Protein Requirements in Children with Phenylketonuria (PKU)
Bibliographic record
Abstract
Phenylketonuria (PKU) is an inherited inborn error of phenylalanine (PHE) metabolism caused by deficiency of the hepatic enzyme, phenylalanine hydroxyls (PAH). Therefore PHE accumulates in plasma leading to developmental delay. The major mode of treatment is nutritional management with dietary restriction of PHE and provision of sufficient protein. The dietary protein requirement in children with PKU is unknown, and currently predicted by a factorial calculation.Our objective was to determine the protein requirements in children with PKU using the indicator amino acid oxidation (IAAO) technique. Four PKU children (5-18y) have been recruited to participate in test protein intakes ranging from deficiency to excess (0.2 – 3.2 g/kg/d) with the IAAO protocol. Each study day, the test protein was provided as 8 hourly meals. With the fifth meal L-[1-13C]leucine was provided orally with collection of breath and urine samples.The protein requirement was determined by using a two-phase linear regression analysis on the change in breath 13C enrichment, reflecting protein synthesis.The mean protein requirement was determined to be 1.93g/kg/d and is significantly higher than the most recent 2014 recommendations (1.14 – 1.33g/kg/d, based on 120-140% above current RDA). These initial findings are the first to directly define a quantitative requirement for protein in children with PKU, and indicate that current recommendations may be underestimated. (Supported by Rare Disease Foundation; Ministry of Higher Education in Saudi Arabia and Saudi Arabian Cultural Bureau)
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".