Google Scholar | Citations: 16429| h-index: 32 | i10-index: 482
Non-ketotic hyperglycinemia using a ketogenic diet: case report
PDF (Portuguese (Brazil))

Keywords

Ketogenic Diet
Non-Ketotic Hyperglycinemia
Seizures
Hypokalemia

How to Cite

Non-ketotic hyperglycinemia using a ketogenic diet: case report. (2024). Electronic Journal Collection Health, 24(2), e14487. https://doi.org/10.25248/reas.e14487.2024

Copyright © | All rights reserved.

The journal holds the exclusive copyright for the publication of this article under the terms of Brazilian law 9610/98.

Partial reproduction
The use of parts of the texts, figures and questionnaire of the article is free, being mandatory the citation of the authors and journal.

Total reproduction
It is expressly prohibited and must be authorized by the journal.

Abstract

Objective: To report a case study on non-ketotic hyperglycinemia and the nutritional treatment prescribed with a ketogenic diet. Case detail: Female patient, 3 years old, born in Cajazeiras-PB. She was admitted to a University Hospital (EBSERH) in the city with excessive sleepiness, associated with worsening seizures. In the first days of hospitalization, the patient used a 4:1 diet similar to the one she was eating at home. On the fourth day of potassium replacement, the blood levels of this mineral reduced significantly even though the replacement therapy had not ceased, which led to the suspicion that the 4:1 ketogenic diet could be influencing the condition. Thus, the diet was involution changing to 3:1 and then 2:1 and at this concentration the patient was able to maintain normal potassium levels and was discharged. Final considerations: It is considered that the ketogenic diet presented itself as an important nutritional strategy in controlling seizures in non-ketotic hyperglycinemia, however the prolonged use of this diet, especially at concentrations of 4g of fat for 1 g of carbohydrate, was related to the development of hypokalemia.

PDF (Portuguese (Brazil))

References

BOAVENTURA V, et al Dieta cetogênica para pacientes adultos com epilepsia: um guia prático. ACM arq. catarin. med, 2019.

DOS SANTOS DK, et al. Utilização da dieta cetogênica como estratégia para o manejo de pacientes com epilepsia refratária: uma revisão da literatura. International J of Health Management Review, 2019; 5: 1.

GONÇALVES CM, et al. Hiperglicinemia não cetótica: relato de caso. Revista Médica de Minas Gerais, 2008; 3(18): 204-207.

GENÇ SÇ, et al. Nonketotic hyperglycinemia: Clinical range and outcome of a rare neurometabolic disease in a single-center. Brain Dev., 2018; 40(10): 865-875.

HOOVER-FONG JE, et al. Natural history of nonketotic hyperglycinemia in 65 patients. Neurology. 2004; 63(10): 1847-53.

KAVA MP, et al. Ketogenic diet, a potentially valuable therapeutic option for the management of refractory epilepsy in classical neonatal nonketotic hyperglycinemia: a case report. Eur J Clin Nutr., 2019; 73(6): 961-965.

KLASSEN PN, et al. Ketogenic and low-sugar diets for patients with cancer: perceptions and practices of medical oncologists in Canada. Supportive Care in Cancer, 2020; 28(11): 5243–5249.

KOSE E, et al. A novel mutation in the glycine decarboxylase gene in patient with non-ketotic hyperglycinemia. Neurosciences (Riyadh), 2017; 22(2): 131-133.

MCARTNEY R, et. What is a ketogenic diet and how does it affect the use of medicines? Arch Dis Child Educ Pract Ed, 2017; 102: 194-99.

MULLIGAN JL. Neonatal nonketotic hyperglycinemia: a case study and review of management of the advanced practice nurse. Neonatal Netw., 2013; 2(32): 95-103.

NERI LCL, et al. Projeto piloto de implantação de uma cozinha cetogênica para tratamento de epilepsia refratária. REVISA - Revista de Divulgação Científica Sena Aires, 2021; 10(1): 156-164.

NONINO-BORGES CB, et al. Dieta cetogênica no tratamento de epilepsias farmacorresistentes. Rev Nutr., 2004; 17(4): 515–21.

OLIVEIRA SANTOS J, et al. Hiperglicinemia não cetótica – relato de caso. Perspectivas Experimentais E Clínicas, Inovações Biomédicas E Educação Em Saúde (PECIBES), 2019; 5(2): 11.

PEREIRA ÉS, et al. Dieta cetogênica: como o uso de uma dieta pode interferir em mecanismos neuropatológicos. Revista De Ciências Médicas E Biológicas, 2010; 9(1): 78–82.

POOTHRIKOVIL RP, et al. Nonketotic Hyperglycinemia: Two Case Reports and Review. Neurodiagn J., 2019; 59(3): 142-151.

RIZZUTTI S, et al. Avaliação do perfil metabólico nutricional e efeitos adversos de crianças com epilepsia refratária em uso da dieta cetogênica. Rev. Nutr, 2006; 9(5): 573-579.

ROLA M e VASCONCELOS C. Dieta Cetogénica - Abordagem Nutricional. Revista Nutrícias, 2014; 22(22): 16–19.

SAMPAIO LPB. ABC da dieta cetogênica para epilepsia refratária / Letícia Pereira de Brito Sampaio – Rio de Janeiro: Editora DOC Content, 2018. 1ª edição – 220p.

SCHWARTZ IV, et al. Tratamento de erros inatos do metabolismo. J. Pediatr. (Rio J.), Porto Alegre, 2008; 84(4)supl: S8-S19.

SHBAROU RM, et al. Outcome of Nonketotic Hyperglycinemia in Lebanon: 14-Year Retrospective Review. Neuropediatrics. 2019; 50(4): 235-243.

WELLS J, et al. Efficacy and safety of a ketogenic diet in children and adolescents with refractory epilepsy—a review. Nutrients, 2020; 12(6): 1–19.