PMID: 3745744Sep 1, 1986Paper

Experience with adolescents with phenylketonuria returned to phenylalanine-restricted diets

Journal of the American Dietetic Association
S E HoganJ T Clarke

Abstract

In order to test the feasibility of returning older children with classical phenylketonuria (PKU) to therapeutically and nutritionally adequate phenylalanine-restricted diets after 8 to 13 years of unrestricted diets, 7 adolescents (13 to 19 years old) of normal or near-normal intelligence with classical PKU were returned to phenylalanine-limited diets for periods of 8 to 10 weeks. During a 4- to 5-week period when the phenylalanine-restricted diet was supplemented with added L-phenylalanine to mimic pretreatment conditions, plasma phenylalanine levels were 1,327 +/- 282 microM on total phenylalanine intakes of 2,794 +/- 248 mg/day (55.3 +/- 11.5 mg/kg/day). During a similar period of dietary phenylalanine restriction, plasma phenylalanine levels were successfully maintained at 713 +/- 266 microM on dietary intakes of 655 +/- 210 mg/day (12.6 +/- 4.3 mg/kg/day). On the basis of 3-day diet records, the intakes of total protein, energy, calcium, phosphorus, iron, vitamins D, E, and A, ascorbic acid, thiamin, riboflavin, niacin, folacin, and vitamin B-12 over the entire 8- to 10-week study period were adequate by the standards of the Recommended Nutrient Intakes (RNI) for Canada, where the study was undertaken. Intakes of magnesiu...Continue Reading

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