Table 3.
The outcomes of the included studies.
| Study | Primary outcomes |
Secondary outcomes |
Other qualitative outcomes |
||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Plasma lactate level |
Plasma pyruvate level |
L/P ratio (<25.6) |
JMDRS |
NMDAS/NPMDSa |
Functional improvements | ||||||
| Before | After | Before | After | Before | After | Before | After | Before | After | ||
| Koga et al.36b | 50.5 ± 12.5 mg/dl | 30.4 ± 5.08 mg/dlf | 1.9 ± 0.5 mg/dl | 1.5 ± 0.3 mg/dl | 28.1 ± 8.6 | 20.7 ± 3.5c | 24.1 ± 19.7 | 21.0 ± 22.4 | 38.6 ± 28.7 | 33.3 ± 30.7 | Significant decrease from the baseline values in serum GDF15 level and lactate cerebral ventricular levels |
| Fujii et al.37d | 1.2 mM | 0.85 mM | NA | NA | 19.7 | 20 | NA | NA | 42.3 | 38.6 | Able to roll over and raise the leg 90° |
| 2.8 mM | 2.4 mM | NA | NA | 23.2 | 23.1 | 52 | 53 | 34.2 | 53.7 | Able to roll over and full oral feeding | |
| 3.9 mM | 5.6 mM | NA | NA | 25 | 30.5 | NA | NA | 44.7 | 28.3 | Able to roll over bilaterally and dysphagia disappearede | |
| 2.3 mM | 2.5 mM | NA | NA | 16.9 | 17.3 | NA | NA | 35 | 64.8 | Mild improvement in the movement of extremitiesf | |
| Komaki et al.23 | 20.5 mg/dl | 10.3 mg/dl | 1.13 mg/dl | 0.88 mg/dl | 18.1 | 11.7 | NA | NA | NA | NA | capable of running; improvement in exercise intolerance and cardiac dysfunction |
| Koga et al.24 | 9.6 ± 0.54 mM | 5.28 ± 1.73 mM | 0.69 ± 0.13 mM | 0.42 ± 0.13mM | 14.5 ± 3.10 | 12.6 ± 1.52 | 58 | 57 | NA | NA | significantly decreased level of alanine improvement in the electroencephalogram |
| Saito et al.38 | 2.3 mM | 2.3 mM | NA | NA | 18 | 18 | NA | NA | 35 | 31 | Able to raise her forearm, lower leg and wrist against gravity |
| Inoue et al.39 | 1.86 mM | 2.94 mM | 0.12 mM | 0.26 mM | 15.4 | 11.3 | 23 | 26 | NA | NA | The improvement of diabetes parameters |
This scale has four age group classifications: 0–24 months, 2–11 years, and 12–18 years from NPMDS, and an adult age group classification from NMDAS.
In addition, a significant decrease from the baseline values in GDF-15 was reported. The JMDRS overall scores did not change significantly from the baseline values at weeks 48 of pyruvate therapy, although the JMDRS scores decreased significantly from the baseline values at weeks 12 of pyruvate therapy.
Significance between none and pyruvate therapy.
The report in detail monitored four bedridden patients with OXPHOS disorders in their treatment process.
The patient gained the ability to roll over bilaterally and the dysphagia disappeared after 2 months of pyruvate therapy, but a slow regression in motor function was observed over next 10 months.
In the included study, two units were used for lactic acid and pyruvate concentration: mg/dl and mM.
JMDRS, Japanese Mitochondrial Disease-Rating Scale; L/P lactate/pyruvate; NMDAS, Newcastle Pediatric Mitochondrial Disease Scale; NPMDS, Newcastle Pediatric Mitochondrial Disease Scale.