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Am J Physiol Endocrinol Metab (August 27, 2002). doi:10.1152/ajpendo.0319.2001
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Articles in PresS, published online ahead of print August 27, 2002
Am J Physiol Endocrinol Metab, 10.1152/ajpendo.0319.2001
Submitted on July 24, 2001
Accepted on August 14, 2002

Phenylalanine Requirement in Children with Classical Phenylketonuria Determined by Indicator Amino Acid Oxidation

Glenda Courtney-Martin1, Rachelle Bross1, Mahroukh Rafii2, Joe T. R Clarke3, Ronald O Ball4, and Paul B Pencharz5*

1 Department of Nutritional Sciences, University of Toronto, Toronto, Ontario, Canada; Gastroenterology and Nutrition, The Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada
2 Gastroenterology and Nutrition, The Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Nutritional Sciences, University of Toronto, Toronto, Ontario, Canada
3 Department of Paediatrics, University of Toronto, Toronto, Ontario, Canada; Gastroenterology and Nutrition, The Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada
4 Department of Nutritional Sciences, University of Toronto, Toronto, Ontario, Canada; Gastroenterology and Nutrition, The Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Agricultural, Food & Nutritional Services, University of Alberta, Edmonton, Alberta, Canada
5 Department of Nutritional Sciences, University of Toronto, Toronto, Ontario, Canada; Department of Paediatrics, University of Toronto, Toronto, Ontario, Canada; Gastroenterology and Nutrition, The Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada

* To whom correspondence should be addressed. E-mail: paul.pencharz{at}sickkids.on.ca.

Dietary restriction of phenylalanine is the main treatment for Phenylketonuria (PKU) and current estimates of requirements are based on plsma phenylalanine concentration and growth. The present study is to determine more precisely phenylalanine requirements in patients with the disease using Indicator Amino Acid Oxidation (IAAO) with L- [1-13C]lysine as the indicator. Breath 13CO2 production (F13 CO2) was used as the endpoint. Finger-prick blood samples were also collected, for measurment of phenylalanine, in order to relate phenylalanine intake to blood phenylalanine level. The mean phenylalanine requirement estimated using a two-phase linear regression crossover analysis,was 14mg.kg-1 day-1 and the safe population intake (upper 95% confidence interval of the mean), was found to be 19.5mg.kg-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. The similarity between these two values (19.5 and 20 mg.kg-1 d-1) suggests that the maximal phenylalanine intake for children with PKU should be no higher than 20 mg.kg-1 d-1.




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