ArticleEuropean journal of clinical nutrition2014
PKU patients on a relaxed diet may be at risk for micronutrient deficiencies.
Article in European journal of clinical nutrition, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
27 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Phenylalanine Tolerance over Time in Phenylketonuria: A Systematic Review and Meta-Analysis.Nutrients · 2023Pooled it
- Improving phenylalanine and micronutrients status of children with phenylketonuria: a pilot randomized study.Orphanet journal of rare diseases · 2021Trial
- Nitrogen Balance after the Administration of a Prolonged-Release Protein Substitute for Phenylketonuria as a Single Dose in Healthy Volunteers.Nutrients · 2021Trial
- One-year follow-up of B vitamin and Iron status in patients with phenylketonuria provided tetrahydrobiopterin (BH4).Orphanet journal of rare diseases · 2018Trial
- Laboratory Monitoring of Nutritional Deficiencies in Children Following Restrictive Diets: A Narrative Review and Risk-Based Considerations.Children (Basel, Switzerland) · 2026Review
- Article
- The First 1000 Days of PKU: A Narrative Review of Maternal PKU and Early Life Management After Positive Newborn Screening.Nutrients · 2026Review
- Risk of inadequate protein and micronutrient intakes in patients with PKU with an increased phe-tolerance: Impact of a micronutrient-dense protein substitute.Molecular genetics and metabolism reports · 2025Article
- Nutritional management of metabolic disorders in neonates and infants in Saudi Arabia: consensus recommendations.Orphanet journal of rare diseases · 2025Article
- Current state of the treatment landscape of phenylketonuria.Orphanet journal of rare diseases · 2025Review
- Impact of a mobile health intervention on health management among children with phenylketonuria based on a multi-theory model of the behavior change wheel theory and family health theory: protocol for a randomized controlled trial.Frontiers in psychiatry · 2025Article
- The Prevention of Maternal Phenylketonuria (PKU) Syndrome: The Development and Evaluation of a Specific Training Program.Nutrients · 2024Article
- Nutrient Status and Intakes of Adults with Phenylketonuria.Nutrients · 2024Article
- Adult PKU Clinics in the UK-Users' Experiences and Perspectives.Nutrients · 2023Article
- Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria.Nutrients · 2023Article
- Characterization of Choline Nutriture among Adults and Children with Phenylketonuria.Nutrients · 2022Article
- The Impact of the Quality of Nutrition and Lifestyle in the Reproductive Years of Women with PKU on the Long-Term Health of Their Children.Nutrients · 2022Review
- Sustaining benefits of nutritional therapy in young adults with phenylketonuria - A 2 year prospective study.Molecular genetics and metabolism reports · 2020Article
- Large neutral amino acid status in association with P:T ratio and diet in adult and pediatric patients with phenylketonuria.JIMD reports · 2019Article
- Nutritional and Metabolic Characteristics of UK Adult Phenylketonuria Patients with Varying Dietary Adherence.Nutrients · 2019Article
Corrections and comments
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Authors and funding
13 authors.
Funding
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Abstract
objectiveTo investigate micronutrient supply in phenylketonuria (PKU) patients on a relaxed diet. SUBJECTS/
methodsSixty-seven patients (6-45 years) with a phenylalanine tolerance ≥ 600 mg/day were included in the study. From a 3-day diet record, protein supply as well as consumption of essential amino acids and several micronutrients were assessed and compared with the current recommendations and data for the healthy population.
resultsProtein supply and consumption of all essential amino acids were sufficient in all patients. Supply of micronutrients depended on dietary regime. Patients with a total protein supply of 120% or more of the recommended amount and at least 0.5 g protein per kg body weight from amino-acid mixture (AAM) were sufficiently supplied with all investigated micronutrients. All patients without AAM supplement showed severe micronutrient deficiencies in their diet records.
conclusionPKU patients under a relaxed diet are at risk of an insufficient nutrient supply, if they have first no substitution with AAM, second a protein supply less than 0.5 g per kg body weight from AAM or third a total protein supply less than 120% of the recommendations. Therefore, close monitoring, specific dietary counseling and potential supplementation is mandatory to prevent micronutrient deficiencies in PKU patients.
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