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.

Terapias dietéticas cetogénicas en epilepsia: experiencia en 160 pacientes durante 18 años / Ketogenic dietary therapies for epilepsy: Experience in 160 patients over 18 years

Introducción: Las terapias dietéticas cetogénicas (TDC) tienen efecto neuroprotector y anticonvulsivante, reducen las crisis epilépticas y mejoran el estado cognitivo en pacientes epilépticos. Nuestro propósito fue evaluar los efectos de las TDC en niños con epilepsia refractaria (eficacia, efectos secundarios, impacto en el estado nutricional y crecimiento).Métodos: Se realizó un estudio observacional descriptivo retrospectivo y prospectivo en un hospital terciario español (enero de 2000-diciembre de 2018). Ciento sesenta pacientes pediátricos con epilepsia fueron tratados con TDC (82 varones; edad media 5 años 9 meses). Las convulsiones, los fármacos antiepilépticos, la antropometría, los efectos secundarios y los parámetros analíticos se controlaron al inicio del tratamiento y a los 3, 6, 12 y 24 meses.Resultados: En estos intervalos los pacientes libres de crisis fueron: 13,7%, 12,5%, 14,4% y 10,6%, respectivamente, lográndose una reducción de las convulsiones≥50% en el 41,9%, 37,5%, 28,7% y 16,2%. Los efectos secundarios fueron frecuentes, especialmente trastornos digestivos, hipercalciuria, hipoglucemia, disfunción hepática y dislipidemia. La prealbúmina, la proteína de unión al retinol, la vitamina A y el magnesio disminuyeron significativamente. La talla se vio afectada, especialmente en niños menores de 2 años.Conclusiones: Las TDC son efectivas para la epilepsia refractaria infantil. Sin embargo, los efectos adversos son frecuentes y pueden afectar al estado nutricional y al crecimiento. (AU)

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.(AU)

Transcriptomic profile of epileptic children treated with ketogenic therapies.

Background: Ketogenic dietary therapies (KDT) are used as a treatment in childhood epilepsy. However, their mechanism has not yet been established. The main objective of this study was to determine the changes in the transcriptomic profile induced by KDT in children with epilepsy in order to shed light on its possible mechanisms. Methods: Eight children with refractory epilepsy were enrolled in the study. Peripheral blood mononuclear cells were obtained before and after the children were treated with KDT for a minimum of 6 months. RNA was extracted and mRNA and miRNA profiling were performed and analyzed. Results: Our intervention with KDT significantly reduced the seizure number in seven of the eight paediatric patients treated and caused important changes in their gene expression profile. Our study reveals modifications in the transcription of 4630 genes and 230 miRNAs. We found that the genes involved in the protection against epileptic crises were among those mainly changed. These genes collectively encode for ion channels, neurotransmitter receptors, and synapse structural proteins. Conclusions: Together our results explain the possible mechanisms of KDT and reinforce its clinical importance in the treatment of epilepsy.

Efficacy and safety of ketogenic dietary theraphies in infancy. A single-center experience in 42 infants less than two years of age.

PURPOSE: Ketogenic dietary therapies (KDT) are high-fat and low-carbohydrate diets that may achieve seizure control and improve cognitive state. We describe our KDT experience in infants (children less than two years of age). RESEARCH METHODS & PROCEDURES: We conducted a retrospective, descriptive and observational study of 42 infants treated with KDT between 2000-2018. RESULTS: The types of KDT started were: classic ketogenic diet ratio 3:1 (40), ratio 4:1 (1) and modified ketogenic diet with medium-chain triglycerides (1). Four patients switched to a modified Atkins diet. During follow-up, 79%, 57%, 38% and 17% of infants remained on KDT at 3, 6, 12 and 24 months, respectively. Seizure reduction ≥50% compared to baseline was achieved in 50%, 45%, 38% and 17% at 3, 6, 12 and 24 months, respectively. Seizure control was excellent (reduction >90%) in 33%, 31%, 26% and 12%, and seizure-free infants were 9, 9, 10 and 4, at different follow-up intervals, respectively. Sixty-three percent of infants with West syndrome were responders to KDT. Mean length of KDT was 390 days (16 days-4.9 years). Ineffectiveness was the reason for withdrawal in 50% of patients. Early adverse effects (during first month) occurred in 40% of infants. The most frequent early side effects were asymptomatic hypoglycemia and gastrointestinal disturbances. Late-onset side effects occurred in 55-14% of infants during therapy, and most frequent were hypercalciuria and dyslipidaemia. CONCLUSION: KDT are useful and effective treatments in infancy. Side effects are frequent but mild and easy to manage.

[Ketogenic dietary therapies for epilepsy: Experience in 160 patients over 18 years]. / Terapias dietéticas cetogénicas en epilepsia: experiencia en 160 pacientes durante 18 años.

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.

Classic Ketogenic Diet and Modified Atkins Diet in SLC2A1 Positive and Negative Patients with Suspected GLUT1 Deficiency Syndrome: A Single Center Analysis of 18 Cases.

BACKGROUND: Glucose transporter type 1 deficiency syndrome (GLUT1DS) is caused by mutations in the SLC2A1 gene and produces seizures, neurodevelopmental impairment, and movement disorders. Ketogenic dietary therapies (KDT) are the gold standard treatment. Similar symptoms may appear in SLC2A1 negative patients. The purpose is to evaluate the effectiveness of KDT in children with GLUT1DS suspected SLC2A1 (+) and (-), side effects (SE), and the impact on patients nutritional status. METHODS: An observational descriptive study was conducted to describe 18 children (January 2009-August 2020). SLC2A1 analysis, seizures, movement disorder, anti-epileptic drugs (AEDS), anthropometry, SE, and laboratory assessment were monitored baseline and at 3, 6, 12, and 24 months after the onset of KDT. RESULTS: 6/18 were SLC2A1(+) and 13/18 had seizures. In these groups, the age for debut of symptoms was higher. The mean time from debut to KDT onset was higher in SLC2A1(+). The modified Atkins diet (MAD) was used in 12 (5 SLC2A1(+)). Movement disorder improved (4/5), and a reduction in seizures >50% compared to baseline was achieved in more than half of the epileptic children throughout the follow-up. No differences in effectiveness were found according to the type of KDT. Early SE occurred in 33%. Long-term SE occurred in 10, 5, 7, and 5 children throughout the follow-up. The most frequent SE were constipation, hypercalciuria, and hyperlipidaemia. No differences in growth were found according to the SLC2A1 mutation or type of KDT. CONCLUSIONS: CKD and MAD were effective for SLC2A1 positive and negative patients in our cohort. SE were frequent, but mild. Permanent monitoring should be made to identify SE and nutritional deficits.

Safety and Effectiveness of the Prolonged Treatment of Children with a Ketogenic Diet.

BACKGROUND: The ketogenic diet (KD) is an effective treatment against drug-resistant epilepsy in children. The KD is a diet rich in fats that produces anticonvulsant and neuroprotective effects that reduces seizures and improves the cognitive state. Nevertheless, it can produce side effects that sometimes can be serious. Further, the effect on growth is quite controversial when used for an extended period of time. The aim of this paper was to assess the effectiveness, side effects, and repercussions in the development of children who have been treated with a KD for more than 2 years. METHODS: Observational descriptive study of 26 pediatric patients on a KD, with data collection at baseline, at 3, 6, and 12 months, and then once a year. Number of seizures, type of seizures, anti-seizure drugs, anthropometry, side effects, and alterations in laboratory assessment were monitored. RESULTS: In every assessment, about 60%-75% of the patients experienced a reduction in number of seizures of over 90%, and at least 50% experienced side effects, of which digestive issues, alteration in the lipid metabolism, and hypercalciuria were the most common. The KD significantly affected height after 2 years of treatment. CONCLUSIONS: The KD is an effective treatment for drug-resistant epilepsy. Its side effects, although common, are very mild; therefore, this constitutes a very safe treatment for children of all ages. More studies are needed to identify and prevent potential causes of growth retardation in children on the KD.

[Controversies and errors related to nutrition and breastfeeding. Guidelines for improvement]. / Controversias y errores en relación con la nutrición y la lactancia materna. Pautas para la mejora.

INTRODUCTION: Although the benefits of breastfeeding are clear and well documented, for both newborns and nursing mothers, breastfeeding rates worldwide are not optimal in many cases. There are multiple myths and errors related to breastfeeding: that certain foods cannot be consumed during breastfeeding, that, however, certain foods must be consumed to increase milk production, that composition of breast milk is not adequate in some cases, that breastfeeding is associated with more risk of suffering caries… Therefore, the objective of this article is to clarify the reality about these aspects of breastfeeding.

INTRODUCCIÓN: Aunque los beneficios de la lactancia materna son claros y están bien documentados tanto para los recién nacidos como para las madres lactantes, las tasas de lactancia materna a nivel mundial en muchos casos no son óptimas. Existen múltiples mitos y errores en relación con la lactancia materna, como que no pueden consumirse determinados alimentos, que deben consumirse determinados alimentos para aumentar la producción láctea, que la composición de la leche materna en algunos casos no es adecuada, que la lactancia materna se asocia a más riesgo de caries… Por todo ello, el objetivo de este artículo es aclarar la realidad acerca de estos aspectos sobre la lactancia materna.

Controversias y errores en relación con la nutrición y la lactancia materna. Pautas para la mejora / Controversies and errors related to nutrition and breastfeeding. Guidelines for improvement

Aunque los beneficios de la lactancia materna son claros y están bien documentados tanto para los recién nacidos como para las madres lactantes, las tasas de lactancia materna a nivel mundial en muchos casos no son óptimas. Existen múltiples mitos y errores en relación con la lactancia materna, como que no pueden consumirse determinados alimentos, que deben consumirse determinados alimentos para aumentar la producción láctea, que la composición de la leche materna en algunos casos no es adecuada, que la lactancia materna se asocia a más riesgo de caries... Por todo ello, el objetivo de este artículo es aclarar la realidad acerca de estos aspectos sobre la lactancia materna

Although the benefits of breastfeeding are clear and well documented, for both newborns and nursing mothers, breastfeeding rates worldwide are not optimal in many cases. There are multiple myths and errors related to breastfeeding: that certain foods cannot be consumed during breastfeeding, that, however, certain foods must be consumed to increase milk production, that composition of breast milk is not adequate in some cases, that breastfeeding is associated with more risk of suffering caries... Therefore, the objective of this article is to clarify the reality about these aspects of breastfeeding

[Subclinical micronutrients deficiencies related to metabolic syndrome]. / Deficiencias subclínicas asociadas al padecimiento de síndrome metabólico.

Metabolic syndrome is the name given to a set of risk factors that increases the risk of cardiovascular disease and other health problems, such as diabetes and stroke. There are different cut-off points to establish the definition of metabolic syndrome according to various international organizations, although in all definitions are considered four main data related to: 1) obesity; 2) alteration of glucose metabolism; 3) alteration of lipid metabolism; and 4) hypertension. Strategies for the treatment of the metabolic syndrome include changes in lifestyle (diet and physical activity), along with pharmacological treatment in certain cases. There is little evidence of the effect of different micronutrients in this syndrome, although there are many investigations in this line.

El síndrome metabólico es el nombre que se da a un conjunto de factores de riesgo que aumenta el riesgo de enfermedad cardiovascular y otros problemas de salud, como diabetes y accidente cerebrovascular.Existen diferentes puntos de corte para establecer la definición de síndrome metabólico según diversos organismos internacionales, si bien en todas las definiciones se consideran cuatro pilares básicos: 1) obesidad; 2) alteración del metabolismo de la glucosa; 3) alteración del metabolismo de los lípidos; e 4) hipertensión arterial.Las estrategias para el tratamiento del síndrome metabólico incluyen cambios en el estilo de vida (dieta y actividad física) junto con tratamiento farmacológico en determinados casos. Se tiene poca evidencia sobre el efecto de diferentes micronutrientes en dicho síndrome, aunque existen múltiples investigaciones en esta línea.

Deficiencias subclínicas asociadas al padecimiento de síndrome metabólico / Subclinical micronutrients deficiencies related to metabolic syndrome

El síndrome metabólico es el nombre que se da a un conjunto de factores de riesgo que aumenta el riesgo de enfermedad cardiovascular y otros problemas de salud, como diabetes y accidente cerebrovascular. Existen diferentes puntos de corte para establecer la definición de síndrome metabólico según diversos organismos internacionales, si bien en todas las definiciones se consideran cuatro pilares básicos: 1) obesidad; 2) alteración del metabolismo de la glucosa; 3) alteración del metabolismo de los lípidos; e 4) hipertensión arterial. Las estrategias para el tratamiento del síndrome metabólico incluyen cambios en el estilo de vida (dieta y actividad física) junto con tratamiento farmacológico en determinados casos. Se tiene poca evidencia sobre el efecto de diferentes micronutrientes en dicho síndrome, aunque existen múltiples investigaciones en esta línea

Metabolic syndrome is the name given to a set of risk factors that increases the risk of cardiovascular disease and other health problems, such as diabetes and stroke. There are different cut-off points to establish the definition of metabolic syndrome according to various international organizations, although in all definitions are considered four main data related to: 1) obesity; 2) alteration of glucose metabolism; 3) alteration of lipid metabolism; and 4) hypertension. Strategies for the treatment of the metabolic syndrome include changes in lifestyle (diet and physical activity), along with pharmacological treatment in certain cases. There is little evidence of the effect of different micronutrients in this syndrome, although there are many investigations in this line

From infant to child. Feeding in different stages / De lactante a niño. Alimentación en diferentes etapas

Proper nutrition during childhood is necessary to: allow adequate growth and development, achieve optimal physical and psychic performance, maintain and improve health and recover more easily in disease processes.The first months of life (the infant stage) are a stage in which many rapid changes take place (anthropometric, body composition, maturation of organs and systems...), which suppose a high demand from nutritional point of view, in the quantitative aspect and especially in the qualitative aspect. After infant stage, preschool children feeding is an opportunity to acquire healthy habits and introduce new foods, textures, flavors, colors, etc., which will favor the autonomy and development of the individual, as well as being essential to maintain growth and development.

Una alimentación correcta durante la infancia es necesaria para: permitir un crecimiento y desarrollo adecuados, conseguir un rendimiento físico y psíquico óptimo, mantener y mejorar la salud y recuperarse más fácilmente en los procesos de enfermedad. Los primeros meses de vida (la etapa de lactante) son una etapa en la que tienen lugar muchos cambios rápidos (antropométricos, de composición corporal, de maduración de órganos y sistemas…), que suponen una demanda exigente desde el punto de vista nutricional, en el aspecto cuantitativo y sobre todo cualitativo.Pasada la etapa de lactante, la alimentación del preescolar supone una oportunidad de adquisición de hábitos saludables e introducción de nuevos alimentos, texturas, sabores, colores, etc., que van a favorecer la autonomía y el desarrollo del individuo, además de ser imprescindible para mantener el crecimiento y desarrollo.

De lactante a niño. Alimentación en diferentes etapas / From infant to child. Feeding in different stages

Una alimentación correcta durante la infancia es necesaria para: permitir un crecimiento y desarrollo adecuados, conseguir un rendimiento físico y psíquico óptimo, mantener y mejorar la salud y recuperarse más fácilmente en los procesos de enfermedad. Los primeros meses de vida (la etapa de lactante) son una etapa en la que tienen lugar muchos cambios rápidos (antropométricos, de composición corporal, de maduración de órganos y sistemas…), que suponen una demanda exigente desde el punto de vista nutricional, en el aspecto cuantitativo y sobre todo cualitativo. Pasada la etapa de lactante, la alimentación del preescolar supone una oportunidad de adquisición de hábitos saludables e introducción de nuevos alimentos, texturas, sabores, colores, etc., que van a favorecer la autonomía y el desarrollo del individuo, además de ser imprescindible para mantener el crecimiento y desarrollo (AU)

Proper nutrition during childhood is necessary to: allow adequate growth and development, achieve optimal physical and psychic performance, maintain and improve health and recover more easily in disease processes. The first months of life (the infant stage) are a stage in which many rapid changes take place (anthropometric, body composition, maturation of organs and systems...), which suppose a high demand from nutritional point of view, in the quantitative aspect and especially in the qualitative aspect. After infant stage, preschool children feeding is an opportunity to acquire healthy habits and introduce new foods, textures, flavors, colors, etc., which will favor the autonomy and development of the individual, as well as being essential to maintain growth and development (AU)