Polyunsaturated fatty acid-enriched diet therapy for a child with epilepsy

Yoon, Jung Rim; Lee, Eun Joo; Kim, Heung Dong; Lee, Jae‐Hwan; Kang, Hoon Chul

doi:10.1016/j.braindev.2013.01.017

Cited by 19 publications

(17 citation statements)

References 10 publications

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“…In the Deletor mouse (a mouse model for mitochondrial diseases, with mutations in the Twinkle helicase which results in multiple mtDNA deletions) a decrease in COX-negative fibres, improved mitochondrial ultrastructure and increased mitochondrial biogenesis were also observed (Ahola-Erkkila et al 2010). The few clinical reports of the use of KD or high fat diet in complex I deficiency noted improvement in seizures (Kang et al 2006; Seo et al 2010; Yoon et al 2014), and transient improvement of oculomotor palsy in one patient with early-onset Leigh-like syndrome due to NDUFV1 mutations (Laugel et al 2007). However, long-term follow up and treatment of larger cohorts has not been reported, and formal clinical trials are required to provide evidence of efficacy (Rahman 2012).…”

Section: Introductionmentioning

confidence: 99%

The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome

Kanabus¹,

Fassone²,

Hughes

et al. 2016

J of Inher Metab Disea

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There is growing interest in the use of the ketogenic diet (KD) to treat inherited metabolic diseases including mitochondrial disorders. However, neither the mechanism whereby the diet may be working, nor if it could benefit all patients with mitochondrial disease, is known. This study focusses on decanoic acid (C10), a component of the medium chain triglyceride KD, and a ligand for the nuclear receptor PPAR-γ known to be involved in mitochondrial biogenesis. The effects of C10 were investigated in primary fibroblasts from a cohort of patients with Leigh syndrome (LS) caused by nuclear-encoded defects of respiratory chain complex I, using mitochondrial respiratory chain enzyme assays, gene expression microarray, qPCR and flow cytometry. Treatment with C10 increased citrate synthase activity, a marker of cellular mitochondrial content, in 50 % of fibroblasts obtained from individuals diagnosed with LS in a PPAR-γ-mediated manner. Gene expression analysis and qPCR studies suggested that treating cells with C10 supports fatty acid metabolism, through increasing ACADVL and CPT1 expression, whilst downregulating genes involved in glucose metabolism (PDK3, PDK4). PCK2, involved in blocking glucose metabolism, was upregulated, as was CAT, encoding catalase. Moreover, treatment with C10 also decreased oxidative stress in complex I deficient (rotenone treated) cells. However, since not all cells from subjects with LS appeared to respond to C10, prior cellular testing in vitro could be employed as a means for selecting individuals for subsequent clinical studies involving C10 preparations.Electronic supplementary materialThe online version of this article (doi:10.1007/s10545-016-9930-4) contains supplementary material, which is available to authorized users.

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Section: Introductionmentioning

confidence: 99%

The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome

Kanabus¹,

Fassone²,

Hughes

et al. 2016

J of Inher Metab Disea

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“…25 Os outros alimentos não obtiveram um consumo significativo, mas apresentam importantes capacidades anti-inflamatórias que devem ser destacadas, como a Salvia hispanica L., mais conhecida como chia, e o óleo de canola merecem destaque, pois possuem, além de muitas outras propriedades, a característica de conter ômega-3, famoso por sua capacidade anti-inflamatória. 26,27 O chá verde, se consumido habitualmente, seria de grande valia para esses pacientes, pois tem grandes quantidades de polifenóis e potente capacidade antioxidante e anti-inflamatória. 28 A goiaba, que é rica em polifenóis, com capacidade antioxidante, antimicrobiana e anti-inflamatória, foi consumida por apenas 11% dos participantes.…”

Section: Resultados E Discussõesunclassified

Perfil Nutricional E Consumo De Alimentos Inflamatórios E Anti-Inflamatórios De Pacientes Atendidos No Ambulatório De Psoríase De Uma Unidade De Saúde-Escola De Itajaí, Sc

Odierno

Coelho

Matos

2015

DEMETRA

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Nutritional profile and consumption of inflammatory and anti-inflammatory food of patients treated in an outpatient clinic of psoriasis health-school unit in Itajaí, SC, Brazil Resumo A psoríase é uma doença inflamatória de pele, mediada por células T, hereditária, que pode sofrer influências ambientais. A psoríase e obesidade possuem uma fisiopatologia subjacente comum, sugerindo que obesidade segue psoríase, não a precede; além disto, há evidências de que determinados nutrientes, com função anti-inflamatória, podem auxiliar no tratamento da psoríase. O presente estudo objetivou avaliar o estado nutricional e o consumo de alimentos inflamatórios e anti-inflamatórios de pacientes com diagnóstico de psoríase atendidos em uma Unidade de Saúde-Escola de Itajaí-SC, de maio a agosto de 2014. Os pacientes atendidos foram avaliados por meio de um questionário semiestruturado, com dados de identificação, socioeconômicos, hábitos de vida e frequência do consumo de alimentos (inflamatório e anti-inflamatórios), sendo foi realizada avaliação do estado nutricional. A análise estatística foi efetuada por meio do programa STATA 13.0 e a associação entre as variáveis categóricas foi avaliada pelo teste do qui-quadrado de Pearson ou teste exato de Fisher. O excesso de peso prevaleceu em 61% dos pacientes, sendo 22% com sobrepeso e 39% com obesidade. Os resultados demonstram que o alho (97%) e o azeite de oliva (53%) foram os alimentos anti-inflamatórios consumidos com maior frequência, enquanto que os demais alimentos apresentaram baixo consumo. Quando questionados sobre o consumo de alimentos inflamatórios, o mais citado foi carne de gado, com 58%, seguida pelo café (45%); os demais alimentos não demonstraram relevância no consumo. Diante da magnitude do tema estudado, torna-se essencial o desenvolvimento de pesquisas e estudos

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“…In a 5-month-old girl with Ohtahara syndrome associated with complex I deficiency Bseizures were completely controlled and suppression-burst patterns disappeared three months after starting^KD (2:1) and mitochondrial cocktail supplementation (n=1; Seo et al 2010). Recently, Yoon et al reported on a B7-year-old boy with Lennox-Gastaut syndrome combined with mitochondrial respiratory chain complex I deficiency, whose medically intractable seizures were successfully brought under control with a PUFA-enriched MAD without any significant adverse events( n=1; Yoon et al 2014).…”

Section: Disorders Of the Mitochondrial Respiratory Chain (Mrc)mentioning

confidence: 95%

Ketogenic diets in patients with inherited metabolic disorders

Scholl‐Bürgi

Höller

Pichler

et al. 2015

J of Inher Metab Disea

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Ketogenic diets (KDs) are diets that bring on a metabolic condition comparable to fasting, usually without catabolism. Since the mid-1990s such diets have been widely used in patients with seizures/epilepsies, mostly children. This review focuses on the use of KDs in patients with various inherited metabolic disorders (IMD). In glucose transporter type 1 deficiency syndrome (GLUT1-DS) and pyruvate dehydrogenase complex (PDHc) deficiency, KDs are deemed the therapy of choice and directly target the underlying metabolic disorder. Moreover, in other IMD, mainly of intermediary metabolism such as glycogen storage diseases and disorders of mitochondrial energy supply, KDs may ameliorate clinical symptoms and laboratory parameters. KDs have also been used successfully to treat symptoms such as seizures/epilepsy in IMD, e.g. in urea cycle disorders and non-ketotic hyperglycinemia. As a note of caution, catabolism may cause the condition of patients with IMD to deteriorate and should thus be avoided during KDs. For this reason, careful monitoring (clinical, laboratory and apparatus-supported) is warranted. In some IMDs specific macronutrient supply is critical. Therefore, in cases of PDHc deficiency the carbohydrate intake tolerated without lactate increase and in urea cycle disorders the protein tolerance should be determined. Considering this, it is particularly important in patients with IMD that the use of KDs be individualized and well documented.

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Polyunsaturated fatty acid-enriched diet therapy for a child with epilepsy

Cited by 19 publications

References 10 publications

The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome

The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome

Perfil Nutricional E Consumo De Alimentos Inflamatórios E Anti-Inflamatórios De Pacientes Atendidos No Ambulatório De Psoríase De Uma Unidade De Saúde-Escola De Itajaí, Sc

Ketogenic diets in patients with inherited metabolic disorders

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