患有GSD1的患者在摄入含有常规尺寸果糖和银河糖来源的餐后是否增加p-乳酸盐? 来自前性,非盲目的交叉试点研究的观察结果
Camilla Diana B Caroee1, Allan M Lund1,2,3
1Department of Pediatrics, Center for Inherited Metabolic Diseases Copenhagen University Hospital, Rigshospitalet Copenhagen Denmark.
JIMD reports
|January 20, 2025
概括
果糖和银河糖摄入量可以提高乳酸在糖原储存疾病1型 (GSD1) 患者. 在这项试点研究中,果汁消费导致乳酸增加的程度高于牛奶.
科学领域:
- 代谢障碍 代谢障碍 代谢障碍
- 儿科内分泌学 儿科内分泌学
- 营养科学 营养科学
背景情况:
- 糖原储存疾病1型 (GSD1) 是一种影响葡萄糖生产的代谢障碍.
- 摄入果糖和银河糖可以在GSD1患者中导致低血糖和乳酸化.
- 在GSD1中,饮食管理通常涉及到玉米粉或连续料以维持葡萄糖水平.
研究的目的:
- 研究来自不同来源的果糖和银河糖对GSD1患者乳酸水平的影响.
- 为了比较对果糖和银河糖的代谢反应,在牛奶,果汁和水中的挑战.
- 评估在GSD1管理中常见的食碳水化合物的安全性和有效性.
主要方法:
- 一项随机交叉试点研究,涉及7名GSD1患者 (6名GSD1a,1名GSD1b).
- 患者在过夜饮食治疗后从200毫升脱脂牛奶,果汁或水中摄取果糖和银河糖.
- 乳酸,葡萄糖,甘油三,尿酸和氨酸水平在基线和摄入后4小时间隔测量.
主要成果:
- 牛奶消费后3名患者的乳酸水平增加 (平均增加0.3 mmol/L).
- 果汁消费后4名患者的乳酸盐水平增加 (平均增加1.3 mmol/L),在30-60分钟达到峰值.
- 果汁诱导的乳酸升高在120分钟内恢复到基线; 牛奶诱导的变化不那么明显.
- 对果糖和银河糖的AUC没有观察到一致的变化.
结论:
- 基于果汁的果糖和银河糖的挑战可以暂时增加GSD1患者的乳酸水平.
- 与果汁相比,基于牛奶的挑战对乳酸的影响不那么重大.
- 需要进一步的研究来确定GSD1患者的安全碳水化合物摄入限值和来源.
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