| Ala | alanine |
| BHB | β-hydroxybutyric acid |
| CMHI- | the Children’s Memorial Health Institute |
| CNS | central nervous system |
| FGF21 | fibroblast growth factor 21 |
| IPMDS | International Pediatric Mitochondrial Disease Scale |
| MAD | modified Atkins diet |
| OXPHOS | oxidative phosphorylation process |
| PDHD | pyruvic acid dehydrogenase deficiency |
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