From: Chest MRI to diagnose early diaphragmatic weakness in Pompe disease
 | Pompe patients with decreased spirometry results (FVC supine  < 80%) (n = 22) | Pompe patients with normal spirometry results (FVC supine  ≥ 80%) (n = 13) | Healthy controls (n = 18) | p value |
---|---|---|---|---|
Sex, number of males (%) | 11 (50%) | 7 (54%) | 8 (44%) | 0.869 |
Age, years | 45 ± 16 | 31 ± 14 | 43 ± 14 | 0.030 |
Height, cm | 178 ± 11 | 174 ± 9 | 178 ± 12 | 0.761 |
Weight, kg | 76 ± 16 | 70 ± 13 | 79 ± 14 | 0.291 |
BMI, kg/m2 | 24 ± 4 | 23 ± 4 | 25 ± 3 | 0.262 |
Disease duration, years | 16 ± 8 | 9 ± 9 | – | 0.018 |
Patients on ERT, n (%) | 18 (82%) | 6 (46%) | – | 0.028 |
Duration of ERT, years | 6 ± 5 | 6 ± 4 | – | 0.811 |
Non-invasive ventilation, n (%) | 1 (3%) | 0 (0%) | – | 0.435 |
FVC upright, % of predicted | 81 ± 11 | 97 ± 9 | 106 ± 8 |  < 0.001 |
FVC supine, % of predicted | 60 ± 12 | 94 ± 9 | 102 ± 8 |  < 0.001 |
Δ FVC, % of predicted | 21 ± 10 | 4 ± 4 | 4 ± 4 |  < 0.001 |
MIP, % of predicted | 72 ± 23 | 88 ± 28 | 106 ± 25 | 0.001 |
MEP, % of predicted | 80 ± 28 | 81 ± 28 | 112 ± 28 | 0.002 |