基尿症中随时间推移的氨耐受性:系统性审查和元分析
Alex Pinto1,2, Fatma Ilgaz3, Sharon Evans1
1Department of Dietetics, Birmingham Women's and Children's Hospital, Birmingham B4 6NH, UK.
Nutrients
|August 26, 2023
概括
基尿症 (PKU) 患者的自然蛋白质和氨耐受性随着年龄的增长而增加,在青春期达到顶峰. 这种耐受性对于终身管理PKU至关重要.
科学领域:
- 代谢障碍 代谢障碍 代谢障碍
- 营养科学 营养科学
- 人类生理学 人类生理学
背景情况:
- 基尿症 (PKU) 管理需要仔细控制氨 (Phe) 摄入量.
- 在PKU中自然蛋白质耐受性在寿命之间有所不同,但终身数据有限.
- 了解终身耐受性是PKU患者最佳管理的关键.
研究的目的:
- 系统地审查和分析Phe和自然蛋白质耐受性的变化,从出生到成年期在得到良好控制的PKU患者.
- 为了提供对整个PKU个体的饮食耐受性在整个生命周期的进展的见解.
主要方法:
- 对2020年7月之前发表的研究的五个电子数据库进行系统审查.
- 包括37篇文章 (n=2464名患者) 符合资格标准.
- 对18项选定的研究进行元分析,以量化耐受性变化.
主要成果:
- 费和天然蛋白质摄入量从婴儿期逐渐增加到6岁.
- 耐受性在童年后期和青春期显著增加,从生命的第一年开始增加了三倍以上.
- 成人的天然蛋白质耐受性在17岁左右达到顶峰,并保持稳定,尽管成人数据有限.
结论:
- 在PKU中,Phe和天然蛋白质耐受性随着年龄的增长而增加,特别是在青春期,在青春期晚期达到峰值水平.
- 研究结果表明,在PKU患者一生中,需要采用动态饮食管理方法.
- 研究之间的高度异质性需要谨慎的解释,特别是对于成人数据.
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