight (kg) | 7.2 (6.2; 9.1) | 7.4 (6.2; 9.3) | 7.4 (6.2; 9.2) | 14.4 (11.5; 19.2) | 13.6 (10.4; 20.0) | 14.2 (10.7; 19.8) |
Weight z-score (WHO) | − 1.64 (− 2.39; − 0.69) | − 0.72 (− 1.73; − 0.05) | − 1.01 (− 2.17; − 0.12)*δ | − 1.04 (− 2.26; − 0.24) | − 1.06 (− 2.17; − 0.36) | − 1.04 (− 2.26; − 0.33)* |
Length (m) | 0.72 (0.67; 0.86) | 0.72 (0.66; 0.82) | 0.72 (0.66; 0.84) | 1.05 (0.89; 1.17) | 0.97 (0.84; 1.11) | 1.02 (0.87; 1.15) |
Length z-score (WHO) | 0.54 (− 0.28; 1.50) | 1.03 (− 0.17; 1.99) | 0.82 (− 0.15; 1.80) | − 0.17 (− 0.78;1.20) | − 0.62 (− 2.12; 0.69) | 0.41 (− 1.78; 0.83) |
BMI (kg/m2) | 13.4 (12.1; 14.9) | 13.9 (12.7; 14.9) | 13.7 (12.3; 14.9) | 14.1 (12.6; 15.9) | 14.1 (13.2; 15.9) | 14.1 (12.8; 15.9) |
BMI z-score (WHO) | − 2.61 (− 3.87; − 1.72) | − 2.22 (− 3.19; − 1.08) | − 2.48 (− 4.17; − 1.43)*δ | − 1.23 (− 2.91; − 0.35) | − 0.75 (− 1.93; − 0.45) | − 1.28 (− 2.61; − 0.13)* |