RÉSUMÉ
BACKGROUND: The ketogenic diet (KD) is a nutritional therapy for the treatment of epilepsies and certain metabolic disorders like the glucose-transporter-deficiency or the pyruvate-dehydrogenase-deficiency. The basis of the ketogenic diet is the change of energy metabolism to utilisation of fatty acids and their metabolites, ketonic bodies. Carnitines, which play an important role in transport and elimination of fatty acids, are essential for effective ketogenesis. Carnitine deficiency is described on ketogenic diet. The aim of this study is to evaluate when to expect a carnitine-deficiency during KD. PATIENTS: The carnitine levels of 19 patients aged 1.4 to 8.5 years (median 4.0 years), who were treated with ketogenic diet because of pharmacoresistent epilepsy, were evaluated retrospectively. RESULTS: Carnitine deficiency during KD was detected in 26 % of the patients and in 57 % of the patients without carnitine substitution. Decreased carnitine level occurred also with carnitine substitution independent from additional valproat therapy. The time of appearance of carnitine deficiency on KD was between 3 days and 248 weeks (median 32 weeks). CONCLUSION: Regular controls of carnitine levels should be performed during the treatment with ketogenic diet, both at the beginning and during longterm-therapy.
Sujet(s)
Carnitine/déficit , Matières grasses alimentaires/administration et posologie , Épilepsie/diétothérapie , Corps cétoniques/sang , Anticonvulsivants/administration et posologie , Carnitine/administration et posologie , Carnitine/analogues et dérivés , Carnitine/sang , Enfant d'âge préscolaire , Association thérapeutique , Hydrates de carbone alimentaires/administration et posologie , Résistance aux substances , Épilepsie/sang , Femelle , Humains , Nourrisson , Nouveau-né , Mâle , Résultat thérapeutique , Acide valproïque/administration et posologieRÉSUMÉ
Propofol is used for the treatment of refractory status epilepticus. When given as a long-term infusion propofol may cause a rare but frequently fatal complication, the propofol infusion syndrome. The hallmarks are metabolic acidosis, lipemia, rhabdomyolysis and myocardial failure. Propofol infusion syndrome is caused by impaired fatty acid oxidation. Beside anticonvulsants the ketogenic diet, a high-fat, low-carbohydrate, adequate-protein diet, is an effective treatment for difficult-to-control seizures. We report a 10-year-old boy with catastrophic epilepsy, who developed fatal propofol infusion syndrome when a ketogenic diet was initiated. Substances like propofol which impair fatty acid oxidation may pose an increased risk if combined with ketogenic diet.