Table 1.
The main results of spiroergometric exercise testing as well as the venous lactate and ammonia results associated with exercise testing in patients with Tarui disease (Tarui 1 and Tarui 2), patients with McArdle disease (McArdle 1 and 2) and the controls.
| Tarui 1 | Tarui 2 | McArdle 1 | McArdle 2 | Controls, N = 8, mean (SD) | |
|---|---|---|---|---|---|
| Gender (m/f) | M | F | M | M | M/F 6/2 |
| Age (y) | 58 | 59 | 35 | 20 | 42.1 (15.9) |
| Height (cm) | 177 | 155 | 174 | 163 | 176.4 (10.4) |
| Weight (kg) | 71 | 76 | 64 | 57 | 74.9 (11.4) |
| Heart rate maximum (1/s) | 173 | 155 | 182 | 176 | 176.0 (14.3) |
| Heart rate maximum percent of predicteda (%) | 98.3 | 88.3 | 96.8 | 90 | 92.6 (11.5) |
| Borg subjective scale 6–20 | 19 | 17 | 20 | 19 | 18.4 (1.3) |
| Breathing frequency (1/min) | 68 | 29 | 34 | 30 | 32.0 (12.0) |
| RQ max | 0.96 | 0.96 | 0.66 | 0.78 | 1.13 (0.07) |
| Wmax/3 min (maximal working capacity) (W) | 93 | 50 | 50 | 80 | 246.5 (102.2) |
| Wmax/3 min percent or predictedb (%) | 56 | 37 | 21.8 | 38 | 115.9 (30.8) |
| V’O2max (maximal oxygen uptake) (L/min) | 2.05 | 1.13 | 1.28 | 1.36 | 3.3 (1.36) |
| V’O2max% of predictedc (%) | 84.6 | 64.6 | 43 | 42 | 121.13 (37.2) |
| V’O2/kgmax (maximal oxygen uptake/weight) (ml/min/kg) | 28.8 | 14.9 | 20 | 23.9 | 43.5 (14.3) |
| V’O2/kg max% of predictedc (%) | 91 | 62 | 51 | 52 | 121.5 (30.2) |
| Wmax/V’O2max (%) | 13.1 | 12.7 | 11.2 | 13.3 | 21.2 (1.3) |
| Lactate at rest (mmol/l) | 1.1 | 1.3 | 0.8 | 1.1 | 1.3 (0.45) |
| Maximal lactate (mmol/l) | 3.2 | 2.4 | 0.9 | 1 | 11.7 (3.3) |
| Ammonia rest (μmol/l) | 36 | 32 | 14 | 54 | 20.4 (11.0) |
| Maximal ammonia (μmol/l) | 409 | 185 | 75 | 243 | 79.3 (33.7) |