自然蛋白质限制饮食及其对和甲基酸血症患者线性生长的影响:系统性审查
Jessica Ramirez1, María Jesús Leal-Witt1, Juan Francisco Cabello1
1Laboratorio de Genética y Enfermedades Metabólicas del Instituto de Nutrición y Tecnología de los Alimentos (INTA), Universidad de Chile, Santiago 7830490, Chile.
Journal of personalized medicine
|January 27, 2026
概括
饮食中的蛋白质摄入量会影响酸血症 (PA) 和甲基酸血症 (MMA) 的增长. 限制天然蛋白质和更高蛋白质替代品 (PS) 摄入与PA和不响应的MMA中线性生长受损 (较低的身高对年龄z-score) 相相关.
科学领域:
- 生物化学 生物化学
- 儿科 儿科 儿科
- 营养科学 营养科学
背景情况:
- propionic acidemia (PA) 和 methylmalonic acidemia (MMA) 破坏了必需氨基酸 (甲,三,,异氨基酸) 的新陈代谢,导致神经毒性有机酸的积聚.
- 目前的治疗包括限制天然蛋白质,并补充含有必需氨基酸的蛋白质替代品 (PS).
研究的目的:
- 研究自然蛋白质和蛋白质替代品 (PS) 摄入量与PA和MMA患者的线性生长障碍之间的关系.
主要方法:
- 根据PRISMA协议进行系统审查,包括1970-2025年的研究.
- 对选定的文章应用了资格标准和质量评估.
- 分析的重点是天然蛋白质和PS摄入量相对于身高与年龄 (H/A) z-score.
主要成果:
- 自然蛋白质摄入量随着年龄的增长而下降,需要补充PS.
- 较高的PS摄入量与PA和不响应的MMA中的H/Az分数负相关.
- 补充PS增加了白蛋白,造成氨基酸不平衡,需要补充L-Val和L-Ile,进一步影响生长.
结论:
- 限制天然蛋白质和高PS摄入量与PA和不响应的MMA的线性生长减少 (较低的H/Az分数) 有关.
- 具有MMA反应的个体耐受了更多的天然蛋白质,需要更少的PS,并表现出更好的生长结果.
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