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doi: 10.1053/j.gastro.2004.04.063 23 GuidaCMcCullochLJGodazgarMStephenSDBakerCBascoDet al

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He hypothesized that lactic acidosis, increased concentration of Krebs cycle metabolites in urine, reduced levels of carnitine in plasma, decreased utilization of glucose in the brain, and lowered ATP levels in these patients are associated with mitochondrial dysfunction [66]