ArticleMolecular genetics and metabolism reports2025
Risk of inadequate protein and micronutrient intakes in patients with PKU with an increased phe-tolerance: Impact of a micronutrient-dense protein substitute.
Article in Molecular genetics and metabolism reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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2 citing papers in PubMed.
- Observational
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9 authors.
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No grant is acknowledged in the PubMed record.
Abstract
Background: Patients with phenylketonuria (PKU) with higher phenylalanine (phe)-tolerance may have inadequate diets due to high-quality protein restriction and reduced intakes of nutrient-fortified phe-free, tyrosine (tyr)-enriched protein substitutes (PS). Methods: Open-label, 24-week, multi-centre intervention assessed the impact of a once-daily, micronutrient-dense PS on nutrient intakes in patients with an increased phe-tolerance. Subjects >12 years, consuming 0-25 g protein/day from a regular PS, were recruited. Diet records and fasting blood samples were collected at baseline and study end. Results: 11/13 subjects, 15-49y (5 males), with 6/11 taking tetrahydrobiopterin dihydrochloride (BH4) and/or a regular PS, completed the study. At baseline, protein and some essential amino acids (EAA) intakes were below recommendations in 4/11 and 3/11 subjects, respectively. Vitamins A, B2, B12, D, calcium, iron, iodine, zinc and magnesium intakes were below recommendations for 7/11. After the intervention, 2/11 had total protein intakes below recommendations, while 11/11 met EAA recommendations. Micronutrient intakes improved to ≥90 % of recommendations for all subjects except for vitamin B12 (4/11) and phosphorus (1/6). Docosahexaenoic acid (DHA) intakes increased. Blood phe levels remained stable. Conclusion: Patients with PKU who have relaxed their phe-restricted diet are at risk of insufficient nutrient supply, and dietary counselling should be offered. Consuming the study PS increased nutrient intakes closer to dietary recommendations.
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