Document Detail

Improvements of hypertriglyceridemia and hyperlacticemia in Japanese children with glycogen storage disease type Ia by medium-chain triglyceride milk.
MedLine Citation:
PMID:  17206455     Owner:  NLM     Status:  MEDLINE    
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. PATIENTS AND 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. CONCLUSION: 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.
Hironori Nagasaka; Ken-ichi Hirano; Akira Ohtake; Takashi Miida; Tomozumi Takatani; Kei Murayama; Tohru Yorifuji; Kunihiko Kobayashi; Masaki Kanazawa; Atsushi Ogawa; Masaki Takayanagi
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2007-01-06
Journal Detail:
Title:  European journal of pediatrics     Volume:  166     ISSN:  0340-6199     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  2007 Oct 
Date Detail:
Created Date:  2007-08-27     Completed Date:  2008-11-18     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  1009-16     Citation Subset:  IM    
Division of Metabolism, Chiba Children's Hospital, 579-1 Heta Cho Midori-Ku, Chiba, Japan.
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MeSH Terms
Biological Markers / blood
Child, Preschool
Cholesterol / blood
Dietary Carbohydrates / metabolism
Dietary Supplements
Glycogen Storage Disease Type I / blood,  complications,  diet therapy*
Hypertriglyceridemia / prevention & control*
Lactates / blood*
Lactic Acid / blood*
Lipoproteins / metabolism
Triglycerides / administration & dosage*,  blood
Reg. No./Substance:
0/Biological Markers; 0/Dietary Carbohydrates; 0/Lactates; 0/Lipoproteins; 0/Triglycerides; 50-21-5/Lactic Acid; 57-88-5/Cholesterol

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