影响基尿症患者遵守低氨饮食的因素:系统性审查
Roza Yagudina1, Andrey Kulikov1, Vyacheslav Serpik1
1Department of Organization of Medical Provision and Pharmacoeconomics, Sechenov First Moscow State Medical University (Sechenov University), Trubetskaya Str. 8/2, 119991 Moscow, Russia.
Nutrients
|September 28, 2024
概括
基尿症 (PKU) 管理需要严格的低氨饮食. 本综述确定了影响遵守这一关键饮食的家庭,患者,环境和治疗因素.
科学领域:
- 代谢障碍 代谢障碍 代谢障碍
- 遗传学 遗传学 是一个
- 营养科学 营养科学
背景情况:
- 基尿症 (PKU) 是一种遗传代谢障碍,需要终身低氨酸 (Phe) 的饮食管理.
- 严格遵守低Phe饮食对于预防PKU患者严重神经损伤至关重要.
- 维持饮食遵守的挑战显著影响患者的结果.
研究的目的:
- 系统地审查和评价影响PKU患者遵守低Phe饮食的因素.
- 确定跨家庭,患者,环境和治疗领域的关键决定因素.
- 为改善PKU饮食管理的策略提供信息.
主要方法:
- 在多个数据库中进行系统的文献搜索.
- 将49项研究纳入最终分析.
- 使用乔安娜·布里格斯研究所证据水平和质量评估与多元研究工具进行质量评估.
主要成果:
- 确定了影响低Phe饮食坚持的四大类因素:与家庭相关的,患者特定的,环境和治疗相关的.
- 家庭因素包括社会,心理,行为和教育方面的因素.
- 患者因素包括心理,行为,教育和人口因素.
- 环境影响包括医疗保健支持,干预措施以及COVID-19流行病等事件的影响.
- 治疗因素包括蛋白质替代剂配方,诊所访问,血液监测和远程医疗.
结论:
- 在PKU中,饮食坚持受到多因素元素的复杂相互作用的影响.
- 为了支持PKU患者及其家人,必须采取全面的,多方面的方法.
- 未来的研究应该专注于高质量的纵向和实验研究,以加强为PKU社区提供基于证据的建议.
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