[Fourteenth Jesús Culebras Lecture. Ketogenic diet, a half-discovered treatment]. / Decimocuarta Lección Jesús Culebras. Dieta cetogénica, un tratamiento a medio descubrir.

Introduction: The ketogenic diet was an amazing approach to treating epilepsy from its beginning. The body undergoes a change in obtaining energy, going from depending on carbohydrates to depending on fats, and then a whole series of biochemical routes are launched that, independently but also complementary, give rise to a set of effects that benefit the patient. This search for its mechanism of action, of devising how to improve compliance and take advantage of it for other diseases has marked its trajectory. This article briefly reviews these aspects, emphasizing the importance of continuing to carry out basic and clinical research so that this treatment can be applied with solid scientific bases.

Introducción: La dieta cetogénica constituyó desde su inicio un planteamiento sorprendente para el tratamiento de la epilepsia. Someter al organismo a un cambio en la obtención de energía, pasando de depender de los carbohidratos a hacerlo de las grasas, pone en marcha toda una serie de rutas bioquímicas que, de forma independiente pero también complementaria, dan lugar a un conjunto de efectos que benefician al paciente. Esta búsqueda de su mecanismo de acción, de idear cómo mejorar el cumplimiento y de aprovecharla para otras enfermedades ha marcado su trayectoria. En este artículo se revisan someramente estos aspectos, haciendo hincapié en la importancia de seguir realizando investigación básica y clínica para que este tratamiento pueda aplicarse con bases científicas sólidas.

[Translated article] Popular Diets and Skin Effects: A Narrative Review.

Numerous diets for losing weight, building strength, and managing a range of cardiovascular, neurologic, and skin diseases have become popular in recent years. The ketogenic diet and intermittent fasting in particular have shown promising results in clinical and sports medicine. The Mediterranean diet, in turn, is widely recognized for its numerous health benefits. Also popular are the paleo diet and vegan and gluten-free diets. Positive effects on inflammatory conditions, such as psoriasis, atopic dermatitis, hidradenitis suppurativa, and acne, have been observed in patients who practice intermittent fasting or follow ketogenic or Mediterranean diets. This last choice may also protect against certain skin cancers. We review the role of several popular diets in the management of skin disorders.

Ketogenic diet in pharmacoresistant epilepsies: a clinical nutritional assessment / Dieta cetogênica na epilepsia fármaco-resistente: avaliação clínica-nutricional

Abstract Background Epilepsies are among the most prevalent chronic neurological diseases, usually beginning in childhood. About 30% of children with epilepsies develop seizures that are difficult to control with medication. Recurrent epileptic seizures hinder diet intake, impairing the nutritional status. Although non-pharmacological interventions (e.g., ketogenic diet therapy) can improve epileptic seizure frequency, few studies analyzed their impact on the nutritional status of children and adolescents with epilepsies. Objective The aim was to evaluate the effects of a ketogenic diet on the nutritional status and clinical course of patients with pharmacoresistant epilepsies. Methods This cross-sectional study included patients under 18 years of age followed up at the Ketogenic Diet Ambulatory Clinic of the Instituto de Medicina Integral Prof. Fernando Figueira between December 2015 and December 2021. Socioeconomic, clinical, nutritional, and laboratory data were collected from medical records at different time points during the ketogenic diet. Results The sample comprised 49 patients aged between 5 months and 17 years (median = 4.4 years), mostly male (62.1%), and from Recife and the metropolitan region (51%). Underweight patients (BMI-for-age) improved their nutritional status in six months. However, patients who were normal weight and overweight maintained their nutritional status. Dyslipidemia was a common and short-term adverse effect. Moreover, the treatment decreased epileptic seizure frequency and antiseizure medication intake. Conclusion The ketogenic diet prevented malnutrition from worsening and reduced epileptic seizures and antiseizure medication intake.

Resumo Antecedentes A epilepsia, uma das doenças neurológicas crônicas mais prevalentes, tem geralmente início na infância. Cerca de 30% das crianças com epilepsia desenvolvem crises de difícil controle medicamentoso. As crises epilépticas recorrentes dificultam a ingestão alimentar, prejudicando o estado nutricional. Intervenções não farmacológicas, como a terapia com dieta cetogênica, podem melhorar a frequência das crises epilépticas, mas existem poucos estudos sobre a repercussão no estado nutricional da criança/adolescente. Objetivo Avaliar o efeito da terapia cetogênica sobre o estado nutricional e a evolução clínica da epilepsia fármaco-resistente. Métodos Estudo tipo corte transversal envolvendo menores de 18 anos acompanhados no Ambulatório de Dieta Cetogênica do Instituto de Medicina Integral Prof. Fernando Figueira entre dezembro de 2015 e dezembro de 2021. Dados socioeconômicos, clínicos, nutricionais e laboratoriais foram coletados nos prontuários dos pacientes em vários momentos da terapia cetogênica. Resultados A amostra foi composta por 49 pacientes com idades entre cinco meses e 17 anos (mediana = 4,4 anos), a maioria do sexo masculino (62,1%) e procedentes de Recife e região metropolitana (51%). Pacientes com baixo peso (de acordo com o IMC para idade) melhoraram seu estado nutricional em seis meses. No entanto, os pacientes com peso adequado e com sobrepeso mantiveram seu estado nutricional. A dislipidemia foi um efeito adverso frequente e de curta duração. Além disso, o tratamento reduziu a frequência de crises epilépticas e a dose de fármacos anticrises. Conclusão A dieta cetogênica preveniu o agravamento da desnutrição e reduziu as crises epilépticas e a dosagem de fármacos anticrises.

Popular Diets and Skin Effects: A Narrative Review. / Dietas populares y su impacto en la piel. Una revisión narrativa.

Numerous diets for losing weight, building strength, and managing a range of cardiovascular, neurologic, and skin diseases have become popular in recent years. The ketogenic diet and intermittent fasting in particular have shown promising results in clinical and sports medicine. The Mediterranean diet, in turn, is widely recognized for its numerous health benefits. Also popular are the paleo diet and vegan and gluten-free diets. Positive effects on inflammatory conditions, such as psoriasis, atopic dermatitis, hidradenitis suppurativa, and acne, have been observed in patients who practice intermittent fasting or follow ketogenic or Mediterranean diets. This last choice may also protect against certain skin cancers. We review the role of several popular diets in the management of skin disorders.

ESTUDIO DEL IMPACTO DE LA DIETA CETOGÉNICA EN LA CALIDAD DE VIDA DE PACIENTES PEDIÁTRICOS CON EPILEPSIA Y SUS FAMILIAS / STUDY OF THE IMPACT OF THE KETOGENIC DIET ON THE QUALITY OF LIFE OF PEDIATRIC PATIENTS WITH EPILEPSY AND THEIR FAMILIES

Introducción: El tratamiento mediante los distintos tipos de dietas cetogénicas ha demostrado en diversos trabajos efectividad en la reducción de las crisis convulsivas, de manera total o parcial. Sin embargo, se reportan tasas de deserción en la realización a largo plazo. Objetivo: Este trabajo tiene como objetivo identificar beneficios y dificultades que perciben las familias de niños en el tratamiento de Epilepsia resistente a fármacos que lleven a cabo la dieta cetogénica y que podrían influir en la adherencia o deserción. Materiales y métodos: Se realizó un estudio descriptivo transversal retrospectivo, en el Servicio de Neurología Infantil del HIBA, sección Dieta Cetogénica. Para el estudio, se realizó una encuesta para recabar características sociodemográficas de la población bajo estudio y también sobre dificultades y beneficios hallados en la terapia cetogénica. Se analizaron frecuencias, medias y chi cuadrado para establecer diferencias significativas con intervalo de confianza de 95%. Resultados: La muestra estuvo compuesta por 51 participantes, de 0 a 18 años. Al indagar sobre si percibían mejoras tras realizar terapia cetogénica, el 81% respondió que sí, el 11% no percibió mejoras y el 8% no sabía. Acerca de la presencia de dificultades para llevar a cabo el tratamiento, el 71% respondió que se encontraba con dificultades, el 23% no registró dificultades y el 6% manifestó que a veces. Al relacionar la presencia de dificultades con el nivel socioeconómico, se observaron diferencias significativas entre los grupos, siendo de mayor incidencia en familias de ingresos más bajos. Respecto de la variable edad, no se encontraron diferencias significativas en relación con las dificultades percibidas. Conclusiones: Es importante continuar trabajando con las familias en las dificultades que presentan para evitar deserción del tratamiento. Asimismo, resulta indispensable destacar el alto porcentaje de mejorías que perciben sus padres para lograr mayor adherencia

Introduction: Treatment using the different types of ketogenic diets has been shown in various studies to be effective in reducing seizures, which can be total or partial. However, dropout rates are reported in the long-term completion of it. Objetive: The objective of this work is to identify the benefits and difficulties perceived by the families of children undergoing treatment for drug-resistant epilepsy who follow the ketogenic diet and that could influence adherence or desertion. Materials and methods: A retrospective cross-sectional descriptive study was carried out at the HIBA Child Neurology Service, Ketogenic Diet section. For the study, a survey was carried out to collect sociodemographic characteristics of the population under study and also about difficulties and benefits found in ketogenic therapy. Frequencies, measures and chi square were analyzed to establish significant differences with a 95% confidence interval. Results: The sample consisted of 51 participants, from 0 to 18 years old. When asked if they perceived improvements after performing ketogenic therapy, 81% answered yes, 11% did not perceive improvements and 8% did not know. About the presence of difficulties to carry out the treatment, 71% answered that they had difficulties, 23% did not have them and 6% sometimes.When relating the presence of difficulties with the socioeconomic level, significant differences were observed between the groups, with a higher incidence in lower-income families. Regarding the age variable, no significant differences were found in relation to the perceived difficulties. Conclusion: It is important to continue working with families on the difficulties they present to avoid dropping out of treatment. Likewise, it is essential to highlight the high percentage of improvements that their parents perceive to achieve greater adherence

Ketogenic therapy in childhood and adolescence: recommendations of the Brazilian experts group / Terapia cetogênica na infância e adolescência: recomendações do grupo de especialistas brasileiros

Abstract Ketogenic dietary therapies (KDTs) are a safe and effective treatment for pharmacoresistant epilepsy in children. There are four principal types of KDTs: the classic KD, the modified Atkins diet (MAD), the medium-chain triglyceride (MCT) diet, and the low glycemic index diet (LGID). The International Ketogenic Diet Study Group recommends managing KDTs in children with epilepsy. However, there are no guidelines that address the specific needs of the Brazilian population. Thus, the Brazilian Child Neurology Association elaborated on these recommendations with the goal of stimulating and expanding the use of the KD in Brazil.

Resumo As terapias dietéticas cetogênicas (TDC) são um tratamento seguro e eficaz para epilepsia farmacorresistente em crianças. Existem quatro tipos principais de TDCs: a dieta cetogênica (DC) clássica, a dieta de Atkins modificada (DAM), a dieta de triglicerídeos de cadeia média (DTCM) e a dieta de baixo índice glicêmico (DBIG). O Grupo Internacional de Estudos de Dietas Cetogênicas (International Ketogenic Diet Study Group) propõe recomendações para o manejo da DC em crianças com epilepsia. No entanto, faltam diretrizes que contemplem as necessidades específicas da população brasileira. Assim, a Associação Brasileira de Neurologia Infantil elaborou essas recomendações com o objetivo de estimular e expandir o uso da DC no Brasil.

Dieta cetogénica y su influencia en el tratamiento de la epilepsia refractaria infantil / Ketogenic diet and its influence on the treatment of refractory epilepsy in children / Dieta cetogênica e sua influência no tratamento da epilepsia refratária em crianças

La dieta cetogénica durante muchos años utilizada como una alternativa en el tratamiento para la epilepsia refractaria infantil, que consiste en un alto consumo de grasas, reducidas cantidades de carbohidratos y las cantidades necesarias de proteína que permitan al paciente por medio de la producción de cetonas un correcto y adecuado aporte energético al cerebro y todo el SNC. Objetivo. Describir el uso de la dieta cetogénica en el tratamiento de la epilepsia refractaria infantil para determinar su efectividad en la reducción de convulsiones y mejora en la calidad de vida de los pacientes. Metodología. Se ha realizado una revisión sistemática de la literatura científica para examinar la relación entre la dieta cetogénica y su efecto en el tratamiento de la epilepsia refractaria infantil. Para identificar y seleccionar las fuentes de información, se aplicó la declaración PRISMA y se llevó a cabo una búsqueda bibliográfica exhaustiva en diversas bases de datos electrónicas, incluyendo PubMed, SCOPUS y SCIELO. Se utilizaron términos descriptores específicos como "dieta cetogénica", "influencia", "tratamiento", "epilepsia refractaria" y "niños", así como conectores como AND, OR o NOT para mejorar la precisión de la búsqueda. Conclusión. La dieta cetogénica ha demostrado ser efectiva en el tratamiento de la epilepsia refractaria al reducir en un 50% el número de crisis convulsivas y mejorar la cognición de los pacientes, lo que la convierte en una opción viable para el manejo de esta afección. Sin embargo, es importante tener en cuenta que la dieta cetogénica también puede tener efectos secundarios.

The ketogenic diet has been used for many years as an alternative in the treatment of refractory epilepsy in children, which consists of a high consumption of fats, reduced amounts of carbohydrates and the necessary amounts of protein to allow the patient, through the production of ketones, a correct and adequate energy supply to the brain and the entire CNS. Objective. To describe the use of the ketogenic diet in the treatment of refractory epilepsy in children to determine its effectiveness in reducing seizures and improving the quality of life of patients. Methodology. A systematic review of the scientific literature was conducted to examine the relationship between the ketogenic diet and its effect in the treatment of refractory epilepsy in children. To identify and select sources of information, the PRISMA statement was applied and a comprehensive literature search was conducted in various electronic databases, including PubMed, SCOPUS and SCIELO. Specific descriptor terms such as "ketogenic diet", "influence", "treatment", "refractory epilepsy" and "children" were used, as well as connectors such as AND, OR or NOT to improve search precision. Conclusion. The ketogenic diet has been shown to be effective in the treatment of refractory epilepsy by reducing the number of seizures by 50% and improving patients' cognition, making it a viable option for the management of this condition. However, it is important to keep in mind that the ketogenic diet can also have side effects.

A dieta cetogênica tem sido usada há muitos anos como um tratamento alternativo para epilepsia refratária em crianças, consistindo em um alto consumo de gorduras, quantidades reduzidas de carboidratos e as quantidades necessárias de proteínas para permitir ao paciente, por meio da produção de cetonas, um fornecimento correto e adequado de energia para o cérebro e todo o SNC. Objetivo. Descrever o uso da dieta cetogênica no tratamento da epilepsia refratária em crianças, a fim de determinar sua eficácia na redução das crises e na melhoria da qualidade de vida dos pacientes. Metodologia. Foi realizada uma revisão sistemática da literatura científica para examinar a relação entre a dieta cetogênica e seu efeito no tratamento da epilepsia refratária em crianças. Para identificar e selecionar as fontes de informação, foi aplicada a declaração PRISMA e realizada uma pesquisa abrangente da literatura em vários bancos de dados eletrônicos, incluindo PubMed, SCOPUS e SCIELO. Foram usados termos descritores específicos, como "dieta cetogênica", "influência", "tratamento", "epilepsia refratária" e "crianças", além de conectores como AND, OR ou NOT para melhorar a precisão da pesquisa. Conclusão. A dieta cetogênica demonstrou ser eficaz no tratamento da epilepsia refratária, reduzindo o número de convulsões em 50% e melhorando a cognição dos pacientes, o que a torna uma opção viável para o tratamento dessa condição. Entretanto, é importante observar que a dieta cetogênica também pode ter efeitos colaterais.

Deficiencia del transportador de glucosa tipo 1: aspectos clínicos, diagnóstico y terapéutica / Glucose transporter type 1 deficiency syndrome: clinical aspects, diagnosis, and treatment

El síndrome de deficiencia del transportador de glucosa tipo 1 es una enfermedad de causa genética, que involucra el gen SLC2A1. En general, se presenta durante los primeros años de vida con retraso en la adquisición de pautas madurativas, epilepsia farmacorresistente y desórdenes del movimiento. La clínica y la disminución de glucosa en líquido cefalorraquídeo permiten sospechar el diagnóstico, el cual debe ser confirmado mediante el estudio molecular del gen SLC2A1. Debido a que se trata de una enfermedad poco frecuente y de expresión clínica variable, el diagnóstico precoz suele representar un desafío para los equipos de salud. Este es importante, ya que la implementación de la terapia cetogénica logra controlar las manifestaciones clínicas y mejora el pronóstico a largo plazo. Presentamos una revisión sobre el déficit del transportador de glucosa tipo 1, que abarca sus características clínicas, bioquímicas, moleculares y terapéuticas.

Glucose transporter type 1 deficiency with a typical onset is a genetic disorder associated with the SLC2A1 gene. Usually appears during the first years of life with severe developmental delay, drugresistant epilepsy, and movement disorders. Diagnosis is suspected based on clinical manifestations and a low glucose level in cerebrospinal fluid, and should be confirmed by the molecular genetic study of the SLC2A1 gene. As it is a rare disease with variable clinical expression, early diagnosis is often challenging for the healthcare team. Nevertheless, this is important because early implementation of ketogenic therapy will lead to control of the clinical manifestations and a better long-term prognosis. Here we review the glucose transporter type 1 deficiency syndrome focusing on its clinical, biochemical, molecular, and therapeutic characteristics.

Implicações odontológicas do consumo da dieta cetogênica para tratamento da epilepsia / Dental implications of the consumption of a ketogenic diet for the treatment of epilepsy

A epilepsia configura-se como sendo uma patologia crónica oriunda de manifestação elétrica cerebral de caráter anormal, que ocasiona repercussões neurológicas. O tratamento da epilepsia emprega a administração medicamentosa e também pode utilizar o recurso da dieta cetogénica. Sabe-se que o recurso da utilização da dieta cetogênica para enfrentamento da epilepsia promove repercussões a nível da saúde bucal dos enfermos em tratamento. O objetivo deste artigo foi evidenciar como o emprego da dieta cetogênica na terapia utilizada para enfrentamento da epilepsia pode influenciar na saúde bucal dos indivíduos em tratamento. O tratamento medicamentoso da epilepsia emprega anticonvulsivantes e muitas vezes requer o uso de mais de um medicamento para tratamento, o que pode acarretar efeitos adversos a nível sistêmico e oral. Merecem atenção e cuidados odontológicos comumente o tratamento com fármacos por intermédio da administração da fenitoína, geralmente utilizada para tratamento da epilepsia, que pode trazer repercussões odontológicas e alterações periodontais, como a hiperplasia gengival. O conhecimento e a conscientização por parte dos cirurgiões dentistas acerca dos cuidados odontológicos que devem ser adotados para esses pacientes especiais portadores de epilepsia são de suma importância para realização de uma abordagem odontológica. Concluiu-se que as repercussões bucais oriundas da epilepsia devem ser identificadas e tratadas imediatamente, ao passo que o cirurgião dentista contata os pacientes enfermos, uma vez que caso contrário pode-se conviver com agravantes e piora no quadro odontológico apresentado, portanto deve-se primar por impedir a evolução desfavorável do estado de saúde bucal dos pacientes.

Epilepsy is a chronic pathology arising from an abnormal electrical brain manifestation, which causes neurological repercussions. The treatment of epilepsy employs drug administration and can also use the ketogenic diet. It is known that the use of the ketogenic diet to cope with epilepsy promotes repercussions in terms of the oral health of patients undergoing treatment. The objective of this article was to show how the use of the ketogenic diet in the therapy used to cope with epilepsy can influence the oral health of individuals undergoing treatment. Drug treatment of epilepsy uses anticonvulsants and often requires the use of more than one drug for treatment, which can lead to adverse systemic and oral effects. Treatment with drugs through the administration of phenytoin, generally used for the treatment of epilepsy, which can bring dental repercussions and periodontal changes, such as gingival hyperplasia, deserves attention and dental care. Knowledge and awareness on the part of dental surgeons about the dental care that should be adopted for these special patients with epilepsy are of paramount importance for carrying out a dental approach. It was concluded that the oral repercussions arising from epilepsy should be identified and treated immediately, while the dental surgeon contacts sick patients, since otherwise one can live with aggravating factors and worsening of the dental condition presented, therefore, one should excel in preventing the unfavorable evolution of the patients' oral health status.

Effectiveness of nutritional interventions on behavioral symptomatology of autism spectrum disorder: a systematic review.

Introduction: Introduction: the main treatment for people with autism spectrum disorder (ASD) corresponds to cognitive behavioral therapy in conjunction with pharmacotherapy. Together they seek to attenuate the behavioral symptoms of these patients, as well as to increase their social functionality. However, other strategies have become popular to achieve the same goal of classical treatment. Particularly, nutritional interventions are positioned above others, and it is necessary to investigate their effectiveness, considering that children with ASD present a marked food selectivity, as well as gastrointestinal alterations. Objective: to evaluate the effectiveness of nutritional interventions in the behavioral symptomatology of infants with ASD. Methods: a systematic search was carried out in the Scopus and PubMed databases, in Spanish and English. The filters of clinical studies and original articles were used, choosing only nutritional interventions in children under 19 years of age and who had had at least 4 weeks of intervention. Results: evidence was found on gluten- and casein-free diets, ketogenic diet, omega-3 supplementation, prebiotics/probiotics, and vitamins/minerals presenting positive results in most of the articles analyzed; however, the heterogeneity presented requires a greater body of evidence to promote its use. Conclusion: the five types of nutritional interventions evaluated show varied evidence that does not allow defining the degree of effectiveness between one or the other in terms of behavioral improvements in the population with ASD.

Introducción: Introducción: el principal tratamiento para las personas con trastorno del espectro autista (TEA) corresponde a la terapia cognitivo conductual en conjunto con farmacoterapia. En conjunto buscan atenuar la sintomatología conductual de estos pacientes, así como aumentar su funcionalidad social. Sin embargo, otras estrategias se han tornado populares para conseguir el mismo objetivo del tratamiento clásico. Particularmente, las intervenciones nutricionales se posicionan por sobre otras y es necesario investigar su efectividad, considerando que los infantes con TEA presentan una marcada selectividad alimentaria, así como alteraciones gastrointestinales. Objetivo: evaluar la efectividad de las intervenciones nutricionales en la sintomatología conductual de infantes con TEA. Método: se realizó una búsqueda sistemática en las bases de datos de Scopus y PubMed, en español e inglés. Se utilizaron los filtros de estudios clínicos y artículos originales, eligiendo solo intervenciones nutricionales en menores de 19 años y que tuvieran al menos 4 semanas de intervención. Resultados: se encontró evidencia sobre las dietas libres de gluten y caseína, la dieta cetogénica, la suplementación de omega-3, los prebióticos/probióticos y las vitaminas/minerales, presentando resultados positivos en la mayoría de los artículos analizados; sin embargo, la heterogeneidad presentada exige un mayor cuerpo de evidencia para promover su utilización. Conclusión: los cinco tipos de intervenciones nutricionales evaluadas muestran evidencia variada que no permite definir el grado de efectividad entre una u otra en términos de mejoras conductuales en la población con TEA.

[Oral desensitization with Ketocal® in an infant with ketogenic diet and cow's milk protein allergy]. / Desensibilización con Ketocal® en lactante con dieta cetogénica y alergia a proteínas de leche de vaca.

Introduction: Introduction: ketogenic diet is a treatment with proven efficacy in drug-refractory childhood epilepsy. Cow's milk protein allergy may be a limitation for treating infants with ketogenic diet, as they need a product that contains cow's milk protein (Ketocal®). Case report: we report the case of an infant with a drug-refractory epileptic encephalopathy and IgE-mediated cow's milk protein allergy, who started a classic ketogenic diet. Oral desensitization was achieved with Ketocal 3:1®, allowing its use in the diet and achieving a clinical improvement with seizure control. Discussion: a patient with epilepsy and cow's milk protein allergy can benefit from the ketogenic diet, since it is possible to perform an oral immunotherapy with Ketocal®, also achieving a probable resolution of his/her allergy.

Introducción: Introducción: la dieta cetogénica es un tratamiento con eficacia demostrada en la epilepsia infantil refractaria a fármacos. La alergia a la proteína de leche de vaca puede ser una limitación para tratar a lactantes con dieta cetogénica, ya que necesitan un producto que contiene proteínas de leche de vaca (Ketocal®). Caso clínico: presentamos el caso de un lactante con una encefalopatía epiléptica refractaria a fármacos y alergia a las proteínas de leche de vaca IgE mediada al que se quiere instaurar una dieta cetogénica clásica. Se consigue la desensibilización con Ketocal 3:1®, pudiendo utilizarlo en la dieta y logrando una mejoría clínica con el control de las crisis. Discusión: un paciente con epilepsia y alergia a las proteínas de leche de vaca puede beneficiarse de la dieta cetogénica, ya que es posible realizar una inmunoterapia oral con Ketocal® y conseguir, además, una probable resolución de su alergia.

Hemidistonía y hemicorea en un paciente pediátrico con déficit del transportador de glucosa de tipo 1 / Hemidystonia and hemichorea in a pediatric patient with glucose transporter type 1 deficiency

El síndrome de deficiencia del transportador de glucosa cerebral de tipo 1 es una enfermedad neurometabólica rara en pediatría. Existe un fenotípico clásico (85 %) y otro no clásico (15 %). Ambos fenotipos se asocian con hipoglucorraquia. Se identifican múltiples mutaciones en el gen SLC2A1. El tratamiento es la terapia cetogénica. Se presenta un varón que comenzó a los cuatro años con hemicorea y hemidistonía medicado con anticonvulsivantes sin respuesta clínica, por lo que consultó nuevamente a los seis años. Con sospecha diagnóstica de síndrome de déficit de glut-1 atípico se realizó punción lumbar; el diagnóstico se confirmó por la presencia de hipoglucorraquia. Inmediatamente después de iniciar la dieta cetogénica, el paciente no presentó más movimientos anormales durante los siguientes 8 años hasta la actualidad, ya cumplidos los 14 años.

Glucose transporter type 1 deficiency syndrome is a rare pediatric neurometabolic disorder. There are two phenotypes: the classical phenotype (85%) and the non-classic (15%). Both phenotypes are associated with hypoglycorrhachia. Multiple mutations are described in the SCL2A1 gene. The treatment is the ketogenic diet. We report a case of a four-year-old male patient who started with hemichorea and hemidystonia and was medicated with drugs for seizures without clinical response, that's why his parents made another pediatric consultation at his six-year-old. With the suggestive clinical findings of glucose transporter type 1 deficiency syndrome the lumbar puncture was made confirming the diagnosis. Immediately after starting the ketogenic diet the patient stopped making abnormal movements up to the moment when he is fourteen years old, eight years after.

Validation of ketogenic diet adherence questionnaire: Keto-check / Validação de questionário de adesão à dieta cetogênica: Keto-check

Abstract Background The ketogenic diet is a therapeutic option to control drug-resistant epileptic seizures and has difficult compliance standards due to adverse effects, psychosocial factors, or dietary restriction. Objective To create and validate a ketogenic diet compliance questionnaire. Methods Methodological study, convergent care. The instrument was analyzed (construct validation) by five judges during two cycles of the Delphi technique. The judges assessed the relevance and clarity by using the Likert scale with three levels and made suggestions. The validation of the instrument was calculated using the agreement rate and content validity index (CVI). The application by two nutritionists made it possible to test reproducibility using the Kappa coefficient. When comparing the classification with a template developed by the health professionals who cared for the patients in question, the Pearson correlation and Fisher exact tests were used. For the reliability test, self-applied data were collected and evaluated using Cronbach Alpha coefficient. Results The questionnaire initially created with 11 multiple choice questions on a Likert scale was reduced to 10 questions with an agreement rate and the CVI classified as excellent after two rounds with judges. In the application of the tool, a kappa of 0.6087 was obtained, a Pearson that showed no significant difference between evaluators and a Cronbach α of 0.6483, with substantial consistency. Conclusions This work resulted in the creation of an unprecedented and reliable questionnaire on adherence to the national ketogenic diet. Further studies should be performed to extrapolate the use of this questionnaire in different populations.

Resumo Antecedentes A dieta cetogênica é um tratamento para epilepsia refratária e tem difícil adesão devido aos efeitos adversos, fatores psicossociais ou à própria restrição alimentar. Objetivo Criar e validar um questionário de adesão à dieta cetogênica. Métodos Estudo metodológico, convergente assistencial. O instrumento foi analisado (validação de constructo) por cinco juízes durante dois ciclos da técnica Delphi. Os juízes avaliaram a pertinência e a clareza com respostas na escala Likert com três níveis, além de sugestões. A validação do instrumento foi calculada pela taxa de concordância e índice de validade de conteúdo (IVC). Posteriormente, houve aplicação por duas nutricionistas (reprodutibilidade), sendo analisada a concordância pelo coeficiente de Kappa. Ao comparar a classificação com um gabarito, elaborado pelos profissionais da saúde responsáveis pelos pacientes em questão, foram utilizados os coeficientes de correlação de Pearson e teste exato de Fisher. Para teste de fidedignidade, a coleta de dados auto-aplicados foi avaliada pelo coeficiente de Alpha de Cronbach. Resultados O questionário inicialmente criado com 11 questões de múltipla escolha em escala Likert foi reduzido para 10 questões com taxa de concordância e IVC excelentes após 2 rodadas com juízes. Na aplicação da ferramenta obteve-se coeficiente de Kappa de 0,6087, o coeficiente de Pearson não demostrou diferença significativa entre avaliadores, e o coeficiente α de Cronbach de 0,6483, com consistência substancial. Conclusões Este trabalho resultou na criação do inédito questionário nacional de adesão à dieta cetogênica, válido e fidedigno. Outros estudos devem ser realizados para extrapolar a utilização deste questionário em diferentes populações.

Ketogenic dietary therapies for epilepsy: Experience in 160 patients over 18 years.

AIM: Ketogenic dietary therapies (KDT) produce anticonvulsant and neuroprotective effects, reduce seizures and improve the cognitive state in patients with epilepsy. Our purpose was to evaluate the effects of KDT in children with refractory epilepsy (effectiveness, side effects, impact on nutritional status and growth). METHODS: A retrospective and prospective observational descriptive study was conducted in a Spanish tertiary hospital (January 2000 to December 2018). One hundred sixty pediatric patients with epilepsy were treated with KDT (82 males; mean age 5 years 9 months). Seizures, anti-epileptic drugs, anthropometric measures, side effects, and laboratory assessment were monitored baseline and at 3, 6, 12 and 24 months after the onset of KDT. RESULTS: In these time intervals, the seizure-free patients were: 13.7, 12.5, 14.4 and 10.6%, respectively, and a reduction of seizures ≥ 50% was achieved in 41.9, 37.5, 28.7 and 16.2%. Side effects were frequent, especially digestive disorders, hypercalciuria, hypoglycemia, hepatic dysfunction and dyslipidemia. Prealbumin, retinol binding protein, vitamin A and magnesium decreased significantly. Height was affected, especially in children below 2 years. CONCLUSIONS: KDT are effective for refractory epilepsy in children. However, adverse effects are frequent, and it may affect nutritional status and growth.

[Controlling carbohydrate content in antiepileptic medications for children on a ketogenic diet]. / Control de carbohidratos en los medicamentos antiepilépticos para niños con dieta cetogénica.

Introduction: Introduction: patients who follow a ketogenic diet for the control of epileptic seizures must carry out a strict control of carbohydrates from the foods they eat and the medicines they are prescribed. In the initiation of a ketogenic diet and when a doctor prescribes a new medication, it is necessary to select the most appropriate pharmaceutical form so that the supply of excipients in the form of carbohydrates from the drugs is minimized. Objectives: the goal of the present paper was to compile a list of carbohydrate and caloric contents in antiepileptic drugs commonly used in pediatric neurology. Methods: in each medication included in the list, the content of excipients considered carbohydrates and derivatives that could influence the patient's ketosis was reviewed. The caloric content from carbohydrates and polyols in each medication was calculated. Results: the table provides the total carbohydrate and caloric content for antiepileptic medications in pediatric patients consuming the ketogenic diet. Conclusions: this table is intended to be a useful tool to help clinicians select a pharmaceutical form that is less likely to affect the ketogenic diet. Additionally, knowing the carbohydrate content of a new medication will allow adjustment of the diet to maintain ketosis.

Introducción: Introducción: los pacientes que siguen una dieta cetogénica para el control de las crisis epilépticas deben llevar a cabo un estricto control de los hidratos de carbono procedentes tanto de los alimentos que consumen como de los medicamentos que tienen pautados. Tanto en la instauración de la dieta cetogénica como cuando el médico prescribe un medicamento nuevo es necesario el ajuste de la medicación a las formas farmacéuticas más adecuadas, de forma que se minimice el aporte de excipientes en forma de hidratos de carbono de los medicamentos. Objetivos: el objetivo que planteamos en el presente trabajo fue elaborar un listado de medicamentos antiepilépticos de utilización habitual en neurología pediátrica que incluyera información sobre su contenido calórico en forma de hidratos de carbono para la administración a pacientes con dieta cetogénica. Métodos: en cada medicamento incluido en el listado se revisó el contenido en excipientes considerados hidratos de carbono y derivados que pudieran influir en la cetosis del paciente. Se calculó el contenido calórico procedente de los hidratos de carbono y polioles de cada medicamento. Resultados: elaboración de una tabla para consulta del contenido calórico de distintos medicamentos antiepilépticos utilizados en neurología pediátrica para pacientes con dieta cetogénica. Conclusiones: la tabla publicada pretende ser una herramienta útil que permita la consulta del contenido calórico de distintos medicamentos antiepilépticos y la selección del medicamento idóneo que menos afecte a la dieta cetogénica. Con el contenido calórico en carbohidratos de las medicaciones pautadas se podrán realizar los ajustes necesarios en la dieta para mantener la cetosis necesaria.

Effects of very low-carbohydrate ketogenic diet on lipid metabolism in patients with type II diabetes mellitus: a meta-analysis.

Introduction: Objective: to evaluate the lipid metabolism of patients with type 2 diabetes mellitus (T2DM) after very low-carbohydrate ketogenic (VLCK) diet treatment, so as to provide an evidence-based basis for better dietary management and comprehensive treatment of diabetic patients. Methods: PubMed, Cochrane Library, Embase, and Web of Science databases were searched for randomized controlled trial about VLCK diet on lipid metabolism of T2DM up to September 2021. The data were analyzed using the Stata 15.0; standardized mean difference (SMD) was used as effect size. Results: ten articles were included in this meta-analysis. There were no significant differences between the two groups in total cholesterol (SMD = -0.07, 95 % CI: -0.06-0.20, p > 0.05), HDL (SMD = 0.13, 95 % CI: -0.05-0.31, p > 0.05) and LDL (SMD = 0.07, 95 % CI: -0.06-0.20, p > 0.05) levels after treatment. No difference was found in total cholesterol, HDL, and LDL levels between the two groups after 3, 6, and 12 months of treatment (p > 0.05). Triglyceride levels decreased after VLCK diet compared with control (SMD = -0.49, 95 % CI: -0.82 to -0.17, p = 0.003). A marked reduction of triglyceride levels was identified after 3 months of VLCK diet treatment (SMD = -0.69, 95 % CI: -1.00 to -0.38), without significant difference after 6 and 12 months. Conclusion: T2DM patients who receive a VLCK diet to lower blood glucose are not associated with increased levels of total cholesterol and LDL, and decreased levels of HDL. Additionally, this diet can achieve a short-term reduction of triglyceride levels.

Introducción: Objetivo: evaluar el metabolismo lipídico de los pacientes con diabetes mellitus de tipo 2 (DMT2) tras el tratamiento con una dieta cetogénica muy baja en carbohidratos (VLCK), con el fin de proporcionar una base basada en la evidencia para un mejor manejo dietético y un tratamiento integral de los pacientes diabéticos. Métodos: se buscaron en las bases de datos PubMed, Cochrane Library, Embase y Web of Science ensayos controlados aleatorios sobre los efectos de la dieta VLCK en el metabolismo de los lípidos de la DMT2 hasta septiembre de 2021. Los datos se analizaron con el Stata 15.0. Se utilizó la diferencia de medias estandarizada (DME) como tamaño del efecto. Resultados: se incluyeron diez artículos en este metaanálisis. No hubo diferencias significativas entre los dos grupos en los niveles de colesterol total. No se encontraron diferencias en los niveles de colesterol total, HDL y LDL entre los dos grupos después de 3, 6 y 12 meses de tratamiento (p > 0,05). Los niveles de triglicéridos disminuyeron después de la dieta VLCK en comparación con el control. Se identificó una marcada reducción de los niveles de triglicéridos después de 3 meses de tratamiento con la dieta VLCK, sin diferencias significativas después de 6 y 12 meses. Conclusión: los pacientes con DMT2 que reciben una dieta VLCK para reducir la glucemia no se asocian a un aumento de los niveles de colesterol total y LDL, y a una disminución de los niveles de HDL.

Dietary patterns in non-alcoholic fatty liver disease (NAFLD): Stay on the straight and narrow path!

Non-alcoholic fatty liver disease (NAFLD) is the most frequent hepatic disease globally. NAFLD patients are at an increased risk of both liver and cardiovascular morbidity and mortality, as well as all-cause death. NAFLD prevalence is rapidly increasing worldwide and, thus, there is an urgent need for health policies to tackle its development and complications. Currently, since there is no drug therapy officially indicated for this disease, lifestyle interventions remain the first-line therapeutic option. In the present narrative review, we discuss the effects of certain dietary patterns on NAFLD incidence and progression. The Mediterranean diet is regarded as the diet of choice for the prevention/treatment of NAFLD and its complications, based on the available evidence. Other plant-based dietary patterns (poor in saturated fat, refined carbohydrates, red and processed meats) are also beneficial [i.e., Dietary Approaches to Stop Hypertension (DASH) and vegetarian/vegan diets], whereas more data are needed to establish the role of ketogenic, intermittent fasting and paleo diets in NAFLD. Nevertheless, there is no "one-size-fits-all" dietary intervention for NAFLD management. Clinicians should discuss with their patients and define the diet that each individual prefers and is able to implement in his/her daily life.

Estado epiléptico super-refratário e dieta cetogênica na unidade de terapia intensiva: relato de uma série de casos / Super-refractory status epilepticus and ketogenic diet in intensive care: a series report

RESUMO Define-se estado epiléptico super-refratário como ocorrência de crises epilépticas persistentes ou que ressurgem em condições de infusão endovenosa de anestésicos por mais de 24 horas. Nos últimos anos, chamou-se a atenção para os potenciais benefícios de uma dieta cetogênica para o controle de tais pacientes. Contudo, o papel específico dessa estratégia na população adulta, assim como o mecanismo de ação, a melhor ocasião para iniciar e o manejo das complicações, permanece como assunto amplamente debatível. Relatamos uma série de casos com três pacientes que foram internados em unidade de terapia intensiva em razão de estado epiléptico super-refratário e tratados com utilização de dieta cetogênica; também propomos uma abordagem clínica para início, transição e manejo das intercorrências clínicas desta intervenção.

Abstract Super-refractory status epilepticus is defined as seizures that persist or reemerge in the setting of an intravenous anesthetic infusion for more than 24 hours. In recent years, attention has been driven to the potential benefits of a ketogenic diet in the management of these patients. However, the specific role of this strategy in the adult population, as well as its underlying mechanism of action and optimal time for the initiation and management of complications, remain widely debatable. We report a case series of three patients admitted to an intensive care unit due to super-refractory status epilepticus who were managed with a ketogenic diet and propose a clinical approach to its initiation, transition, and management of clinical intercurrences.

[Treatment of refractory epilepsy. A comparison between classic ketogenic diet and modified Atkins diet in terms of efficacy, adherence, and undesirable effects]. / Tratamiento de la epilepsia refractaria. Comparación entre la dieta cetogénica clásica y la de Atkins modificada en cuanto a eficacia, adherencia y efectos indeseables.

INTRODUCTION: Background: the ketogenic diet (CD) is an established, effective non-pharmacological treatment for refractory epilepsy in childhood. Aim: the objective of this study was to compare the efficacy, the presence of undesirable effects, and adherence between the classic ketogenic diet (DCC) and the modified Atkins diet (DAM). Materials and methods: a retrospective and comparative investigation was carried out to evaluate the medical records of all the patients who started treatment with a ketogenic diet by the same team between 2008 and 2018. In all, 57 patients were started on a DAM diet and 19 patients were given a DCC diet. Results: it was observed that both the Atkins and the classic diets were equally effective (approximately, 80 %; p = 0.252). Regarding adherence, there was a significantly higher percentage of adherence to the Atkins diet than to the classic diet (p = 0.018). Fewer adverse effects were observed with DAM than with DCC (p = 0.012). In all, 21 % of patients under DAM had unfavorable effects (12/57), while 52.63 % of patients on DCC had complications (10/19). Conclusion: a comparable effectiveness in terms of crisis control was found between DAM and DCC. However, DAM exhibits a much better adherence than DCC, and its undesirable effects are milder, less common. That is why, according to other works, it is likely that DAM should be first-choice for patients with refractory epilepsy in a large percentage of cases.

INTRODUCCIÓN: Introducción: la dieta cetogénica (CD) es un tratamiento no farmacológico efectivo, ya establecido para la epilepsia refractaria en la infancia. Objetivo: el objetivo de este estudio fue comparar la eficacia, la presencia de efectos indeseables y la adherencia entre la dieta cetogénica clásica (DCC) y la dieta de Atkins modificada (DAM). Materiales y métodos: se realizó una investigación retrospectiva y comparativa, evaluando las historias clínicas de todos los pacientes que iniciaron tratamiento con dieta cetogénica a cargo de un mismo equipo de trabajo entre 2008 y 2018. Se incluyeron 57 pacientes que iniciaron una dieta DAM y 19 pacientes con dieta DCC. Resultados: se observó que tanto la dieta Atkins como la clásica fueron igualmente eficaces (80 %, aproximadamente; p = 0,252). En relación a la adherencia, se registró un porcentaje significativamente mayor de adherencia a la dieta Atkins que a la clásica (p = 0,018). Se observaron menos efectos adversos con la DAM que con la dieta CC (p = 0,012). El 21 % de los pacientes con DAM presentaron efectos desfavorables (12/57), mientras que el 52.63 % de los pacientes en DCC tuvieron complicaciones (10/19). Conclusión: se describe una efectividad equiparable en cuanto al control de crisis entre la DAM y la DCC. Sin embargo, la DAM presenta una adherencia mucho mejor que la observada con la DCC y sus efectos indeseables son más leves y más infrecuentes. Es por eso que, de acuerdo con otros trabajos, la DAM probablemente sea la primera elección para los pacientes con epilepsia refractaria en un gran porcentaje de los casos.