ArticleEuropean journal of clinical nutrition2017
Nutritional status in patients with phenylketonuria using glycomacropeptide as their major protein source.
Article in European journal of clinical nutrition, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers, 3 of them syntheses that pooled it.
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Who cites it
21 citing papers in PubMed, 3 syntheses or guidelines pooled it, 43 citations in OpenAlex.
- Systematic literature review of the somatic comorbidities experienced by adults with phenylketonuria.Orphanet journal of rare diseases · 2024Pooled it
- Systematic Review and Meta-Analysis of Dietary Interventions and Microbiome in Phenylketonuria.International journal of molecular sciences · 2023Pooled it
- The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis.Nutrients · 2018Pooled it
- Nutritional management of metabolic disorders in neonates and infants in Saudi Arabia: consensus recommendations.Orphanet journal of rare diseases · 2025Article
- The relationship between adult phenylketonuria and the cardiovascular system - insights into mechanisms and risks.Orphanet journal of rare diseases · 2025Review
- Glycomacropeptide-Based Protein Substitutes for Children with Phenylketonuria in Italy: A Nutritional Comparison.Nutrients · 2024Review
- Body Composition Evaluation and Clinical Markers of Cardiometabolic Risk in Patients with Phenylketonuria.Nutrients · 2023Review
- Total Protein Intake in Patients with PKU: Adequacy Evaluation According to the European PKU Guidelines from 2017.Nutrients · 2023Article
- Evaluation of a New Glycomacropeptide-Based Protein Substitute in Powdered and Liquid Format in Patients with PKU.Nutrients · 2023Article
- Growth and Nutritional Status of Phenylketonuric Children and Adolescents.BMC pediatrics · 2022Article
- Genetic etiology and clinical challenges of phenylketonuria.Human genomics · 2022Review
- Glycomacropeptide in PKU-Does It Live Up to Its Potential?Nutrients · 2022Review
- Metabolic Control of Patients with Phenylketonuria in a Portuguese Metabolic Centre Comparing Three Different Recommendations.Nutrients · 2021Observational
- Phenylketonuria.Nature reviews. Disease primers · 2021Review
- Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective.Orphanet journal of rare diseases · 2021Article
- Article
- Sex effects of dietary protein source and acid load on renal and bone status in the PahPhysiological reports · 2019Article
- Glycomacropeptide: long-term use and impact on blood phenylalanine, growth and nutritional status in children with PKU.Orphanet journal of rare diseases · 2019Review
- Comparison of Glycomacropeptide with Phenylalanine Free-Synthetic Amino Acids in Test Meals to PKU Patients: No Significant Differences in Biomarkers, Including Plasma Phe Levels.Journal of nutrition and metabolism · 2018Article
- Dietary management of maternal phenylketonuria with glycomacropeptide and amino acids supplements: A case report.Molecular genetics and metabolism reports · 2017Article
Corrections and comments
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Authors and funding
10 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND/
objectivesLow phenylalanine (PHE), glycomacropeptide-based protein substitute (GMP) is an alternative to traditional L-amino acid supplements (AA) used in the dietary management of phenylketonuria (PKU). In a retrospective, longitudinal study, we report the nutritional status of PKU patients taking AA and GMP. SUBJECTS/
methodsEleven PKU patients aged 27±10 years (1 HPA, 4 mild and 6 classical PKU) on dietary treatment were evaluated (anthropometry, body composition, blood pressure measurements, biochemical markers including vitamin, mineral, lipids, carbohydrates and protein status/metabolism, and nutritional intake assessment) at two different annual reviews. The mean time taking AA was 13±5 months and GMP 13±7 months. Blood phenylalanine (PHE) and tyrosine (TYR) were analysed before and after GMP introduction.
resultsBoth GMP and AA protein substitutes provided similar protein equivalent intake (0.85 vs 0.75 g/kg/day, P=0.182). In the GMP group, it contributed 57% (27-100%) of the protein substitute intake (with AA delivering the rest of protein substitute intake), providing an additional 34±12 mg/day PHE. Nutritional intake, anthropometry and body composition measurements were similar in both the groups. Median blood PHE did not change (P=0.594), although values within target range improved (36 vs 46%), but this was not statistically significant. Mean blood TYR increased (52.0±19.2 vs 63.2±25.6 μmol/l, P=0.033), and all biochemical markers remained stable, except for a lower A1C haemoglobin (P=0.011).
conclusionsPartial GMP contribution to total protein substitute intake did not affect nutritional status in patients with PKU. Blood PHE control was not adversely affected. The increased blood TYR after GMP introduction necessitates further study.
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