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Total Protein Intake in Patients with PKU: Adequacy Evaluation According to the European PKU Guidelines from 2017

Melanie Gomes1, Manuela Ferreira Almeida2,3,4, Catarina Sousa Barbosa2

  • 1Nephrocare Portugal, Fresenius Medical Care Nutrition Departament, Rua Professor Salazar de Sousa, Lote 12, 1750-233 Lisboa, Portugal.

Nutrients
|December 9, 2023
PubMed

Insights

Protein intake in phenylketonuria (PKU) patients varies significantly. Most patients exceed recommended levels, suggesting individualized protein recommendations are necessary due to PKU heterogeneity.

Area of Science:

  • Metabolic disorders
  • Nutritional science
  • Genetics

Background:

  • Protein requirements in phenylketonuria (PKU) are debated.
  • Early-treated PKU patients require ongoing nutritional assessment.

Purpose of the Study:

  • To evaluate protein intake and adequacy in early-treated PKU patients.
  • To compare intake with European PKU Guidelines (EPG) recommendations.

Main Methods:

  • Studied 99 early-treated PKU patients (aged 19.3 ± 8.2 years).
  • Assessed total protein (TP), natural protein (NP), and protein equivalent from protein substitutes (PE) intake.
  • Calculated total protein adequacy (TPA) against WHO (2007) safe levels.

Main Results:

  • 78% of patients exceeded EPG protein intake recommendations.
  • Median TPA was 171%, with 79% from NP and 84% from PE.
  • Only 6% of patients had TPA below 100%.

Conclusions:

  • Significant heterogeneity exists in PKU protein intake.
  • A 'one-size-fits-all' protein recommendation for PKU may not be appropriate.
  • Further research is needed to establish individualized protein requirements.

Keywords:
European PKU guidelinesnatural proteinphenylalaninephenylketonuriaprotein intakeprotein intake adequacyprotein substitutes

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