title: Improvements of hypertriglyceridemia and hyperlacticemia in Japanese children with glycogen storage disease type Ia by medium-chain triglyceride milk.
abstract: BACKGROUND
  Besides profound hypoglycemia with hyperlacticemia, glycogen storage disease type Ia (GSD Ia) presents hypertriglyceridemia that is often resistant to dietary treatment with cornstarch. The present study aimed to evaluate the effects of medium-chain triglycerides (MCT)--which are absorbed via the portal vein without being incorporated into chylomicrons--on hypertriglyceridemia and to explore otherwise metabolic changes in children with GSD Ia.
  
  
  METHODS
  A 13-year-old boy with GSD Ia who received a dietary treatment with MCT milk after cornstarch administration and two infants also with GSD Ia, ages 6 and 7 months, who received MCT milk after carbohydrate-rich, lipid-poor milk were enrolled. In addition to serum glucose and lactate levels, serum levels of total cholesterol, triglycerides, and high-density lipoprotein (HDL) cholesterol were serially determined. Simultaneously, serum levels of total carnitine, free carnitine, acylcarnitine, and ketone bodies were determined to evaluate fatty acid beta-oxidation.
  
  
  RESULTS
  Mean glucose level (mmol/l) of patient 1 remained stable, the value being around 4.5, while those of patients 2 and 3 increased to this level from 4.00 and 3.72, respectively. Lactate levels were significantly decreased in all patients. Mean triglyceride levels (mM) of patient 1 decreased from 3.00 to 2.05. Also, triglyceride levels of patients 2 and 3 decreased from 2.74 and 3.15 to 2.13 and 2.70, respectively. HDL cholesterol, acylcarnitine, and ketone body levels increased in all patients after MCT administration, while total and free carnitine levels decreased.
  
  
  CONCLUSIONS
  We describe here the beneficial effects on lipid and carbohydrate metabolisms in three Japanese children with GSD Ia. In light of the unfavorable influence of lipid restriction on growth and development in infancy, dietary treatment with MCT milk may be a better treatment for infants with GSD Ia. Further investigation should be required to confirm the efficacy of MCT milk in GSD Ia.
authors.affiliation: Division of Metabolism, Chiba Children's Hospital, 579-1 Heta Cho Midori-Ku, Chiba, Japan. nagasa-hirono@k2.dion.ne.jp
authors.forename: Hironori
authors.initials: H
authors.lastname: Nagasaka
authors.forename: Ken-ichi
authors.initials: K
authors.lastname: Hirano
authors.forename: Akira
authors.initials: A
authors.lastname: Ohtake
authors.forename: Takashi
authors.initials: T
authors.lastname: Miida
authors.forename: Tomozumi
authors.initials: T
authors.lastname: Takatani
authors.forename: Kei
authors.initials: K
authors.lastname: Murayama
authors.forename: Tohru
authors.initials: T
authors.lastname: Yorifuji
authors.forename: Kunihiko
authors.initials: K
authors.lastname: Kobayashi
authors.forename: Masaki
authors.initials: M
authors.lastname: Kanazawa
authors.forename: Atsushi
authors.initials: A
authors.lastname: Ogawa
authors.forename: Masaki
authors.initials: M
authors.lastname: Takayanagi
mesh_terms: D000293:Adolescent; D000818:Animals; D015415:Biomarkers / Q000097:blood; D002675:Child, Preschool; D002784:Cholesterol / Q000097:blood; D004040:Dietary Carbohydrates / Q000378:metabolism; D019587:Dietary Supplements; D005953:Glycogen Storage Disease Type I / Q000097:blood / Q000150:complications / Q000178:diet therapy*; D006128:Growth; D006801:Humans; D015228:Hypertriglyceridemia / Q000517:prevention & control*; D007564:Japan; D007773:Lactates / Q000097:blood*; D019344:Lactic Acid / Q000097:blood*; D008074:Lipoproteins / Q000378:metabolism; D008297:Male; D008892:Milk; D014280:Triglycerides / Q000008:administration & dosage* / Q000097:blood
chemical_list: D015415:Biomarkers; D004040:Dietary Carbohydrates; D007773:Lactates; D008074:Lipoproteins; D014280:Triglycerides; D019344:Lactic Acid; D002784:Cholesterol