PKU Protein Requirements

"NaH13CO3 \[99% atom percent excess (APE) and L-\[1-13C\]Leu (99% APE) will be given orally.~Isotope administration will begin with the fifth meal on each study day with each remaining meal.~The nonradioactive stable oral isotope is being administered to study the physiological process of amino acid oxidation."

"Test diets will be provided on study days in 8 hourly isocaloric and isonitrogenous meals to maintain a metabolic steady state.~Each meal will provide one-twelfth of the participant's daily needs to model a 12-hour fasted and 12-hour fed feeding pattern.~The diet will be composed of PFD2 (Mead Johnson), Tang and Kool-Aid (Kraft), corn oil, and protein-free wheat starch cookies. Each participant will receive 1 of 7 test protein intakes (0.2-3.2 g ⋅ kg-1 ⋅ d-1) on each study day. Protein will be provided as a crystalline L-amino acid mixture based on an egg protein pattern. Phe will be provided separately based on the Phe tolerance for each patient established by the metabolic dietitian listed on the IRB protocol. Leucine will be provided at a constant amount of 82.6 mg/kg/day as it is used as the indicator amino acid. The medical food used in this study is being fed to subjects for nutritional purposes to study the oxidation of the nonradioactive oral isotope."