ReviewCurrent opinion in clinical nutrition and metabolic care2014
Advances in the nutritional and pharmacological management of phenylketonuria.
Review in Current opinion in clinical nutrition and metabolic care, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
21 citing papers in PubMed, 44 citations in OpenAlex.
- Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial.The American journal of clinical nutrition · 2016Trial
- How Well Is Blood Phenylalanine Controlled in Maternal PKU in Europe? Results from 102 Pregnancies.Nutrients · 2026Article
- Toxicity spectrum of pegvaliase: A pharmacovigilance analysis using the FAERS database.Orphanet journal of rare diseases · 2025Article
- Current Insights into Nutritional Management of Phenylketonuria: An Update for Children and Adolescents.Children (Basel, Switzerland) · 2025Review
- Evaluation of bone mineral density and biochemical markers in pediatric patients with phenylketonuria.Molecular genetics and metabolism reports · 2024Article
- Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe: The Impact of Age and Phenylketonuria Severity.Nutrients · 2024Article
- Amino Acid Profile Alterations in Phenylketonuria: Implications for Clinical Practice.Metabolites · 2024Article
- Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria.Nutrients · 2023Article
- Protein substitutions as new-generation pharmanutrition approach to managing phenylketonuria.Clinical and experimental pediatrics · 2023Article
- Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein.Nutrients · 2022Review
- Growth and Nutritional Status of Phenylketonuric Children and Adolescents.BMC pediatrics · 2022Article
- Cheese Whey Processing: Integrated Biorefinery Concepts and Emerging Food Applications.Foods (Basel, Switzerland) · 2019Article
- Fractionation of Glycomacropeptide from Whey Using Positively Charged Ultrafiltration Membranes.Foods (Basel, Switzerland) · 2018Article
- The drug transporter OAT3 (SLC22A8) and endogenous metabolite communication via the gut-liver-kidney axis.The Journal of biological chemistry · 2017Article
- Molecular epidemiology, genotype-phenotype correlation and BH4 responsiveness in Spanish patients with phenylketonuria.Journal of human genetics · 2016Article
- Protein substitutes for phenylketonuria in Europe: access and nutritional composition.European journal of clinical nutrition · 2016Article
- Phenylketonuria and Gut Microbiota: A Controlled Study Based on Next-Generation Sequencing.PloS one · 2016Article
- Emerging trends in nutraceutical applications of whey protein and its derivatives.Journal of food science and technology · 2015Review
- Phenylketonuria: a review of current and future treatments.Translational pediatrics · 2015Review
- Glycomacropeptide is a prebiotic that reduces Desulfovibrio bacteria, increases cecal short-chain fatty acids, and is anti-inflammatory in mice.American journal of physiology. Gastrointestinal and liver physiology · 2015Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
purpose of reviewThe purpose is to discuss advances in the nutritional and pharmacological management of phenylketonuria (PKU). RECENT
findingsGlycomacropeptide (GMP), a whey protein produced during cheese production, is a low-phenylalanine (phe) intact protein that represents a new dietary alternative to synthetic amino acids for people with PKU. Skeletal fragility is a long-term complication of PKU that based on murine research, appears to result from both genetic and nutritional factors. Skeletal fragility in murine PKU is attenuated with the GMP diet, compared with an amino acid diet, allowing greater radial bone growth. Pharmacologic therapy with tetrahydrobiopterin, acting as a molecular chaperone for phenylalanine hydroxylase, increases tolerance to dietary phe in some individuals. Large neutral amino acids inhibit phe transport across the intestinal mucosa and blood-brain barrier, and are most effective for individuals unable to comply with the low-phe diet. SUMMARY: Although a low-phe synthetic amino acid diet remains the mainstay of PKU management, new nutritional and pharmacological treatment options offer alternative approaches to maintain lifelong low phe concentrations. GMP medical foods provide an alternative to amino acid formula that may improve bone health, and tetrahydrobiopterin permits some individuals with PKU to increase tolerance to dietary phe. Further research is needed to characterize the long-term efficacy of these new approaches for PKU management.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.