Abstract
and the IQ in the offspring; there was a significant correlation between the mother's phenylalanine concentration and the child's IQ. Our patient's outcome, in comparison with the natural history of untreated PKU patients and with the outcome of his untreated sister, strongly suggests that tyrosine supplementation had little if any beneficial effect. We conclude that a tyrosine intake of 1 mmol/kg/day did not prevent the development of the typical neurologic sequelae of classical phenylketonuria. REFERENCES
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