ABSTRACT
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.
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Epilepsy/diet therapy , Dietary Fats/therapeutic use , Anticonvulsants/therapeutic use , Kidney Calculi/etiology , Ketosis/metabolism , Cholesterol/blood , Seizures/diet therapy , Ketone Bodies/biosynthesis , Epilepsy/metabolism , Epilepsy/blood , Follow-Up Studies , Dietary Fats/adverse effects , Time Factors , Treatment OutcomeABSTRACT
The effect of stevioside, an inhibitor of long-chain fatty acid transport, on ketogenesis and on [14C]CO2 production from [1-14C] palmitate (100-300µM) was investigated in the isolated and hemoglobin-free perfused rat liver. Stevioside (2.5mM), a sweet glycoside found in Stevia rebaudiana leaves, inhibited both parameters, but had a lower effection on [14C]CO2 production. At 300µM palmitate and 150 µM albumin, for example, ketogenesis was inhibited by 66.3%, whereas no significant inhibition of [14C]CO2 was demonstrable. These results were interpreted to reflect 1) different degress of saturation of the citric acid cycle and the ketogenic patheay and 2) changes in the redox state of the mitochondrial NAD+-NADH couple which may also occur upon stevioside infusion
Subject(s)
Animals , Rats , Ketone Bodies/biosynthesis , Carbon Dioxide/metabolism , Glucosides/pharmacology , Palmitates/antagonists & inhibitors , Cell Membrane Permeability , Oxygen Consumption , Liver/metabolismABSTRACT
Production of ketone bodies, activation of oxygen uptake and production of [14C] carbon dioxide due to fatty acids of various chain lengths were measured in the isolated perfused rat liver. As ketogenic substrates, the fatty acids investigated can be ordered in the following sequence: strate < palmitate < laureate < decanoate < miristate < octanoate. For activation of oxygen consumptor, the same sequence is observed. A good linear correlation between the increase in oxygen uptake (deltaO2) and production of ketone bodies was found, with a mean deltaO2 of 0.66 mol per mol ketone bodies. This is a relatively low value when confronted with the known stoichiometry of ß-oxidation, and may be an indication of complexities in the metabolism of exogenously added fatty acids in the perfused liver