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During periods of fasting, improper utilization of fatty acids impairs gluconeogenesis and characteristically leads to nonketotic or hypoketotic (no or minimal ketone body production respectively) hypoglycemia.[3] When fatty acid oxidation is impaired, glucose is readily consumed without replenishment from gluconeogenesis.[6] In carnitine deficiency states, various intermediary metabolic pathways such as Kreb's cycle, amino acid metabolism, and beta-oxidation of fatty acids are also affected