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

Cansu Kök Şan; Mücahit Muslu; Gülden Fatma Gökçay

doi:10.33808/clinexphealthsci.1310203

Review

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

Year 2024, Volume: 14 Issue: 1, 283 - 295, 28.03.2024

Cansu Kök Şan Mücahit Muslu Gülden Fatma Gökçay

https://doi.org/10.33808/clinexphealthsci.1310203

Abstract

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.

Keywords

Ketogenic diet, modified Atkins diet, glycogen storage disease, mitochondrial disease, GLUT1 deficiency

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Year 2024, Volume: 14 Issue: 1, 283 - 295, 28.03.2024

Cansu Kök Şan Mücahit Muslu Gülden Fatma Gökçay

https://doi.org/10.33808/clinexphealthsci.1310203

Abstract

References

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Details

Primary Language	English
Subjects	Nutritional Science, Pediatric Metabolism Diseases
Journal Section	Review
Authors	Cansu Kök Şan 0000-0002-4754-1635 Mücahit Muslu 0000-0002-8761-5061 Gülden Fatma Gökçay 0000-0003-3726-5726
Early Pub Date	March 23, 2024
Publication Date	March 28, 2024
Submission Date	June 5, 2023
Published in Issue	Year 2024 Volume: 14 Issue: 1

Cite

APA	Kök Şan, C., Muslu, M., & Gökçay, G. F. (2024). Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article. Clinical and Experimental Health Sciences, 14(1), 283-295. https://doi.org/10.33808/clinexphealthsci.1310203
AMA	Kök Şan C, Muslu M, Gökçay GF. Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article. Clinical and Experimental Health Sciences. March 2024;14(1):283-295. doi:10.33808/clinexphealthsci.1310203
Chicago	Kök Şan, Cansu, Mücahit Muslu, and Gülden Fatma Gökçay. “Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article”. Clinical and Experimental Health Sciences 14, no. 1 (March 2024): 283-95. https://doi.org/10.33808/clinexphealthsci.1310203.
EndNote	Kök Şan C, Muslu M, Gökçay GF (March 1, 2024) Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article. Clinical and Experimental Health Sciences 14 1 283–295.
IEEE	C. Kök Şan, M. Muslu, and G. F. Gökçay, “Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article”, Clinical and Experimental Health Sciences, vol. 14, no. 1, pp. 283–295, 2024, doi: 10.33808/clinexphealthsci.1310203.
ISNAD	Kök Şan, Cansu et al. “Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article”. Clinical and Experimental Health Sciences 14/1 (March 2024), 283-295. https://doi.org/10.33808/clinexphealthsci.1310203.
JAMA	Kök Şan C, Muslu M, Gökçay GF. Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article. Clinical and Experimental Health Sciences. 2024;14:283–295.
MLA	Kök Şan, Cansu et al. “Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article”. Clinical and Experimental Health Sciences, vol. 14, no. 1, 2024, pp. 283-95, doi:10.33808/clinexphealthsci.1310203.
Vancouver	Kök Şan C, Muslu M, Gökçay GF. Ketogenic Diet Interventions in Inborn Errors of Metabolism: A Review Article. Clinical and Experimental Health Sciences. 2024;14(1):283-95.

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