Can a Low-Carbohydrate Diet Improve Exercise Tolerance in Mcardle Disease?

Sl, Reason; Ec, Westman; Godfrey, Richard; Maguire, Emily

doi:10.21767/2380-7245.100054

Cited by 4 publications

(5 citation statements)

References 11 publications

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“…LCKD has shown promise as a treatment option for GSD V. A recent pilot study found that a modified ketogenic diet composed of a minimum of 75% fat and a maximum of 10% carbohydrates seemed to improve exercise capacity and improve GSD V-related symptoms [6,51]. This is in line with published case series [66,89], which also showed promising effects, and with patient experiences published in an IamGSD survey [90].…”

Section: Lckd In Gsd Vsupporting

confidence: 69%

Clinical practice guidelines for glycogen storage disease V & VII (McArdle disease and Tarui disease) from an international study group

Lucía¹,

Martinuzzi

Nogales‐Gadea

et al. 2021

Neuromuscular Disorders

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Section: Lckd In Gsd Vsupporting

confidence: 69%

Clinical practice guidelines for glycogen storage disease V & VII (McArdle disease and Tarui disease) from an international study group

Lucía¹,

Martinuzzi

Nogales‐Gadea

et al. 2021

Neuromuscular Disorders

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“…In addition, plasma creatine kinase (CK) was significantly lowered in all three cases. Overall, all three patients described an improved QoL [10].…”

Section: Role Of a Patient Advocacy Organization In Informing Researchmentioning

confidence: 84%

“…Over the past 5 years, numerous patients with GSDV have adopted a LCKD and anecdotally reported a distinct improvement in PA and exercise tolerance, especially pre second-wind [10]. Harnessing the use of alternative fuels, ketone bodies (KB) for immediate use, and fats from the 10 min mark onward may be a reasonable solution for this patient population [7 ▪▪ ].…”

Section: Patient Perspectivementioning

confidence: 99%

“…Harnessing the use of alternative fuels, ketone bodies (KB) for immediate use, and fats from the 10 min mark onward may be a reasonable solution for this patient population [7 ▪▪ ]. KBs do not require energetic transport across the mitochondrial membrane, and therefore may help to address the energy crisis before free fatty acids (FFA) become readily available in second-wind [10].…”

Section: Patient Perspectivementioning

confidence: 99%

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International patient group harnesses social media to help inform rare disease research: use of a low carbohydrate ketogenic diet in McArdle disease

Reason¹,

Løkken

Voermans

2021

Current Opinion in Endocrinology, Diabetes &Amp; Obesity

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Purpose of review To highlight the benefits of a partnership between patient advocacy organizations (PAO) and clinical researchers in order to clinically study the prior anecdotal patient experience of utilizing a low-carbohydrate ketogenic diet (LCKD) to manage McArdle disease. Recent findings The bedside-to-bench and back again method of translational research is well suited to explore anecdotal patient experiences, particularly for rare diseases. In McArdle disease, patients have explored the use of a LCKD to address the ubiquitous occurrence of physical activity intolerance and minimize associated adverse events. The International Association for Muscle Glycogen Storage Disease harnessed the power of social media to better understand this patient-centric finding and has subsequently partnered with clinical researchers to convey patient-centered priorities for research. From this partnership, a series of clinical and survey studies have been initiated. Summary Collaboration between PAOs and clinical researchers has the potential to foster patient empowerment, advance anecdotal experiences into scientific hypotheses, and ultimately guide the development of management guidelines that reflect the patient perspective.

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“…However, 31-phosphorus magnetic resonance spectroscopy data did not improve during rest, work, or recovery. In three adult GSD V patients, a low-carbohydrate KD was similarly reported to improve activity and exercise tolerance, lower CK levels, and improve quality of life (28).…”

Section: Swoboda Et Al (32)mentioning

confidence: 88%

Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article

Kök Şan,

Muslu,

Gökçay

2024

Clinical and Experimental Health Sciences

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Objective: The ketogenic diet, which has been used in the treatment of epilepsy since the 1920s, is a diet containing high fat, sufficient protein, and low carbohydrate. The ketogenic diet mimics the metabolic effects of fasting by shifting metabolism towards fat utilization. The ketogenic diet, which has different variants, such as the classical ketogenic diet, modified Atkins diet, and medium-chain triglyceride diet, is used in inborn errors of metabolism to target the underlying metabolic state by bypassing the damaged metabolic pathway or to treat the clinical symptoms of inborn errors of metabolism, such as epileptic seizures. In this review, we assessed the evidence for ketogenic diet interventions in the treatment of inborn errors of metabolism. Methods: The Google Scholar search engine, PubMed, Scopus, and Science Direct databases were used to find studies on the use of ketogenic diet interventions in the treatment of inborn errors of metabolism. Results: The beneficial effects of different variants of the ketogenic diet on glucose transport type 1 deficiency syndrome and pyruvate dehydrogenase complex deficiency have long been recognized. There are also favorable data on its use in myopathic glycogen storage diseases, mitochondrial diseases, and nonketotic hyperglycinemia accompanied by epilepsy. Conclusion: The evidence is mostly based on individual case reports, case series, and clinical trials with small sample sizes and is insufficient to make recommendations.

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Can a Low-Carbohydrate Diet Improve Exercise Tolerance in Mcardle Disease?

Cited by 4 publications

References 11 publications

Clinical practice guidelines for glycogen storage disease V & VII (McArdle disease and Tarui disease) from an international study group

Clinical practice guidelines for glycogen storage disease V & VII (McArdle disease and Tarui disease) from an international study group

International patient group harnesses social media to help inform rare disease research: use of a low carbohydrate ketogenic diet in McArdle disease

Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article

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