Factors Affecting Adherence to a Low Phenylalanine Diet in Patients with Phenylketonuria: A Systematic Review.

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.

Patient Flows, Patient Distribution Computations and Medicines Accounting in the Pharmacoeconomic Models Through Procurement Perspective.

PURPOSE: Stimulating cost reduction of pharmaceutical companies to optimize the structure of distribution of patients by the level of treatment costs in various programs. PATIENTS AND METHODS: In this article, we rise up the issues of pharmacoeconomic modeling related to the description of the patient flows in the pharmacoeconomic model and methods to determining the course dose of drugs under the restriction of integer computations. We established two possible ways of distributing patients through treatment regimens in pharmacoeconomic models, also analyzed the effects of simultaneous and uniform entry of patients into the model. Also, we considered the limitations and possibilities of calculations based on the active substance and packaging, as well as the transition factor of the remainder of the drug in the next time period. RESULTS: A mathematical model of the analysis of the system assessment of patients by the level of risk of abandoning a healthy lifestyle in connection with the growing problems of the difficult-to-control process is developed. The use of a rational data convolution mode allowed us to obtain a criterion for the optimality of the process and a logical point of stability of the pharmaceutical company by rationally applying treatment methods according to established standards (percentage base). This approach makes it possible to influence the management of private clinics through clear ideas on the algorithms for prescribing drugs in each group of patients and their zoning in the vector recovery mode. CONCLUSION: Initial data and sample size: 552 measurements of the intervals of changes in the subject's indicators in seconds (smoothing and scaling the data to the level of the base (analytical) period or the final (barrier) period). Regular use of this approach makes it possible to reserve the resources of the body of a healthy and physically active person in a timely manner for a very reliable functioning of all body systems, taking into account the dosed intake of prescribed drugs and the conditions of comfortable (decent) maintenance of patients during the course of treatment according to the method chosen by the doctor.