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Review
. 2024 Feb 25;16(5):639.
doi: 10.3390/nu16050639.

Supplementation for Performance and Health in Patients with Phenylketonuria: An Exercise-Based Approach to Improving Dietary Adherence

Domingo González-Lamuño  1   2   3 , Carmenmelina Morencos  4 , Francisco J Arrieta  3   5 , Eva Venegas  3   6 , Germán Vicente-Rodríguez  2   7   8   9   10 , José Antonio Casajús  2   7   8   9   10 , Maria Luz Couce  3   11   12 , Luís Aldámiz-Echevarría  3   12
Affiliations
Review

Supplementation for Performance and Health in Patients with Phenylketonuria: An Exercise-Based Approach to Improving Dietary Adherence

Domingo González-Lamuño et al. Nutrients. .

Abstract

Supplementation is crucial for improving performance and health in phenylketonuria (PKU) patients, who face dietary challenges. Proteins are vital for athletes, supporting muscle growth, minimizing catabolism, and aiding muscle repair and glycogen replenishment post-exercise. However, PKU individuals must limit phenylalanine (Phe) intake, requiring supplementation with Phe-free amino acids or glycomacropeptides. Tailored to meet nutritional needs, these substitutes lack Phe but fulfill protein requirements. Due to limited supplement availability, athletes with PKU may need higher protein intake. Various factors affect tolerated Phe levels, including supplement quantity and age. Adhering to supplement regimens optimizes performance and addresses PKU challenges. Strategically-timed protein substitutes can safely enhance muscle synthesis and sports performance. Individualized intake is essential for optimal outcomes, recognizing proteins' multifaceted role. Here, we explore protein substitute supplementation in PKU patients within the context of physical activity, considering limited evidence.

Keywords: adherence; amino acid; glycomacropetides; phenyalanine; phenylketonuria; physical activity; protein substitutes; supplement regimens; tyrosine.

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Conflict of interest statement

The participation of C.M., an expert in PKU supplements from Nutricia-Danone, does not influence the content of the article nor have any commercial interest.

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Grants and funding

This research received no external funding. We thank AECOM for support in the publication fees.