Pulmonary function studies
| Timing | Ventilatory function | ||||
|---|---|---|---|---|---|
| FVC, L | FEV1/FVC | TLC, L | RV, L | MVV, L/min | |
| Values in parentheses represent percent predicted of normal. | |||||
| FVC = forced vital capacity; FEV1 = forced expiratory volume in 1 sec; TLC = total lung capacity; RV = residual volume; MVV = minute ventilatory volume. | |||||
| At presentation | 1.8 (41) | 82 (104) | 3.05 (48) | 1.4 (88) | 61 (39) |
| 18 months later | 1.5 (35) | 84 (107) | 2.6 (43) | 1.07 (62) | 60 (47) |