title: Carnitine deficiency: Risk factors and incidence in children with epilepsy.
abstract: BACKGROUND
  Carnitine deficiency is relatively common in epilepsy; risk factors reportedly include combination antiepileptic drug (AED) therapy with valproic acid (VPA), young age, intellectual disability, diet and enteral or parenteral feeding. Few studies have examined the correlation between each risk factor and carnitine deficiency in children with epilepsy. We examined the influence of these risk factors on carnitine deficiency, and identified a formula to estimate plasma free carnitine concentration in children with epilepsy.
  
  
  METHODS
  Sixty-five children with epilepsy and 26 age-matched controls were enrolled. Plasma carnitine concentrations were measured using an enzyme cycling assay, and correlations were sought with patients' other clinical characteristics.
  
  
  RESULTS
  Carnitine deficiency was found in approximately 17% of patients with epilepsy and was significantly associated with carnitine-free enteral formula only by tube feeding, number of AEDs taken (independent of VPA use), body weight (BW), body height and Gross Motor Function Classification System (GMFCS) score. Stepwise multiple linear regression analysis indicated that carnitine concentration (in μmol/L) could be accurately estimated from a formula that does not require blood testing: 42.44+0.14×(BW in kg)-18.16×(feeding)-3.19×(number of AEDs), where feeding was allocated a score of 1 for carnitine-free enteral formula only by tube feeding and 0 for taking food orally (R(2)=0.504, P<0.001).
  
  
  CONCLUSIONS
  Carnitine-free enteral formula only by tube feeding, multiple AED treatment and low BW are risk factors for carnitine deficiency in children with epilepsy. l-carnitine should be administered to children at risk of deficiency to avoid complications. Treatment decisions can be informed using an estimation formula that does not require blood tests.
authors.affiliation: Department of Pediatrics, Ehime University Graduate School of Medicine, Toon, Ehime, Japan. Electronic address: fukumits@m.ehime-u.ac.jp.
authors.forename: Mitsumasa
authors.initials: M
authors.lastname: Fukuda
authors.affiliation: Department of Pediatrics, Ehime University Graduate School of Medicine, Toon, Ehime, Japan.
authors.forename: Mika
authors.initials: M
authors.lastname: Kawabe
authors.affiliation: Department of Pediatrics, Ehime University Graduate School of Medicine, Toon, Ehime, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Takehara
authors.affiliation: Department of Pediatrics, Ehime University Graduate School of Medicine, Toon, Ehime, Japan.
authors.forename: Sachiko
authors.initials: S
authors.lastname: Iwano
authors.affiliation: Ehime Rehabilitation Center for Children, Toon, Ehime, Japan.
authors.forename: Kozue
authors.initials: K
authors.lastname: Kuwabara
authors.affiliation: Ehime Rehabilitation Center for Children, Toon, Ehime, Japan.
authors.forename: Chiya
authors.initials: C
authors.lastname: Kikuchi
authors.affiliation: Ehime Rehabilitation Center for Children, Toon, Ehime, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Wakamoto
authors.affiliation: Ehime Rehabilitation Center for Children, Toon, Ehime, Japan.
authors.forename: Takehiko
authors.initials: T
authors.lastname: Morimoto
authors.affiliation: Ehime Rehabilitation Center for Children, Toon, Ehime, Japan.
authors.forename: Yuka
authors.initials: Y
authors.lastname: Suzuki
authors.affiliation: Department of Pediatrics, Ehime University Graduate School of Medicine, Toon, Ehime, Japan.
authors.forename: Eiichi
authors.initials: E
authors.lastname: Ishii
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use; D009202:Cardiomyopathies / Q000097:blood* / Q000178:diet therapy; D002331:Carnitine / Q000008:administration & dosage / Q000097:blood* / Q000172:deficiency*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D055423:Diet, Ketogenic; D004827:Epilepsy / Q000097:blood* / Q000188:drug therapy / Q000453:epidemiology; D005260:Female; D006801:Humans; D022124:Hyperammonemia / Q000097:blood* / Q000178:diet therapy; D015994:Incidence; D007223:Infant; D007564:Japan / Q000453:epidemiology; D008297:Male; D009135:Muscular Diseases / Q000097:blood* / Q000178:diet therapy; D012307:Risk Factors; D014635:Valproic Acid / Q000627:therapeutic use
chemical_list: D000927:Anticonvulsants; D014635:Valproic Acid; D002331:Carnitine
keywords: Antiepileptic drugs; Carnitine deficiency; Childhood epilepsy; Estimated formula; Intellectual disability; Tube feeding