Abstract | BACKGROUND: OBJECTIVE: METHODS: Four children (2 males; 2 females) aged 9-18 y with phenylketonuria [mild hyperphenylalanemia (mHPA); 6-10 mg/dL (360-600 μmol/L)] were recruited to participate in ≥7 separate test protein intakes (range: 0.2-3.2 g ⋅ kg-1 ⋅ d-1) with the IAAO protocol with the use of l-[1-13C]Leu followed by the collection of breath and urine samples over 8 h. The diets were isocaloric and provided energy at 1.7 times the resting energy expenditure. Protein was provided as a crystalline amino acid mixture based on an egg protein pattern, except Phe and Leu, which were maintained at a constant across intakes. Protein requirement was determined with the use of a 2-phase linear-regression crossover analysis of the rate of l-[1-13C]Leu tracer oxidation. RESULTS: The mean protein requirement was determined to be 1.85 g ⋅ kg-1 ⋅ d-1 (R2 = 0.66; 95% CI: 1.37, 2.33). This result is substantially higher than the 2014 phenylketonuria recommendations (1.14-1.33 g ⋅ kg-1 ⋅ d-1; based on 120-140% above the current RDA for age). CONCLUSIONS: To our knowledge, this is the first study to directly define a quantitative requirement for protein intake in children with mHPA and indicates that current protein recommendations in children with phenylketonuria may be insufficient. This trial was registered at clinicaltrials.gov as NCT01965691.
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Authors | Abrar Turki, Keiko Ueda, Barbara Cheng, Alette Giezen, Ramona Salvarinova, Sylvia Stockler-Ipsiroglu, Rajavel Elango |
Journal | The Journal of nutrition
(J Nutr)
Vol. 147
Issue 2
Pg. 211-217
(02 2017)
ISSN: 1541-6100 [Electronic] United States |
PMID | 28053173
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Copyright | © 2017 American Society for Nutrition. |
Chemical References |
- Amino Acids
- Carbon Isotopes
- Dietary Proteins
- Leucine
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Topics |
- Adolescent
- Amino Acids
(metabolism)
- Carbon Isotopes
- Child
- Dietary Proteins
- Female
- Humans
- Isotope Labeling
- Leucine
(chemistry, metabolism)
- Male
- Nutritional Physiological Phenomena
- Nutritional Requirements
- Oxidation-Reduction
- Phenylketonurias
(metabolism)
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