Purpose of review 

To explore the potential of a low carbohydrate ketogenic diet (LCKD) to counter physical activity intolerance, pain and muscle damage for glycogen storage disease (GSD) V and VII, and highlight the realistic possibility that nutrition could be key.

Recent findings 

Carbohydrate (CHO) ingestion during physical activity in GSDV and a LCKD for GSDVII is common. For the latter, a long-term study demonstrated improvement in physiological markers while on a LCKD. This included improvement in aerobic power and activity tolerance. In GSDV, preliminary research on a LCKD suggest a diet of 75% fat, 15% protein, 10% CHO, is best for improved function and compliance. Ketones provide immediate fuel for acute physical activity, and have an epigenetic role, improving ketone and lipid use. Evidence from elite athletes found a LCKD can increase fat oxidation and is optimal at 70% VO2max. This suggests the need to also improve conditioning via exercise to maximize the benefit of a LCKD.

Summary 

A high CHO diet in GSDV and VII comes with a restricted physical activity capacity alongside significant pain, muscle damage and risk of renal failure. Mounting evidence suggests a LCKD is efficacious for both disorders providing an immediate fuel source which may negate the need for a ‘warm-up’ prior to every activity and restore ‘normal’ function.

中文翻译：

---

生酮饮食有可能最大程度地减少新陈代谢缺陷对糖原贮积病V和VII的影响

审查目的 

探索低碳水化合物生酮饮食（LCKD）对抗糖原贮积病（GSD）V和VII的身体活动不耐受，疼痛和肌肉损伤的潜力，并强调营养可能是关键的现实可能性。

最近的发现 

在GSDV和LSD的GSDVII中，身体活动期间会摄入碳水化合物（CHO）。对于后者，一项长期研究表明，使用LCKD时，生理指标有所改善。这包括改善有氧能力和活动耐力。在GSDV中，对LCKD的初步研究表明，饮食中75％的脂肪，15％的蛋白质，10％的CHO是改善功能和依从性的最佳方法。酮可为急性体育活动提供直接燃料，并具有表观遗传作用，可改善酮和脂质的使用。精英运动员的证据表明，LCKD可以增加脂肪氧化，最理想的最大摄氧量为70％。这表明还需要通过运动来改善适应状况，以最大程度地提高LCKD的益处。

概要 

GSDV和VII中的高CHO饮食会限制身体活动，并伴有明显的疼痛，肌肉损伤和肾衰竭的风险。越来越多的证据表明，LCKD对两种疾病都是有效的，提供了直接的燃料来源，可能会在每次活动之前消除对“热身”的需求，并恢复“正常”功能。