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Although there are a number of potential exacerbating factors that may have contributed to the mental status changes observed in this patient, including thiamine deficiency, micronutrient insufficiencies (e.g., zinc), and occult liver dysfunction, the manifestation of refractory hyperammonemia in the setting of low-serum carnitine, free carnitine, and acylcarnitine ester levels, in conjunction with the temporal response to l-carnitine administration, support our contention that carnitine deficiency was the primary abnormality

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