{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T21:55:31Z","timestamp":1790632531466,"version":"4.1.0"},"reference-count":8,"publisher":"S. Karger AG","issue":"3","license":[{"start":{"date-parts":[[2010,1,1]],"date-time":"2010-01-01T00:00:00Z","timestamp":1262304000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2010,1,1]],"date-time":"2010-01-01T00:00:00Z","timestamp":1262304000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Ann Nutr Metab"],"published-print":{"date-parts":[[2010]]},"abstract":"<jats:p>&lt;i&gt;Background\/Objective:&lt;\/i&gt; Hypoketotic hypoglycaemia and hypertriglyceridaemia are biochemical hallmarks of glycogen storage disease (GSD) 1. Increased malonyl coenzyme A production which compromises oxidation of long-chain fatty acids via carnitine palmitoyltransferase (CPT) 1 inhibition plays a crucial role in the pathogenesis of these complications. Therapy consists primarily of nutritional support including frequent carbohydrate-rich meals. We studied the effect of a diet enriched in medium-chain triglycerides (MCT) on metabolic control\/growth in GSD 1 as medium-chain fatty acids can be oxidised independently of CPT 1. &lt;i&gt;Methods:&lt;\/i&gt; An adult female, a 1.6-year-old boy with GSD 1a and a 6.5-year-old girl with GSD 1b treated with a classical GSD diet were enrolled; their \u2018classical GSD diet\u2019 was supplemented with MCT fats. Concentrations of glucose, lactate, ketone bodies triglycerides, uric acid, acylcarnitines in blood and organic acids in urine were determined. &lt;i&gt;Results:&lt;\/i&gt; No clinical or biochemical side-effects were observed. The MCT diet led to a decrease in uric acid concentrations in all patients. Triglyceride levels were reduced only in the youngest patient, while lactate concentrations did not significantly decrease. The MCT diet allowed for a reduction in carbohydrate and caloric intake required to maintain euglycaemia and led to improvement in growth in the two prepubertal patients. &lt;i&gt;Conclusions:&lt;\/i&gt; MCT supplementation had a positive effect on metabolic control and growth in our patients suffering from GSD 1.<\/jats:p>","DOI":"10.1159\/000283242","type":"journal-article","created":{"date-parts":[[2010,3,30]],"date-time":"2010-03-30T07:11:08Z","timestamp":1269933068000},"page":"225-232","source":"Crossref","is-referenced-by-count":25,"title":["Glycogen Storage Disease Type 1: Impact of Medium-Chain Triglycerides on Metabolic Control and Growth"],"prefix":"10.1159","volume":"56","author":[{"given":"Anibh M.","family":"Das","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"L\u00fccke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uta","family":"Meyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans","family":"Hartmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"Illsinger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2010,3,30]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1007%2FBF02680007"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1016%2Fj.plefa.2003.06.003"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1016%2FS0022-3476%2873%2980531-1"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1023%2FA%3A1005504223140"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1196%2Fannals.1320.001"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1007%2Fs00018-005-5121-4"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1203%2FPDR.0b013e31816c9013"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1007%2Fs00431-006-0372-0"}],"container-title":["Annals of Nutrition and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/283242","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/283242","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T21:07:51Z","timestamp":1745442471000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000283242"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010]]},"references-count":8,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2010,4,1]]},"published-print":{"date-parts":[[2010,4,19]]}},"URL":"https:\/\/doi.org\/10.1159\/000283242","archive":["Portico"],"relation":{},"ISSN":["0250-6807","1421-9697"],"issn-type":[{"value":"0250-6807","type":"print"},{"value":"1421-9697","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010]]}}}