ArticleEuropean journal of clinical nutrition2016
Protein substitutes for phenylketonuria in Europe: access and nutritional composition.
Article in European journal of clinical nutrition, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 4 of them syntheses that pooled it.
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The trial behind it
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Who cites it
25 citing papers in PubMed, 4 syntheses or guidelines pooled it, 43 citations in OpenAlex.
- Pooled it
- Pooled it
- Pooled it
- The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis.Nutrients · 2018Pooled it
- Children's health: insights from space medicine on metabolic health.NPJ microgravity · 2025Review
- Global Clinical Practice in Transitioning Protein Substitutes for Children with Phenylketonuria.Nutrients · 2025Article
- A Cross-Sectional Study on Protein Substitutes for Paediatric Phenylketonuria Diet: Time to Pay Attention.Nutrients · 2025Article
- A 12-month, longitudinal, intervention study examining a tablet protein substitute preparation in the management of tyrosinemia.Molecular genetics and metabolism reports · 2024Article
- Evaluating Therapy and Growth in Children with Phenylketonuria: A Retrospective Longitudinal Study from Two Romanian Centers.Medicina (Kaunas, Lithuania) · 2024Article
- Glycomacropeptide-Based Protein Substitutes for Children with Phenylketonuria in Italy: A Nutritional Comparison.Nutrients · 2024Review
- Total Protein Intake in Patients with PKU: Adequacy Evaluation According to the European PKU Guidelines from 2017.Nutrients · 2023Article
- Phenylalanine free infant formula in the dietary management of phenylketonuria.Orphanet journal of rare diseases · 2023Article
- Quality of life aspects of a low protein diet using GMP in patients with phenylketonuria.The Journal of international medical research · 2022Observational
- Food Regime for Phenylketonuria: Presenting Complications and Possible Solutions.Journal of multidisciplinary healthcare · 2022Review
- Metabolic Control of Patients with Phenylketonuria in a Portuguese Metabolic Centre Comparing Three Different Recommendations.Nutrients · 2021Observational
- Current Practices and Challenges in the Diagnosis and Management of PKU in Latin America: A Multicenter Survey.Nutrients · 2021Article
- Observational
- Nutrient intake, body composition, and blood phenylalanine control in children with phenylketonuria compared to healthy controls.Molecular genetics and metabolism reports · 2020Article
- Reply to Jakovac; Severity of COVID-19 infection in patients with phenylketonuria: is vitamin D status protective?American journal of physiology. Endocrinology and metabolism · 2020Article
- Optimising amino acid absorption: essential to improve nitrogen balance and metabolic control in phenylketonuria.Nutrition research reviews · 2019Review
Corrections and comments
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Authors and funding
11 authors at 11 institutions in 9 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND/
objectivesProtein substitutes (PS) are an essential component in the dietary management of phenylketonuria (PKU). PS are available as phenylalanine-free amino-acid mixtures (AAM), glycomacropeptide-based PS (GMP) and large neutral amino acids (LNAA). There is a lack of information regarding their availability in different countries and comparison of their nutritional composition is limited. The objectives of this study were to identify the number of PS available in different European countries and Turkey and to compare their nutritional composition. SUBJECTS/
methodsMembers of the European Nutritionist Expert Panel on PKU (ENEP) (Portugal, Spain, Belgium, Italy, Germany, Netherlands, United Kingdom, Denmark and Turkey) provided data on PS available in each country. The nutritional composition of PS available in Portugal was analyzed.
resultsThe number of PS available in each country varied from 30 (Turkey) to 105 (Germany), with a median of 64. GMP was available only in Portugal, whereas LNAA was an option in Portugal, Italy, Turkey and Denmark. Some PS were designed for weaning. Many PS did not contain added fat and fiber. GMP contained the highest carbohydrate (CHO) and energy content as well as higher LNAA content compared with AAM. Only one AAM contained added fructo-oligosaccharides and galacto-oligosaccharides. AAM designed for the first year of life had the highest CHO, fat and LNAA contribution. Liquid AAM had lower CHO and fat contents compared with powdered AAM, but contained higher LNAA.
conclusionsThere was widely dissimilar numbers of PS available in different countries. Nutritional composition of different PS was variable and should be considered before prescription.
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