Dieta cetogénica en epilepsia refractaria: eficacia, evolución y complicaciones a largo plazo / Ketogenic diet in refractory epilepsy: efficacy, evolution and long-term complications

Background: Ketogenic diet (KD) represents an alternative in treatment of refractory epilepsy (RE). Objective: To evaluate the efficacy of the diet and the frequency of complications in patients belonging to the KD Program from Luis Calvo Mackenna Children's Hospital (HLCM). Methods: Evaluation of all children enrolled in the program between 1999 and 2004, with analysis every 6 months of the diet efficacy, digestive tolerance, nutritional status, cholesterol levels and nephrolithiasis. Results: 21 children were admitted, 14 boys, age between 6 months - 17 years-old. 76 percent, 71 percent and 67 percent of patients followed KD at 6, 12 and 18 months, respectively, with KD efficacy of 67 percent. At 12 months, 24 percent of patients did not present seizures. At 18 months, 85 percent remained close to ideal body weight (15 percent obesity) and height/age Z score decreased (-0,7 +/- 0,4; p < 0,05). Total cholesterol significantly increased at 6 months (64 percent hypercholesterolemia; decreased to 15 percent at 18 months). 2 patients developed nephrolithiasis. Conclusions: The study shows high efficacy of the KD for treatment of refractory epilepsy, with low rate of complications. It should be considered as a therapeutic alternative for these patients.

La dieta cetogénica (DK) es una opción de tratamiento en epilepsia refractaria (ER). En Chile no hay estudios publicados al respecto. Objetivo: Evaluar la eficacia de la dieta en el control de las convulsiones y la frecuencia de complicaciones en los pacientes del programa de DK, para el tratamiento de ER, del Hospital Luis Calvo Mackena (HLCM). Pacientes y Método: Evaluamos todos los niños ingresados al programa entre 1999-2004. Para efectos de este trabajo se consideró el control al ingreso y cada 6 meses, evaluándose: eficacia de la dieta, tolerancia digestiva, evolución nutricional, niveles de colesterol plasmático y litiasis renal. Resultados: Ingresaron 21 niños de 6,2 años (6 meses a 17 años), 14 de sexo masculino. A los 6, 12 y 18 meses, 76 por ciento, 71 por ciento y 67 por ciento de los pacientes, respectivamente, se mantenía en dieta. La eficacia del tratamiento fue 67 por ciento. A los 12 meses, 24 por ciento de los pacientes estaba sin crisis. A los 18 meses 85 por ciento de los pacientes estaba eutrófico y 15 por ciento obeso. Se observó deterioro en la talla (delta zT/E -0,7 +/- 0,4; p < 0,05). El colesterol total aumentó significativamente a los 6 meses, encontrándose el 64 por ciento hipercolesterolémico; a los 18 meses este porcentaje se redujo a 15 por ciento. Dos pacientes presentaron litiasis renal (9 por ciento). Conclusiones: Este estudio muestra una muy buena eficacia de la dieta cetogénica para el tratamiento de la epilepsia refractaria, y una baja frecuencia de complicaciones, por lo que debería ser considerada como alternativa terapéutica en estos pacientes.

Dieta cetogênica para epilepsia intratável em crianças e adolescentes: relato de seis casos / Ketogenic diet for intractable epilepsy in children and adolescents: report of six cases

OBJETIVO: Descrever a introdução e o manejo da dieta cetogênica em um grupo de seis crianças e adolescentes com epilepsia refratária. MÉTODOS: Os autores reviram o prontuário médico de cada paciente menor de 15 anos submetido à dieta cetogênica entre abril de 1999 e julho de 2003 e compararam os resultados terapêuticos e efeitos adversos e benéficos com a literatura pertinente. RESULTADOS: A dieta cetogênica foi introduzida para seis pacientes, com idade mediana de sete anos (faixa: 1,8-12,2). A duração média da aplicação da dieta foi 9,7 meses (faixa: 7 dias-4 anos). Observou-se uma redução igual ou maior que 50 por cento da freqüência das crises epilépticas em metade dos casos. As complicações observadas foram leucopenia, constipação, desidratação, priapismo e recorrência das crises epilépticas. CONCLUSÕES: A dieta cetogênica foi eficaz e segura em três pacientes de uma série de seis casos com epilepsia intratável. A complicação mais comum foi leucopenia.

Ketogenic diet: an alternative treatment for refractory epilepsy in children.

RATIONALE: The aim of this study was to establish the first ketogenic diet treatment program for refractory epilepsy in Thailand and to assess its feasibility as well as its efficacy. METHOD: Children with refractory epilepsy were enrolled in the study. This was a prospective open trial study with 35 children (16 boys and 19 girls). Not all patients started on the diet at the same time. Each patient was cumulatively enrolled in this study over the period of 4 years. The mean age on diet was 5.37 +/- 3.57 years (2 months-13 years), mean age of onset of seizures was 19.2 +/- 27.47 months (1 days-8 years), and an average duration on ketogenic diet was 7.67 months (6 days to 29 months). The classic "4:1" formula ketogenic diet was used with some modification. The patient's parents were allowed to improvise and use any fatty diets available in the market such as coconut milk if needed. Parents were closely supervised and instructed on how to prepare the patient's own meals while in the hospital and continued to attend neurology and nutrition clinics. The seizure outcome and side effects were monitored as well as a daily test for urine ketone. RESULTS: At 1 month, 3 months, 6 months, and 12 months duration on the diet, 90 per cent seizure reductions were achieved in 62.5 per cent, 68.18 per cent, 75 per cent, and 66.67 per cent of patients remaining on the diet, respectively. The number of antiepileptic drugs (AEDs) used by each patient also decreased as a result of better seizure control. CONCLUSION: Ketogenic diet can be tried as a management option for refractory epilepsy. It is not difficult to implement even in a developing country like Thailand where resources are limited. It may also help reduce the cost of treatment especially in view of the high prices of the new AEDs.

Enfermedades metabólicas en el recién nacido / Metabolic diseases in the newborn

Se revisan las enfermedades metabólicas que pueden manifestarse en el período neonatal, destacando los elementos clínicos y de laboratorio que el pediatra general debe considerar para aproximarse al diagnóstico y ofrecer el manejo inicial apropiado de estos pacientes. La evaluación de los gases en sangre, el hiato aniónico, el ácido láctico en el suero, la amenomia y la glicemia debe hacerse cuidadosamente para un diagnóstico diferencial apropiado. El tratamiento incial incluye, además del manejo general correspondiente a cualquier recién nacido enfermo, el control de la hipoglicemia, la acidosis y los trastornos del metabolismo del agua y los electrolitos, la restricción de las proteínas, el aporte energético necesario, la suplementación de cofactores metabólicos considerados específicos y la solicitud oportuna de apoyo especializado