[Asymptomatic carnitine depletion on ketogenic diet in patients with pharmacoresistant epilepsies].
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.


METHODS
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).


CONCLUSIONS
Regular controls of carnitine levels should be performed during the treatment with ketogenic diet, both at the beginning and during longterm-therapy.
Liebhaber
G
G
Kinderklinik und Poliklinik der Technischen Universität München, Kinderklinik München Schwabing.
Pascher
B
B
Gempel
K
K
Baumeister
F A
FA
D000927:Anticonvulsants / Q000008:administration & dosage; D002331:Carnitine / Q000008:administration & dosage / Q000031:analogs & derivatives / Q000097:blood / Q000172:deficiency*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D004040:Dietary Carbohydrates / Q000008:administration & dosage; D004041:Dietary Fats / Q000008:administration & dosage*; D004351:Drug Resistance; D004827:Epilepsy / Q000097:blood / Q000178:diet therapy*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007657:Ketone Bodies / Q000097:blood*; D008297:Male; D016896:Treatment Outcome; D014635:Valproic Acid / Q000008:administration & dosage
D000927:Anticonvulsants; D004040:Dietary Carbohydrates; D004041:Dietary Fats; D007657:Ketone Bodies; C116917:acylcarnitine; D014635:Valproic Acid; D002331:Carnitine