{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T09:57:33Z","timestamp":1753869453983,"version":"3.41.2"},"reference-count":15,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,1,16]],"date-time":"2025-01-16T00:00:00Z","timestamp":1736985600000},"content-version":"vor","delay-in-days":15,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JIMD Reports"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Ingestion of fructose and galactose may result in elevated lactate concentrations in patients with glycogen storage disease type 1 (GSD1). In this randomized cross\u2010over pilot study, 7 patients with GSD 1a (6) and GSD1b (1) orally consumed a common\u2010size fructose and galactose from either 200\u2009mL of skimmed milk, 200\u2009mL juice or 200\u2009mL water. This was given after a night with their usual dietary treatment using either cornstarch, glycosade or continuous feed. P\u2010lactate and \u2010glucose were measured 2\u2009h before dosing (<jats:italic>T<\/jats:italic>\u2009=\u2009\u2212120\u2009min and \u221260\u2009min). At baseline (<jats:italic>T<\/jats:italic>\u2009=\u20090), p\u2010lactate, p\u2010glucose, p\u2010triglycerides, p\u2010uric acid and p\u2010alanine were measured just before dosing. P\u2010lactate and p\u2010glucose were measured every 30\u2009min for 4\u2009h. Four hours after the consumption (<jats:italic>T<\/jats:italic>\u2009=\u2009240\u2009min, end\u2010of\u2010test), levels of p\u2010lactate, p\u2010glucose, p\u2010triglycerides, p\u2010uric acid and p\u2010alanine were measured. P\u2010lactate increased in three patients with mean of 0.3\u2009mmol\/L (range 0.2\u20130.6\u2009mmol\/L) after consuming milk. The highest level was seen after 60\u2009min. A decrease was seen in three patients. P\u2010lactate increased in four patients with a mean increase at 1.3\u2009mmol\/L (range 0.2\u20132.2\u2009mmol\/L) after consuming 200\u2009mL juice. A peak increase was seen after the first 30\u2009min in two patients whereas the peak in the remaining two patients was at 60\u2009min; all values decreased to baseline values after further 60\u2009min. In two patients, p\u2010lactate was unchanged, respectively, decreased after juice ingestion. Calculation of galactose and fructose AUC percentage change after challenge did not reveal consistent increase or decreases.<\/jats:p>","DOI":"10.1002\/jmd2.12457","type":"journal-article","created":{"date-parts":[[2025,1,17]],"date-time":"2025-01-17T05:45:45Z","timestamp":1737092745000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Does p\u2010lactate increase in patients with <scp>GSD1<\/scp> after ingesting a meal with common\u2010size sources of fructose and galactose? Observations from a prospective, non\u2010blinded, crossover pilot study"],"prefix":"10.1002","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-1245-3842","authenticated-orcid":false,"given":"Camilla Diana B.","family":"Caroee","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Center for Inherited Metabolic Diseases Copenhagen University Hospital, Rigshospitalet  Copenhagen Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Allan M.","family":"Lund","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Center for Inherited Metabolic Diseases Copenhagen University Hospital, Rigshospitalet  Copenhagen Denmark"},{"name":"Department of Clinical Genetics, Center for Inherited Metabolic Diseases Copenhagen University Hospital, Rigshospitalet  Copenhagen Denmark"},{"name":"Department of Clinical Medicine, Faculty of Health and Medical Sciences University of Copenhagen  Copenhagen Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,1,16]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02679990"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-8223(93)92246-T"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2014.128"},{"key":"e_1_2_11_5_1","doi-asserted-by":"crossref","first-page":"S15","DOI":"10.1097\/MPG.0b013e3181818ea5","article-title":"Nutritional therapy for glycogen storage diseases","volume":"47","author":"Heller S","year":"2008","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-011-9322-8"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02680007"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1974.tb16992.x"},{"key":"e_1_2_11_9_1","first-page":"470","article-title":"Studies on the utilization of hexoses in liver glycogen disease","volume":"35","author":"Fernandes J","year":"1965","journal-title":"Pediatrics"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.19.4.585"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/advances\/nmz092"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/jmd2.12253"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/8904_2018_131"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.3390\/nu13113828"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.3168\/jds.S0022-0302(82)82198-X"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-005-0095-9"}],"container-title":["JIMD Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jmd2.12457","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,28]],"date-time":"2025-01-28T07:43:43Z","timestamp":1738050223000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jmd2.12457"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1]]},"references-count":15,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2025,1]]}},"alternative-id":["10.1002\/jmd2.12457"],"URL":"https:\/\/doi.org\/10.1002\/jmd2.12457","archive":["Portico"],"relation":{},"ISSN":["2192-8312","2192-8312"],"issn-type":[{"type":"print","value":"2192-8312"},{"type":"electronic","value":"2192-8312"}],"subject":[],"published":{"date-parts":[[2025,1]]},"assertion":[{"value":"2024-07-03","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-10-07","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-01-16","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e12457"}}