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Change history 27 September 2021 A Correction to this paper has been published: Abbreviations AE: Adverse event KD: Ketogenic diet MeSH: Medical subject headings MD: Mitochondrial disease MAD: Modified Atkins diet OXPHOS: Oxidative phosphorylation PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-Analyses PDHC: Pyruvate Dehydrogenase Complex References Thompson K, Collier JJ, Glasgow RIC, Robertson FM, Pyle A, Blakely EL, et al