Table 1.
Open-label trial of HEW in 14 myopathic patients
| Progressive muscular dystrophy (PMD) | Polymyositis (PM)/Dermatomyositis (DM) | Mitochondrial myopathies (MM) | |||||||
|---|---|---|---|---|---|---|---|---|---|
| Before | 12 weeks | After | Before | 12 weeks | After | Before | 12 weeks | After | |
| CK (U/L) | 3067 ± 1492 | 3419 ± 1610 | 3107 ± 2382 | 124 ± 31 | 180 ± 97 | 140 ± 86 | 187 ± 75 | 124 ± 47 | 156 ± 40 |
| HbA1c (%) | 5.25 ± 0.44 | 5.14 ± 0.31 | 5.16 ± 0.42 | 6.68 ± 1.61 | 6.70 ± 1.53 | 6.90 ± 2.03 | 7.40 ± 1.70 | 7.32 ± 1.48 | 7.38 ± 1.74 |
| Fasting glucose (mmol/L) | 5.52 ± 0.16** | 5.51 ± 0.08** | 5.82 ± 0.11** | 7.66 ± 0.11 | 7.29 ± 1.57 | 7.69 ± 1.85 | 8.94 ± 3.24 | 9.31 ± 4.18 | 8.96 ± 3.19 |
| Lactate (mmol/L) |
0.95 ± 0.34 | 1.15 ± 0.40 | 1.35 ± 0.40 | 1.42 ± 0.18 | 1.66 ± 0.32 | 1.30 ± 0.27 | 1.84 ± 0.50 | 1.87 ± 0.78 | 1.73 ± 0.65 |
| L/P ratio |
12.1 ± 0.7* | 10.7 ± 1.3* | 13.6 ± 2.2* | 13.1 ± 0.9 | 15.0 ± 3.2 | 12.7 ± 1.0 | 20.7 ± 2.9* | 14.9 ± 3.5* | 20.3 ± 3.1* |
| Creatinine (μmol/L) | 34.8 ± 3.1 | 34.5 ± 6.9 | 34.7 ± 8.8 | 58.6 ± 13.7 | 56.3 ± 10.7 | 56.6 ± 14.1 | 48.8 ± 9.0 | 47.7 ± 9.7 | 48.6 ± 8.8 |
| BUN (mmol/L) | 4.74 ± 1.16 | 4.20 ± 0.60 | 4.21 ± 1.05 | 4.33 ± 0.71 | 4.11 ± 0.48 | 4.68 ± 0.74 | 5.28 ± 1.69 | 5.89 ± 1.09 | 5.00 ± 1.58 |
| Uric acid (μmol/dL) | 295 ± 46 | 315 ± 61 | 300 ± 30 | 319 ± 44 | 331 ± 71 | 329 ± 40 | 208 ± 50 | 220 ± 60 | 217 ± 45 |
| Urinary 8-isoprostane (ng/mg Cr) |
303 ± 155 | 392 ± 173 | n.d. | 222 ± 88 | 237 ± 86 | n.d. | 274 ± 117 | 261 ± 59 | n.d. |
| T-chol (mmol/L) | 5.42 ± 0.99 | 5.74 ± 1.02 | 5.70 ± 0.81 | 5.28 ± 0.31 | 5.55 ± 0.93 | 5.97 ± 1.35 | 4.52 ± 0.75 | 4.53 ± 0.34 | 4.42 ± 0.77 |
| LDL-chol (mmol/L) | 3.30 ± 1.05 | 2.86 ± 1.46 | 2.99 ± 1.15 | 2.66 ± 0.28 | 3.21 ± 0.99 | 3.13 ± 1.02 | 2.44 ± 0.43 | 2.27 ± 0.44 | 2.22 ± 0.45 |
| HDL-chol (mmol/L) | 1.62 ± 0.21 | 1.49 ± 0.19 | 1.62 ± 0.18 | 2.01 ± 0.73 | 1.99 ± 0.76 | 2.02 ± 0.67 | 1.14 ± 0.79 | 1.03 ± 0.72 | 1.04 ± 0.69 |
| Triglycerides (mmol/L) | 1.31 ± 0.46 | 3.62 ± 4.83 | 3.07 ± 3.67 | 2.17 ± 0.63* | 2.01 ± 1.21* | 3.09 ± 1.22* | 0.78 ± 0.34 | 0.89 ± 0.45 | 0.73 ± 0.37 |
| WBC (109/L) | 5.30 ± 1.32 | 5.40 ± 1.12 | 4.80 ± 0.38 | 10.78 ± 2.08 | 8.35 ± 3.41 | 10.10 ± 0.17 | 5.12 ± 1.27 | 7.27 ± 2.29 | 5.93 ± 1.35 |
| RBC (1012/L) | 4.17 ± 0.64 | 3.79 ± 0.42 | 3.87 ± 0.80 | 4.01 ± 0.62 | 4.34 ± 0.36 | 4.49 ± 0.45 | 4.33 ± 0.43 | 4.31 ± 0.78 | 4.35 ± 0.59 |
| Platelets (109/L) | 262 ± 42 | 260 ± 33 | 270 ± 20 | 337 ± 123 | 265 ± 82 | 270 ± 65 | 215 ± 25 | 207 ± 28 | 217 ± 25 |
| Hematocrit | 0.375 ± 0.040 | 0.374 ± 0.038 | 0.407 ± 0.055 | 0.337 ± 0.069 | 0.376 ± 0.041 | 0.395 ± 0.045 | 0.381 ± 0.036 | 0.370 ± 0.047 | 0.378 ± 0.038 |
| MMP3 (ng/ml) | n.d. | n.d. | n.d. | 307.8 ± 59.1* | 224.3 ± 53.4* | 283.3 ± 77.6* | n.d. | n.d. | n.d. |
| IgG (mg/dl) | n.d. | n.d. | n.d. | 1343 ± 470 | 1396 ± 550 | 1429 ± 581 | n.d. | n.d. | n.d. |
Values represent mean ± SD. n.d., not determined. *p < 0.05 and **p < 0.005 by one-way repeated measures ANOVA.