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

Author:

González-Lamuño Domingo123ORCID,Morencos Carmenmelina4,Arrieta Francisco J.35ORCID,Venegas Eva36,Vicente-Rodríguez Germán278910ORCID,Casajús José Antonio278910ORCID,Couce Maria Luz31112ORCID,Aldámiz-Echevarría Luís312

Affiliation:

1. University Hospital “Marqués de Valdecilla”, Universidad de Cantabria and Research Institute Valdecilla (IDIVAL), 39008 Santander, Spain

2. Exercise and Health in Special Population Spanish Research Net (EXERNET), 50009 Zaragoza, Spain

3. Asociación Española para el Estudio de los Errores Congénitos del Metabolismo (AECOM)—AECOM&Sociedad, 28221 Majadahonda, Spain

4. Danone Nutricia Metabolics, 28043 Madrid, Spain

5. Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), 28034 Madrid, Spain

6. Unidad de Gestión Clínica de Endocrinología y Nutrición, Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Rocío, CSIC, Universidad de Sevilla, 41013 Seville, Spain

7. Instituto de Investigación Sanitaria Aragón (IIS Aragón), 50009 Zaragoza, Spain

8. EXER-GENUD (Growth, Exercise, Nutrition and Development) Research Group, Universidad de Zaragoza, 50009 Zaragoza, Spain

9. Centro de Investigación Biomédica en Red de Fisiopatología de la Obesidad y Nutrición (CIBERObn), 28040 Madrid, Spain

10. Faculty of Health and Sport Sciences, FCSD, Ronda Misericordia 5, 22001 Huesca, Spain

11. Unit for Diagnosis and Treatment of Congenital Metabolic Disorders, University Hospital of Santiago de Compostela, 15706 Santiago de Compostela, Spain

12. Instituto de Investigación Sanitaria de Santiago de Compostela, 15706 Santiago de Compostela, Spain

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.

Funder

AECOM

Publisher

MDPI AG

Reference62 articles.

1. Phenylketonuria;Blau;Lancet,2010

2. The complete European guidelines on phenylketonuria: Diagnosis and treatment;MacDonald;Orphanet J. Rare Dis.,2017

3. How practical are recommendations for dietary control in phenylketonuria?;Walter;Lancet,2002

4. (2024, February 03). Dietary Supplement Health and Education Act of 1994. Public Law 103–417, 103rd Congress, Available online: https://www.congress.gov/bill/103rd-congress/senate-bill/784#.

5. Physical development in patients with phenylketonuria on dietary treatment: A retrospective study;Mol. Genet. Metab.,2011

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