{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:57:13Z","timestamp":1760144233126,"version":"build-2065373602"},"reference-count":50,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T00:00:00Z","timestamp":1712016000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004035","name":"Deanship of Scientific Research at Jordan University of Science and Technology","doi-asserted-by":"publisher","award":["516\/2019"],"award-info":[{"award-number":["516\/2019"]}],"id":[{"id":"10.13039\/501100004035","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Foods"],"abstract":"<jats:p>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\u2019 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 &lt; 0.0001). The serum levels of triglycerides, cholesterol, uric acid and lactate decreased significantly after three months of intervention (100\u201371.1%, 73.7\u201321.1%, 97.4\u201352.6% and 94.7\u201318.4%, respectively). In contrast, there was no statistical difference in neutrophil count. Regarding clinical parameters, liver span was significantly reduced from (16.01 \u00b1 2.65 cm) to (14.85 \u00b1 2.26 cm) (p &lt; 0.0001). There were significant improvements in growth parameters, including height-for-age and BMI-for-age for children aged \u22652 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 \u2264 0.05). In conclusion, medium-chain triglyceride therapy is found to improve biochemical and growth parameters in children with GSD-1 in Jordan.<\/jats:p>","DOI":"10.3390\/foods13071091","type":"journal-article","created":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T10:58:31Z","timestamp":1712055511000},"page":"1091","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["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"],"prefix":"10.3390","volume":"13","author":[{"given":"Hadil S.","family":"Subih","sequence":"first","affiliation":[{"name":"Department of Nutrition and Food Technology, Faculty of Agriculture, Jordan University of Science and Technology, P.O. Box 3030, Irbid 22110, Jordan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Reem A.","family":"Qudah","sequence":"additional","affiliation":[{"name":"Department of Nutrition and Food Technology, Faculty of Agriculture, Jordan University of Science and Technology, P.O. Box 3030, Irbid 22110, Jordan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5222-2065","authenticated-orcid":false,"given":"Sana","family":"Janakat","sequence":"additional","affiliation":[{"name":"Department of Nutrition and Food Technology, Faculty of Agriculture, Jordan University of Science and Technology, P.O. Box 3030, Irbid 22110, Jordan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hanadi","family":"Rimawi","sequence":"additional","affiliation":[{"name":"Royal Medical Services, P.O. Box 712996, Amman 11171, Jordan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nour Amin","family":"Elsahoryi","sequence":"additional","affiliation":[{"name":"Department of Nutrition, Faculty of Pharmacy and Medical Sciences, University of Petra, Amman 11196, Jordan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Linda","family":"Alyahya","sequence":"additional","affiliation":[{"name":"School of Pharmaceutical Sciences, Universiti Sains Malaysia, Gelugor 11800, Malaysia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,4,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"R131","DOI":"10.1530\/JOE-18-0120","article-title":"Biochemical and clinical aspects of glycogen storage diseases","volume":"238","author":"Ellingwood","year":"2018","journal-title":"J. Endocrinol."},{"key":"ref_2","first-page":"45","article-title":"Glycogen storage diseases: Diagnosis, treatment and outcome","volume":"1","author":"Chen","year":"2016","journal-title":"Transl. Sci. Rare Dis."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2326409817733009","DOI":"10.1177\/2326409817733009","article-title":"Hepatic glycogen storage diseases: Toward one global collaborative network","volume":"5","author":"Derks","year":"2017","journal-title":"J. Inborn Errors. Metab. Screen."},{"key":"ref_4","first-page":"30","article-title":"Molecular diagnosis of glycogen storage disease type I: A review","volume":"18","author":"Beyzaei","year":"2019","journal-title":"EXCLI J."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1093\/advances\/nmz092","article-title":"Dietary Management of the glycogen storage diseases: Evolution of treatment and ongoing controversies","volume":"11","author":"Ross","year":"2020","journal-title":"Adv. Nutr."},{"key":"ref_6","unstructured":"Bali, D.S., El-Gharbawy, A., Austin, S., Pendyal, S., and Kishnani, P.S. (1993). Glycogen Storage Disease Type I, University of Washington."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1038\/ejcn.2013.186","article-title":"Continuous glucose monitoring in children with glycogen storage disease type I","volume":"68","author":"Kasapkara","year":"2014","journal-title":"Eur. J. Clin. Nutr."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1038\/gim.2014.128","article-title":"Diagnosis and management of glycogen storage disease type I: A practice guideline of the American College of Medical Genetics and Genomics","volume":"16","author":"Kishnani","year":"2014","journal-title":"Genet. Med."},{"key":"ref_9","unstructured":"NORD (2020, January 02). Glycogen Storage Disease Type I. Available online: https:\/\/rarediseases.org\/rare-diseases\/glycogen-storage-disease-type-i\/."},{"key":"ref_10","first-page":"e1548","article-title":"Dietary therapy for Von Gierke\u2019s disease: A Case Report","volume":"9","author":"Raza","year":"2017","journal-title":"Cureus"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1002\/1873-3468.13666","article-title":"Glycogen storage disease type Ib: Role of glucose-6-phosphate transporter in cell metabolism and function","volume":"594","author":"Sim","year":"2020","journal-title":"FEBS Lett."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"474","DOI":"10.21037\/atm.2018.10.59","article-title":"Glycogen metabolism and glycogen storage disorders","volume":"6","author":"Kanungo","year":"2018","journal-title":"Ann. Transl. Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1097\/MCO.0000000000000181","article-title":"Hepatic glycogen storage disorders: What have we learned in recent years?","volume":"18","author":"Burda","year":"2015","journal-title":"Curr. Opin. Clin. Nutr. Metab. Care"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1007\/s10545-015-9811-2","article-title":"Lipids in hepatic glycogen storage diseases: Pathophysiology, monitoring of dietary management and future directions","volume":"38","author":"Derks","year":"2015","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/j.pcl.2017.11.005","article-title":"Inborn errors of metabolism with hypoglycemia: Glycogen storage diseases and inherited disorders of gluconeogenesis","volume":"65","author":"Weinstein","year":"2018","journal-title":"Pediatr. Clin. N. Am."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1186\/2251-6581-12-25","article-title":"Glycogen storage disease type 1a\u2013a secondary cause for hyperlipidemia: Report of five cases","volume":"12","author":"Carvalho","year":"2013","journal-title":"J. Diabetes Metab. Disord."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1016\/j.livres.2017.12.001","article-title":"Recent development and gene therapy for glycogen storage disease type Ia","volume":"1","author":"Chou","year":"2017","journal-title":"Liver Res."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1159\/000283242","article-title":"Glycogen storage disease type 1: Impact of medium-chain triglycerides on metabolic control and growth","volume":"56","author":"Das","year":"2010","journal-title":"Ann. Nutr. Metab."},{"key":"ref_19","first-page":"682","article-title":"Clinical presentation and biochemical findings in children with glycogen storage disease type 1a","volume":"65","author":"Saeed","year":"2015","journal-title":"Pak. Armed Forces Med. J."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"977","DOI":"10.1007\/s10545-018-0199-7","article-title":"Long-term safety and efficacy of AAV gene therapy in the canine model of glycogen storage disease type Ia","volume":"41","author":"Lee","year":"2018","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"183","DOI":"10.3109\/01913123.2011.601404","article-title":"Glycogen storage diseases: A brief review and update on clinical features, genetic abnormalities, pathologic features, and treatment","volume":"35","author":"Hicks","year":"2011","journal-title":"Ultrastruct. Pathol."},{"key":"ref_22","unstructured":"Sundaram, S.S., and Alonso, E.M. (2006). Pediatric Gastrointestinal and Liver Disease, Elsevier Inc."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"985","DOI":"10.1007\/s10545-018-0149-4","article-title":"Insulin-resistance in glycogen storage disease type Ia: Linking carbohydrates and mitochondria?","volume":"41","author":"Rossi","year":"2018","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1038\/gim.2012.36","article-title":"Bone mineral density in glycogen storage disease type Ia and Ib","volume":"14","author":"Minarich","year":"2012","journal-title":"Genet. Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1007\/8904_2014_344","article-title":"Regression of hepatocellular adenomas with strict dietary therapy in patients with glycogen storage disease type I","volume":"Volume 18","author":"Zschocke","year":"2014","journal-title":"JIMD Reports"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1111\/pedi.12540","article-title":"Prevention of complications in glycogen storage disease type Ia with optimization of metabolic control","volume":"18","author":"Dambska","year":"2017","journal-title":"Pediatr. Diabetes"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1186\/s13023-015-0301-2","article-title":"Glycogen storage disease type Ia (GSDIa) but not glycogen storage disease type Ib (GSDIb) is associated to an increased risk of metabolic syndrome: Possible role of microsomal glucose 6-phosphate accumulation","volume":"10","author":"Melis","year":"2015","journal-title":"Orphanet. J. Rare Dis."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.12816\/0044680","article-title":"Glycogen storage disease in pediatric population","volume":"70","author":"Assiri","year":"2018","journal-title":"Egypt. J. Hosp. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"S20","DOI":"10.1007\/BF02679990","article-title":"Glycogen storage disease type I: Diagnosis, management, clinical course and outcome. Results of the European study on glycogen storage disease type I (ESGSD I)","volume":"161","author":"Rake","year":"2002","journal-title":"Eur. J. Pediatr."},{"key":"ref_30","unstructured":"Parikh, N.S., and Ahlawat, R. (2022, February 01). Glycogen Storage Disease Type I, StatPearls [Internet], Available online: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK534196\/."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"S112","DOI":"10.1007\/BF02680007","article-title":"Guidelines for management of glycogen storage disease type I\u2013European study on glycogen storage disease type I (ESGSD I)","volume":"161","author":"Rake","year":"2002","journal-title":"Eur. J. Pediatr."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1177\/0884533615586201","article-title":"Inherited metabolic disorders: Aspects of chronic nutrition management","volume":"30","author":"Boyer","year":"2015","journal-title":"Nutr. Clin. Pract."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.ymgme.2013.02.005","article-title":"Dietary treatment of glycogen storage disease type Ia: Uncooked cornstarch and\/or continuous nocturnal gastric drip-feeding?","volume":"109","author":"Derks","year":"2013","journal-title":"Mol. Genet. Metab."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1186\/1750-1172-6-27","article-title":"Glucose-6-phosphatase deficiency","volume":"6","author":"Froissart","year":"2011","journal-title":"Orphanet. J. Rare Dis."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"239","DOI":"10.5223\/pghn.2014.17.4.239","article-title":"Does type I truly dominate hepatic glycogen storage diseases in Korea?: A single center study","volume":"17","author":"Jeong","year":"2014","journal-title":"Pediatr. Gastroenterol. Hepatol. Nutr."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1016\/j.jped.2014.02.005","article-title":"Glycogen storage disease type I: Clinical and laboratory profile","volume":"90","author":"Santos","year":"2014","journal-title":"J. Pediatr."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.ymgme.2018.07.004","article-title":"Safety issues associated with dietary management in patients with hepatic glycogen storage disease","volume":"125","author":"Steunenberg","year":"2018","journal-title":"Mol. Genet. Metab."},{"key":"ref_38","doi-asserted-by":"crossref","unstructured":"Bernstein, L.E., Rohr, F., and Helm, J.R. (2015). Nutrition Management of Inherited Metabolic Diseases, Springer International Publishing.","DOI":"10.1007\/978-3-319-14621-8"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/j.clnesp.2017.03.002","article-title":"Body composition measurements using bioelectrical impedance analysis (BIA) in pediatric patients with hepatic glycogen storage disease\u2013Preliminary data","volume":"19","author":"Szymanska","year":"2017","journal-title":"Clin. Nutr. ESPEN"},{"key":"ref_40","doi-asserted-by":"crossref","unstructured":"Shaw, V. (2015). Clinical Paediatric Dietetics, John Wiley & Sons Ltd.. [4th ed.].","DOI":"10.1002\/9781118915349"},{"key":"ref_41","first-page":"1","article-title":"Use of World Health Organization and CDC Growth Charts for Children Aged 0-59 Months in the United States","volume":"59","author":"Krebs","year":"2010","journal-title":"MMWR Surveill Summ."},{"key":"ref_42","unstructured":"The Study Spot (2023, January 02). Von Gierke (Glycogen Storage Disease 1) [Video File]. 14 December 2014. Available online: https:\/\/youtu.be\/p4dU4deqssw."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"S65","DOI":"10.1007\/BF02679998","article-title":"Disturbed lipid metabolism in glycogen storage disease type 1","volume":"161","author":"Bandsma","year":"2002","journal-title":"Eur. J. Pediatr."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1007","DOI":"10.1007\/s10545-018-0180-5","article-title":"Molecular biology and gene therapy for glycogen storage disease type Ib","volume":"41","author":"Chou","year":"2018","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"2843","DOI":"10.1182\/blood-2013-05-502435","article-title":"Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib","volume":"123","author":"Jun","year":"2014","journal-title":"Blood"},{"key":"ref_46","first-page":"387","article-title":"Dietary or enteral medium-chain triglyceride usage in a Chinese general hospital","volume":"24","author":"Li","year":"2015","journal-title":"Asia Pac. J. Clin. Nutr."},{"key":"ref_47","first-page":"20","article-title":"The use of medium-chain triglycerides in gastrointestinal disorders","volume":"160","author":"Shah","year":"2017","journal-title":"Pract. Gastroenterol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.1007\/s00431-006-0372-0","article-title":"Improvements of hypertriglyceridemia and hyperlacticemia in Japanese children with glycogen storage disease type Ia by medium-chain triglyceride milk","volume":"166","author":"Nagasaka","year":"2007","journal-title":"Eur. J. Pediatr."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1007\/8904_2015_461","article-title":"Vitamin E improves clinical outcome of patients affected by glycogen storage disease type Ib","volume":"25","author":"Melis","year":"2015","journal-title":"JIMD Rep."},{"key":"ref_50","first-page":"286","article-title":"Diet supplemented with MCT oil in the management of childhood diarrhea","volume":"16","author":"Tanchoco","year":"2007","journal-title":"Asia Pac. J. Clin. Nutr."}],"container-title":["Foods"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2304-8158\/13\/7\/1091\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:22:27Z","timestamp":1760106147000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2304-8158\/13\/7\/1091"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,2]]},"references-count":50,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2024,4]]}},"alternative-id":["foods13071091"],"URL":"https:\/\/doi.org\/10.3390\/foods13071091","relation":{},"ISSN":["2304-8158"],"issn-type":[{"type":"electronic","value":"2304-8158"}],"subject":[],"published":{"date-parts":[[2024,4,2]]}}}