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一个跨部分研究的蛋白质替代儿科芬基尿症饮食:时间来注意注意

Albina Tummolo¹, Rosa Carella¹, Donatella De Giovanni¹

¹Department of Metabolic Diseases and Clinical Genetics, Giovanni XXIII Children Hospital, Azienda Ospedaliero-Universitaria Consorziale, 70126 Bari, Italy.

|June 13, 2025

概括

基尿症 (PKU) 的蛋白质替代品 (PSs) 通常超过推的微量营养素水平,在产品和年龄组之间存在显著的变化. 胆在许多配方中显著缺失,需要仔细考虑饮食.

科学领域:

营养科学营养科学
代谢障碍代谢障碍代谢障碍
儿童营养学儿童营养学

背景情况:

蛋白质替代品 (PSs) 对于管理经典性尿症 (PKU) 是必不可少的,提供蛋白质和微量营养素.
最近PS的进步改善了氨基酸的输送和配方的多样性,但微量营养素含量需要评估.
在儿科PKU管理中使用的PS的微量营养素概况受到有限的关注.

研究的目的:

分析意大利为儿科PKU患者提供PS配方的微量营养素成分.
为了比较PSs的微量营养素贡献与不同年龄组的推饮食补贴 (RDAs).
评估PS产品和年龄段之间微量营养素补充剂的变化.

主要方法:

对儿科PKU患者的63个PSS分析了28种微量营养素.
根据RDA年龄范围分组PS:0-6个月,6-12个月,1-3年,3-8年,8-14年.
每10克蛋白质等效 (PE) 的标准化微量营养素含量.

主要成果:

与所有年龄组的RDA相比,许多微量营养素被过度补充,特别是婴儿 (0-6个月) 的维生素K,和.
1-3岁的年龄组对大多数微量营养素的补充水平最低.
在PS中观察到补充剂的高变化,特别是在老年人群中对生物素和铜的高变化. 胆经常没有补充.

结论:

当前的PS通常会提供过多的某些微量营养素,产品间和年龄间的差异很大.
观察到的变化和潜在的过量补充需要仔细考虑规定的PS量,以避免微量营养素失衡.
评估每天的微量营养素总摄入量,考虑到PS的数量,对于最佳的PKU饮食管理至关重要.

关键词:

在PKU中,PKU是PKU.基尿症基尿症在 RDA RDA 中使用.饮食饮食饮食饮食微量营养素微量营养素蛋白质替代品蛋白质替代品要求要求要求要求要求要求要求要求要求要求要求补充补充补充补充补充

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