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Clinical Presentation Lethargy nausea or vomiting hypoglycemia- with lack or only trace of urinary ketones hepatomegaly Reye like syndrome seizures coma near/rescued SIDS Affected infants and children usually present between 3 and 24 months of age particularly when being weaned from nighttime feeds but neonatal cases have been described and adults have become ill after severe exertion (e.g

doi: 10.1016/j.joca.2015.05.015 82 HonsawekSWilairatanaVUdomsinprasertWSinlapavilawanPJirathanathornnukulN

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Together, these studies suggest that RNS-altered mitochondrial targets leads to a loss of function

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