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Cited by 2Glycogen Storage Diseases and MyoclonusMetabolism and Genetic DisordersDiet and metabolism studies

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

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Subih, Hadil S., Qudah, Reem A., Janakat, Sana, Rimawi, Hanadi, Elsahoryi, Nour Amin, Alyahya, Linda·2024·Foods
DOI 10.3390/foods13071091PMC PMC11011708
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Abstract

Glycogen storage diseases (GSDs) are a group of carbohydrate metabolism disorders, most of which are inherited in autosomal recessive patterns. GSDs are of two types: those that have to do with liver and hypoglycaemia (hepatic GSDs) and those that are linked to neuromuscular presentation. This study aims to assess the impact of dietary intervention, including medium-chain triglyceride (MCT) oil, on anthropometric measurements, body composition analysis and metabolic parameters among Jordanian children and is expected to be the first in the country. A sample of 38 children with glycogen storage disease type 1 (GSD-1) (median age = 6.4 years) were on a diet that included uncooked cornstarch therapy and a fructose-, sucrose- and lactose-restricted diet. Patients started to take MCT oil along with the prescribed diet after the first body composition test. Patients’ nutritional status was re-evaluated three months later. The study results show that the percentage of patients who suffered from hypoglycaemia at the beginning of the study decreased significantly from 94.7% to 7.9% (p < 0.0001). The serum levels of triglycerides, cholesterol, uric acid and lactate decreased significantly after three months of intervention (100–71.1%, 73.7–21.1%, 97.4–52.6% and 94.7–18.4%, respectively). In contrast, there was no statistical difference in neutrophil count. Regarding clinical parameters, liver span was significantly reduced from (16.01 ± 2.65 cm) to (14.85 ± 2.26 cm) (p < 0.0001). There were significant improvements in growth parameters, including height-for-age and BMI-for-age for children aged ≥2 years (p = 0.034 and p = 0.074, respectively). Significant improvements in skeletal muscle mass and bone mineral content were also noticed at the end of the trial (p ≤ 0.05). In conclusion, medium-chain triglyceride therapy is found to improve biochemical and growth parameters in children with GSD-1 in Jordan.

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  • MCT oil has been found to inhibit de novo lipogenesis and slow glucose uptake.

    “MCT oil has been found to inhibit de novo lipogenesis and slow glucose uptake [39].”

    From Non-Diabetic Hypoglycemia: Evaluation and Management in Adults (2025) · cites this paper as [39]

  • When used as an adjunct to diet modification, MCT oil led to a significant reduction in hypoglycemia, triglycerides, and uric acid levels.

    “When used as an adjunct to diet modification, MCT oil led to a significant reduction in hypoglycemia, triglycerides, and uric acid levels [39].”

    From Non-Diabetic Hypoglycemia: Evaluation and Management in Adults (2025) · cites this paper as [39]

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Authors
Subih, H. S., Qudah, R. A., Janakat, S., Rimawi, H., Elsahoryi, N. A., & Alyahya, L.
Affiliations
  • Jordan University of Science and Technology (JO)
  • Royal Medical Services, P.O. Box 712996, Amman 11171, Jordan
  • Petra University (JO)
  • Universiti Sains Malaysia (MY)
Corresponding author
Subih, Hadil S.
ORCID
Venue
Foods
Publisher
MDPI AG
Published
2024-04-02
Volume
13
Issue
7
Pages
1091
ISSN
2304-8158 (ISSN-L)
DOI
10.3390/foods13071091
PMC
PMC11011708
OpenAlex
W4393409116
Cited by
2
References
1
Citations by year
2025: 2
FWCI
0.65
Type
journal article
Language
en
Open access
Licence
Locations
  • Foods (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Funders
Deanship of Scientific Research at Jordan University of Science and Technology (516/2019)
Topics
  • Health Sciences › Medicine › Rheumatology › Glycogen Storage Diseases and Myoclonus
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Health Sciences › Medicine › Physiology › Diet and metabolism studies
Keywords
glycogen storage disease type 1, medium-chain triglyceride oil, dietary intervention, hypoglycemia, body composition, skeletal muscle mass, bone mineral content, growth parameters, liver span, serum lipids, uric acid, lactate, children

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