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Adequacy of Low-Phenylalanine Diet

Tullio Mereu, MD
Am J Dis Child. 1967;113(5):522-523. doi:10.1001/archpedi.1967.02090200054002.
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AN INCREASING number of phenylketonuric infants are being fed a low-phenylalanine diet with the aim of preventing the development of mental retardation. Severe phenylalanine deprivation may result from such a diet unless the serum phenylalanine level is closely monitored and necessary adjustments of intake are promptly made. The observed lag in weight gain and linear growth1,2 in phenylketonuric infants on a low-phenylalanine diet, should not be considered a "necessary evil" or a "minor price to pay," but rather should prompt the search for a potentially correctable dietary imbalance. The consequences of amino acid imbalance on animal growth have been discussed recently by Harper et al.3 If the dietary intake of an indispensable amino acid is maintained at a suboptimal level, any further administration of an amino acid mixture with exclusion of the limiting amino acid is bound to produce more marked signs of protein deficiency, such as growth


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