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

被引:4
作者
Yagudina, Roza [1 ]
Kulikov, Andrey [1 ]
Serpik, Vyacheslav [1 ]
Protsenko, Marina [1 ]
Kopeyka, Kirill [1 ]
机构
[1] Sechenov Univ, Sechenov Moscow State Med Univ 1, Dept Org Med Provis & Pharmacoecon, Trubetskaya Str 8-2, Moscow 119991, Russia
关键词
phenylketonuria; PKU; dietary adherence; low-Phe diet; compliance; barriers; facilitators; systematic review; BONE-MINERAL STATUS; PROTEIN SUBSTITUTE; METABOLIC-CONTROL; MATERNAL PHENYLKETONURIA; YOUNG-ADULTS; CHILDREN; ADOLESCENTS; KNOWLEDGE; IMPACT; MANAGEMENT;
D O I
10.3390/nu16183119
中图分类号
R15 [营养卫生、食品卫生]; TS201 [基础科学];
学科分类号
100403 ;
摘要
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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页数:25
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