Table 2.
Pulmonary function tests
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | |
|---|---|---|---|---|---|
| VC max (L) | 4.01 | 3.79 | 1.83 | 1.5 | 2.01 |
| VC max (%) | 91 | 78 | 46 | 40 | 61 |
| FEV1 (L) | 1.32 | 2.33 | 1.36 | 0.55 | 1.18 |
| FEV1 (%) | 38 | 62 | 43 | 18 | 41 |
| Tiffeneau (FEV1/VC) (%) | 33 | 61 | 74 | 37 | 59 |
| TLC-He (L) | 5.52 | 5.38 | 2.84 | 3.6 | 2.94 |
| TLC-He (%) | 84 | 77 | 49 | 71 | 66 |
| TLC-Pleth | 3.50 | ||||
| TLC-Pleth (%) | 60 | ||||
| DLCO (%) | 73 | 94 | 46 | 44 | 43 |
| DLCO/VA(%) | 108 | 140 | 132 | 109 | 129 |
| Age at time of start ventilatory support | 50 | N/A | 40 | 31 | N/A |
| Type of ventilatory support | CPAP | N/A | BiPAP | CPAP | N/A |
VC vital capacity (in litre and as % of predicted), FEV1 forced expiratory volume in 1 s (in litre and as % of predicted), TLC-He total lung capacity measured by helium dilution (in litre and as % of predicted), TLC-Pleth total lung capacity measured by bodyplethysmography (in liter and as % of predicted), DLCO carbon monoxide diffusing capacity (% of predicted), DLCO/VA carbon monoxide diffusing capacity adjusted for lung volume (% of predicted). CPAP continuous positive airway pressure, BiPAP bilevel positive airway pressure