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.
The objective of this study is to develop a universally applicable approach for establishing the optimal dose range for the irradiation of plant and animal products. The approach involves the use of the optimization function for establishing the optimal irradiation dose range for each category of plant and animal product to maximize the suppression of targeted pathogens while preserving the surrounding molecules and biological structures. The proposed function implies that pathogens found in the product can be efficiently suppressed provided that irradiation is performed with the following criteria in mind: a high irradiation dose uniformity, a high probability of irradiation hitting pathogens and controlled heterogeneity of radiobiological sensitivity of pathogens. This study compares the optimal dose ranges for animal and plant products using beef tenderloin and seed potato tubers as examples. In a series of experiments, our team traced the dose dependencies of myoglobin oxidation in beef and the amount of potential damage to albumin’s native structure. The behavior patterns of myoglobin derivatives and the amount of potential damage to albumin found in this study determined the optimal dose range, which appeared to be wider for beef irradiation compared to that for seed potato tubers, as they do not require uniform irradiation of the entire volume since targeted phytopathogens are predominantly found within the surface layers of the tubers. The use of proprietary methods involving spectrophotometry and high-performance liquid chromatography–mass spectrometry provides a novel perspective on the quantitative assessment of the myoglobin oxidation level and the potential damage to albumin’s native structure.
30 May 2025 in The European Physical Journal Special Topics