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Am J Physiol Endocrinol Metab 283: E1249-E1256, 2002. First published August 27, 2002; doi:10.1152/ajpendo.0319.2001
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Vol. 283, Issue 6, E1249-E1256, December 2002

Phenylalanine requirement in children with classical PKU determined by indicator amino acid oxidation

Glenda Courtney-Martin1,3, Rachelle Bross1,3, Mahroukh Raffi3, Joe T. R. Clarke2,3, Ronald O. Ball1,3,4, and Paul B. Pencharz1,2,3,4

Departments of 1 Nutritional Sciences and 2 Paediatrics, University of Toronto, Toronto M5S 3E2; 3 The Research Institute, The Hospital for Sick Children, Toronto, Ontario M5G 1X8; and 4 Department of Agricultural, Food and Nutritional Services, University of Alberta, Edmonton, Alberta, Canada T6G 2P5

Dietary restriction of phenylalanine is the main treatment for phenylketonuria (PKU), and current estimates of requirements are based on plasma phenylalanine concentration and growth. The present study aimed to determine more precisely the phenylalanine requirements in patients with the disease by use of indicator amino acid oxidation, with L-[1-13C]lysine as the indicator. Breath 13CO2 production (F13CO2) was used as the end point. Finger-prick blood samples were also collected for measurement of phenylalanine to relate phenylalanine intake to blood phenylalanine levels. The mean phenylalanine requirement, estimated using a two-phase linear regression crossover analysis, was 14 mg · kg-1 · day-1, and the safe population intake (upper 95% confidence interval of the mean) was found to be 19.5 mg · kg-1 · day-1. A balance between phenylalanine intake and the difference between fed and fasted blood phenylalanine concentration was observed at an intake of 20 mg · kg-1 · day-1. The similarity between these two values (19.5 and 20 mg · kg-1 · day-1) suggests that the maximal phenylalanine intake for children with PKU should be no higher than 20 mg · kg-1 · day-1.

phenylketonuria; amino acid requirements; lysine


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