International Association for Muscle Glycogen Storage Disease, San Francisco, California, USA.
Centre for Human Performance, Exercise and Rehabilitation, Brunel University, London, UK.
Curr Opin Endocrinol Diabetes Obes. 2020 Oct;27(5):283-290. doi: 10.1097/MED.0000000000000567.
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.
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.
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.
探索低碳水化合物生酮饮食（LCKD）在对抗糖原贮积病（GSD）V 和 VII 型患者的身体活动不耐受、疼痛和肌肉损伤方面的潜力，并强调营养可能是关键。
在 GSDV 患者进行身体活动期间以及 GSDVII 患者进行 LCKD 时，摄入碳水化合物（CHO）是常见的。对于后者，一项长期研究表明，在进行 LCKD 时，生理标志物得到改善。这包括有氧能力和活动耐受性的提高。在 GSDV 中，关于 LCKD 的初步研究表明，75%脂肪、15%蛋白质、10%CHO 的饮食最有利于改善功能和依从性。酮体为急性身体活动提供即时燃料，并具有表观遗传作用，可改善酮体和脂质的利用。从精英运动员那里获得的证据表明，LCKD 可以增加脂肪氧化，在 70% VO2max 时效果最佳。这表明需要通过运动来改善身体状况，以最大限度地发挥 LCKD 的益处。
在 GSDV 和 VII 型中，高 CHO 饮食伴随着身体活动能力受限、严重疼痛、肌肉损伤和肾衰竭的风险。越来越多的证据表明，LCKD 对这两种疾病都有效，它提供了即时的燃料来源，可能不需要在每次活动前进行“热身”，并恢复“正常”功能。
