ArticleEuropean journal of pediatrics2007
Improvements of hypertriglyceridemia and hyperlacticemia in Japanese children with glycogen storage disease type Ia by medium-chain triglyceride milk.
Article in European journal of pediatrics, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03665636 (Anaplerotic Therapy Using Triheptanoin for Patients With Glycogen Storage Disease Type I), which is not on this map. Cited by 15 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Anaplerotic Therapy Using Triheptanoin for Patients With Glycogen Storage Disease Type I
Who cites it
15 citing papers in PubMed, 1 synthesis or guideline pooled it, 30 citations in OpenAlex.
- Dietary lipids in glycogen storage disease type III: A systematic literature study, case studies, and future recommendations.Journal of inherited metabolic disease · 2020Pooled it
- Medium-Chain Triglyceride Oil and Dietary Intervention Improved Body Composition and Metabolic Parameters in Children with Glycogen Storage Disease Type 1 in Jordan: A Clinical Trial.Foods (Basel, Switzerland) · 2024Article
- Glycogen storage diseases: An update.World journal of gastroenterology · 2023Review
- Glycogen storage disease type 1a in the Ohio Amish.JIMD reports · 2022Article
- A Novel Lipoprotein Lipase Mutation in an Infant With Glycogen Storage Disease Type-Ib and Severe Hypertriglyceridemia.Frontiers in pediatrics · 2021Article
- Preclinical Research in Glycogen Storage Diseases: A Comprehensive Review of Current Animal Models.International journal of molecular sciences · 2020Review
- The Diagnostic Criteria 2020 for Triglyceride Deposit Cardiomyovasculopathy.Annals of nuclear cardiology · 2020Review
- Insulin-resistance in glycogen storage disease type Ia: linking carbohydrates and mitochondria?Journal of inherited metabolic disease · 2018Article
- Non-ketogenic combination of nutritional strategies provides robust protection against seizures.Scientific reports · 2017Article
- Natural Progression of Canine Glycogen Storage Disease Type IIIa.Comparative medicine · 2016Article
- Inherited Metabolic Disorders: Aspects of Chronic Nutrition Management.Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition · 2015Review
- Lipids in hepatic glycogen storage diseases: pathophysiology, monitoring of dietary management and future directions.Journal of inherited metabolic disease · 2015Review
- Glycogen Storage Disease type 1a - a secondary cause for hyperlipidemia: report of five cases.Journal of diabetes and metabolic disorders · 2013Article
- Dietary dilemmas in the management of glycogen storage disease type I.Journal of inherited metabolic disease · 2011Review
- Glycogen storage disease type I and G6Pase-β deficiency: etiology and therapy.Nature reviews. Endocrinology · 2010Review
Corrections and comments
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Authors and funding
11 authors at 8 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundBesides 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
methodsA 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.
resultsMean 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.
conclusionWe 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.
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