Table 1.
Demographic Characteristics of the sample.
| Age (years) | Gender | BMI (kg/cm2) | FVC pred (%) | FEV1 Pred (%) | PIMAX (cmH2O) | PEMAX (cmH2O) | ETCO2 (mm Hg) | VE (L/min) | Age at Diagnosis (years) | Use of ERT | Nighttime Support? | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Pompe Subjects | ||||||||||||
| P1 | 50 | F | 27.12 | 69.7 | 69 | 49 | 62 | 34 | 9.7 | 42 | yes | NO |
| P2 | 47 | F | 25.4 | 67 | 66.2 | 69 | 79 | 45 | 9.9 | 37 | yes | CPAP |
| P3 | 52 | M | 26.4 | 36.7 | 35.9 | 36 | 88 | 48 | 10.2 | 40 | no | BiPAP |
| P4 | 32 | M | 26.58 | 70.8 | 73.9 | 85 | 139 | 41 | 12.1 | 30 | yes | NO |
| P5 | 32 | F | 25.08 | 95 | 86.6 | 79 | 67 | 38 | 12.7 | 32 | no | NO |
| P6 | 29 | F | 16.22 | 29.2 | 31.1 | 36 | 46 | 48 | 10.4 | 25 | yes | BiPAP |
| P7 | 63 | F | 21.48 | 51.4 | 60.5 | 56 | 59 | 44 | 12.5 | 59 | yes | BiPAP |
| Average | 43.6 (±12.8) | 24.0 (±3.9) | 60.0 (±22.6) | 60.5 (±20.2) | 58.6 (±19.8) | 77.1 (±30.5) | 42.6 (±5.2) | 11.1 (±1.3) | 37.8 (±11.0) | |||
| Control Subjects | ||||||||||||
| C1 | 55 | F | 35.47 | 99.7 | 109.6 | 83 | 125 | 45 | 7.3 | — | — | — |
| C2 | 49 | F | 24.86 | 133.2 | 119.6 | 118 | 116 | 41 | 9.1 | — | — | — |
| C3 | 53 | M | 32.09 | 75 | 77.9 | 61 | 100 | 40 | 11.7 | — | — | — |
| C4 | 29 | M | 26.18 | 141.6 | 106.5 | 156 | 115 | 39 | 11.1 | — | — | — |
| C5 | 31 | F | 22.72 | 112.5 | 121 | 78 | 96 | 40 | 9.7 | — | — | — |
| C6 | 20 | F | 23.32 | 117.7 | 121 | 88 | 102 | 37 | 10.4 | — | — | — |
| C7 | 61 | F | 23.16 | 95.3 | 102.9 | 83 | 125 | 36 | 7.2 | — | — | — |
| Average | 42.6 (±15.7) | 26.8 (±5.0) | 110.7 (±22.9) | 108.4 (±15.3) | 95.3 (±31.7) | 111.3 (±12.0) | 39.7 (±2.9) | 9.5 (±1.8) | — | — | — | |
BiPAP: bi-level positive airway pressure; BMI: body mass index; CPAP: continuous positive airway pressure; ERT: enzyme replacement therapy; ETCO2: end-tidal carbon dioxide; FEV1: forced expiratory volume in first second; FVC: forced vital capacity; PIMAX: maximal inspiratory pressure; PEMAX: maximal expiratory pressure; VE: minute ventilation.