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用中链甘油三丰富饮食长期治疗脂蛋白脂酶缺乏症:一个案例系列
Liali Aljouda1, Laura Nagy1, Andreas Schulze1,2,3
1Clinical and Metabolic Genetics, The Hospital for Sick Children, Toronto, ON M5G 1E8, Canada.
Nutrients
|August 26, 2023
概括
限制长链甘油三 (LCT) 和补充中链甘油三 (MCT) 的专门饮食有效地降低了患有脂蛋白脂酶 (LPL) 缺乏症的儿童的高甘油三水平,从而预防了严重的并发症.
科学领域:
- 遗传学和代谢障碍 遗传学和代谢障碍
- 儿童营养学 儿童营养学
- 胃肠病学 胃肠病学
背景情况:
- 脂蛋白脂酶 (LPL) 缺乏症是一种遗传性疾病,在儿童中引起严重的高甘油三血症.
- 目前针对LPL缺乏症的药理疗法在降低甘油三 (TGs) 方面缺乏持续的有效性.
- 低脂缺乏症的症状包括腹痛和复发性胰腺炎,通常在10岁之前出现.
研究的目的:
- 评估LCT受限,MCT补充饮食在治疗儿科LPL缺乏症中的有效性.
- 评估饮食对甘油三水平和LPL相关症状的影响.
- 为了确定这种饮食干预的可行性和耐受性.
主要方法:
- 一个回顾七名儿科患者的病例系列,患有疑似LPL缺乏的高三糖血症.
- 从2000年1月到2022年12月收集的数据包括人口统计,诊断,临床和生化资料.
- 六名患者确诊患有LPL缺乏症;其中一人患有1a型 (GSD1a) 糖原储存疾病.
主要成果:
- 在LPL缺乏的患者中,LCT受限,MCT补充的饮食显著降低了TGs,从中位数40.9 mmol/L降至12.0 mmol/L.
- 总胆固醇和胰腺脂酶水平也大幅下降.
- 在48个患者年中,只有一个胰腺炎并发症发生,所有患者的生长和发育均正常.
结论:
- 饮食限制LCT与MCT补充剂相结合是儿科LPL缺乏症的可行和有效的长期管理策略.
- 这种饮食方法成功地降低了TG水平,并预防了特定疾病的并发症.
- 这种饮食被很好地容忍,并支持儿科患者的正常生长和发育.
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