Biochemical assessment of patients following ketogenic diets for epilepsy: Current practice in the UK and Ireland

Schoeler, Natasha E.; Simpson, Zoe; Whiteley, Victoria J; Nguyen, Patty; Meskell, Rachel; Lightfoot, Kathyrn; Martin‐McGill, Kirsty J; Olpin, S. E.; Ivison, Fiona

doi:10.1002/epi4.12371

Cited by 5 publications

(2 citation statements)

References 22 publications

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“…In inherited metabolic diseases such as Leigh syndrome, glucose transporter type 1 (Glut-1) and pyruvate dehydrogenase complex (PDH) deficiency and in a subset of patients with complex I deficiency [ 305 , 308 ], KD has also demonstrated to be useful. Under KD, most patients with epilepsy become seizure free and improve speech, motor, and mental development, and ability [ 316 ] despite its use is only partially able to reverse the clinical course of the neurodegenerative condition [ 317 ].…”

Section: Diet As a Therapymentioning

confidence: 99%

Nutrition, Bioenergetics, and Metabolic Syndrome

et al. 2020

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According to the World Health Organization (WHO), the global nutrition report shows that whilst part of the world’s population starves, the other part suffers from obesity and associated complications. A balanced diet counterparts these extreme conditions with the proper proportion, composition, quantity, and presence of macronutrients, micronutrients, and bioactive compounds. However, little is known on the way these components exert any influence on our health. These nutrients aiming to feed our bodies, our tissues, and our cells, first need to reach mitochondria, where they are decomposed into CO2 and H2O to obtain energy. Mitochondria are the powerhouse of the cell and mainly responsible for nutrients metabolism, but they are also the main source of oxidative stress and cell death by apoptosis. Unappropriated nutrients may support mitochondrial to become the Trojan horse in the cell. This review aims to provide an approach to the role that some nutrients exert on mitochondria as a major contributor to high prevalent Western conditions including metabolic syndrome (MetS), a constellation of pathologic conditions which promotes type II diabetes and cardiovascular risk. Clinical and experimental data extracted from in vitro animal and cell models further demonstrated in patients, support the idea that a balanced diet, in a healthy lifestyle context, promotes proper bioenergetic and mitochondrial function, becoming the best medicine to prevent the onset and progression of MetS. Any advance in the prevention and management of these prevalent complications help to face these challenging global health problems, by ameliorating the quality of life of patients and reducing the associated sociosanitary burden.

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Section: Diet As a Therapymentioning

confidence: 99%

Nutrition, Bioenergetics, and Metabolic Syndrome

et al. 2020

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“…Pyruvate dehydrogenase deficiency has been reported to have a beneficial response to the KD. However, the KD is not appropriate for some patients with conditions of primary carnitine deficiencies and β-oxidation defects due to the inability to metabolize ketone bodies (120). A small percentage of patients have adverse effects like gastrointestinal symptoms and high serum lipids in short-term studies, which do not allow them to follow the diet as a treatment, which suggests that genetic changes are involved (50,117).…”

Section: Ketogenic Dietmentioning

confidence: 99%

Caloric Restriction and Ketogenic Diet Therapy for Epilepsy: A Molecular Approach Involving Wnt Pathway and KATP Channels

et al. 2020

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Epilepsy is a neurological disorder in which, in many cases, there is poor pharmacological control of seizures. Nevertheless, it may respond beneficially to alternative treatments such as dietary therapy, like the ketogenic diet or caloric restriction. One of the mechanisms of these diets is to produce a hyperpolarization mediated by the adenosine triphosphate (ATP)-sensitive potassium (K ATP) channels (K ATP channels). An extracellular increase of K + prevents the release of Ca 2+ by inhibiting the signaling of the Wnt pathway and the translocation of β-catenin to the cell nucleus. Wnt ligands hyperpolarize the cells by activating K + current by Ca 2+. Each of the diets described in this paper has in common a lower use of carbohydrates, which leads to biochemical, genetic processes presumed to be involved in the reduction of epileptic seizures. Currently, there is not much information about the genetic processes implicated as well as the possible beneficial effects of diet therapy on epilepsy. In this review, we aim to describe some of the possible genes involved in Wnt pathways, their regulation through the K ATP channels which are implicated in each one of the diets, and how they can reduce epileptic seizures at the molecular level.

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Biochemical assessment of patients following ketogenic diets for epilepsy: Current practice in the UK and Ireland

et al. 2019

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Objective Biochemical assessment is recommended for patients prior to initiating and following a ketogenic diet (KD). There is no published literature regarding current practice in the UK and Ireland. We aimed to explore practice in comparison with international guidelines, determine approximate costs of biochemical testing in KD patients across the UK and Ireland, and promote greater consistency in KD services nationally. Methods A survey was designed to determine the biochemical tests requested for patients at baseline, 3, 6, 12, 18, and 24 months + on KD. The survey was circulated to 39 centers across the UK and Ireland. Results Sixteen centers completed the survey. Full blood count, electrolytes, calcium, liver function tests (LFTs), lipid profile, and vitamin D were requested at all centers at baseline, in keeping with international guidelines. Bicarbonate, total protein, and urinalysis were less consistently requested. Magnesium and zinc were requested by all centers, despite not being specifically recommended for pre‐diet evaluation in guidelines. Urea and electrolyte profiles and some LFTs were consistently requested at follow‐up, in accordance with guidelines. Other LFTs and renal tests, full blood count, lipid profile, acylcarnitine profile, selenium, vitamin D, and urinalysis were less consistently requested at follow‐up. The mean costs of the lowest and highest number of tests requested at baseline in our participating centers were £167.54 and £501.93; the mean costs of the lowest and highest number of tests requested at 3‐month follow‐up were £19.17 and £450.06. Significance Biochemical monitoring of KD patients varies widely across the UK and Ireland and does not fully correspond to international best practice guidelines. With an ongoing drive for cost‐effectiveness within health care, further work is needed to streamline practice while ensuring patient safety.

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Biochemical assessment of patients following ketogenic diets for epilepsy: Current practice in the UK and Ireland

Cited by 5 publications

References 22 publications

Nutrition, Bioenergetics, and Metabolic Syndrome

Nutrition, Bioenergetics, and Metabolic Syndrome

Caloric Restriction and Ketogenic Diet Therapy for Epilepsy: A Molecular Approach Involving Wnt Pathway and KATP Channels

Biochemical assessment of patients following ketogenic diets for epilepsy: Current practice in the UK and Ireland

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