title: [Supplementation of L-carnitine to the severe motor and intellectual disabilities fed with enteral carnitine-deficient formulas for years].
abstract: OBJECTIVE
  We clarified the asymptomatic deficiency of serum free-carnitine in the severe motor and intellectual disabilities (SMIDs) fed with enteral carnitine-deficient formulas for years, and investigated the adequate method to supply enteral L-carnitine and maintain its normal levels.
  
  
  METHODS
  In 45 SMIDs who has been fed with carnitine-deficient formulas and/or receiving valproate, the serum free-carnitine levels were examined. To the carnitine deficient cases we introduced L-carnitine and/or a carnitine-supplemented formula to normalize and maintain the serum free-carnitine levels.
  
  
  RESULTS
  Thirty-one out of 34 cases (91.2%) fed with carnitine-deficient formulas for years had serum free-carnitine deficiency. Supplement of 15 - 30 mg/kg/day L-carnitine was effective to normalize the serum free-carnitine levels within 3 months. After successful supplementation, smaller dosage of L-carnitine (100 or 300 mg/day) was enough to maintain the normal levels. Replacement of the carnitine-deficient formulas to carnitine-supplemented ones was also useful to normalize the serum free-carnitine levels.
  
  
  CONCLUSIONS
  Smaller dosage of L-carnitine or a carnitine-supplemented formula is sufficient to normalize and maintain the serum free-carnitine levels in SMIDs fed with carnitine-deficient formulas for years.
authors.forename: Syuichi
authors.initials: S
authors.lastname: Tsuneishi
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Taguchi
authors.forename: Ryuzaburo
authors.initials: R
authors.lastname: Yagi
mesh_terms: D000293:Adolescent; D000328:Adult; D002331:Carnitine / Q000172:deficiency / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D019587:Dietary Supplements; D004750:Enteral Nutrition; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D000068079:Motor Disorders / Q000188:drug therapy*; D055815:Young Adult
chemical_list: D002331:Carnitine