SynthesisNutrients2024
Factors Affecting Adherence to a Low Phenylalanine Diet in Patients with Phenylketonuria: A Systematic Review.
Synthesis in Nutrients, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed.
- Longitudinal Metabolic Control in Children With Early-Treated Phenylketonuria.JAMA network open · 2026Article
- Article
- Minicells derived fromApplied and environmental microbiology · 2026Article
- Observational
- Article
- Quality of life after diet liberalization in individuals with phenylketonuria treated with Pegvaliase.Molecular genetics and metabolism reports · 2026Article
- Clinical experience with Pegvaliase in phenylketonuria: A retrospective chart review of outcomes, safety, and dosing patterns.Molecular genetics and metabolism reports · 2026Article
- Article
- Disease Burden and Pharmacological Treatment Patterns in Children and Adults With Phenylketonuria: A Real-World Matched Cohort Study.Journal of inherited metabolic disease · 2026Article
- Nutritional knowledge of the phenylketonuria diet among healthcare providers in Saudi Arabia.Saudi medical journal · 2025Article
- Article
- Current Insights into Nutritional Management of Phenylketonuria: An Update for Children and Adolescents.Children (Basel, Switzerland) · 2025Review
- Phenylketonuria as an Adherence Disease.Patient preference and adherence · 2025Review
- Article
- Transitioning of protein substitutes in patients with phenylketonuria: a pilot study.Frontiers in nutrition · 2024Article
Corrections and comments
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Authors and funding
5 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Phenylketonuria (PKU) is an inherited metabolic disorder that requires lifelong adherence to a low-phenylalanine (Phe) diet to prevent severe neurological complications. However, maintaining dietary adherence can be challenging for patients and their families. This systematic review aimed to comprehensively evaluate the factors affecting adherence to a low-Phe diet in patients with PKU. A systematic search of multiple databases was conducted, and 49 studies were included in the final analysis. The quality of evidence was assessed using the Joanna Briggs Institute levels of evidence and the Quality Assessment with Diverse Studies tool. The review identified four main categories of factors influencing dietary adherence: family-related factors (social, psychological, behavioral, and educational), patient-specific factors (psychological, behavioral, educational, and demographic), environmental factors (healthcare professional support, educational and camp-based interventions, and the COVID-19 pandemic), and therapy-related factors (protein substitute formulation, clinic visits, blood tests, and telemedicine). The findings highlight the complex interplay between elements contributing to dietary adherence in PKU patients and underscore the importance of a multifaceted approach to support patients and their families. Future research should prioritize high-quality longitudinal and experimental studies to provide stronger evidence for the PKU community.
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