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Serum Carnitine Level in Phenylketonuric Children under Dietary Control in Greece

ABSTRACT. Although total, free and esterified carnitine blood levels were found to be low (p < 0.001) in phenylketonuric patients under dietary treatment compared to controls, no clinical signs of carnitine deficiency were noticed. Exclusion from the PKU diet of nutrients rich in carnitine has been suggested. Supplementation of the diets with carnitine or preferably enrichment of the PKU formulas with carnitine will rectify the restriction of extrinsic carnitine in PKU dietary treatment.