{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T19:04:40Z","timestamp":1780340680760,"version":"3.54.1"},"reference-count":15,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2023,7,17]],"date-time":"2023-07-17T00:00:00Z","timestamp":1689552000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2023,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>It is well\u2010established that oral sucrose ingested shortly before exercise improves early exercise tolerance in individuals with McArdle disease. This is by supplying blood\u2010borne glucose for muscle metabolism to compensate for the blocked glycogenolysis. The present study investigated if individuals with McArdle disease could benefit further from repeated sucrose ingestion during prolonged exercise. In this double\u2010blind, placebo\u2010controlled, cross\u2010over study, the participants were randomized to ingest either sucrose or placebo first and subsequently the opposite on two separate days. The participants ingested the drink 10\u2009min before and thrice (after 10, 25, and 40\u2009min) during a 60\u2010min submaximal exercise test on a cycle ergometer. The primary outcome was exercise capacity as indicated by heart rate (HR) and perceived exertion (PE) responses to exercise. Secondary outcomes included changes in blood metabolites, insulin and carbohydrate, and fatty acid oxidation rates during exercise. Nine participants with McArdle disease were included in the study. We confirmed improvement of exercise capacity with oral sucrose vs. placebo during early exercise (pre\u2010second wind) indicated by lower peak HR and PE (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.02). We found no further beneficial effect with repeated sucrose versus placebo ingestion during prolonged exercise, as indicated by no difference in HR or PE post\u2010second wind (<jats:italic>p<\/jats:italic>\u2009&gt;\u20090.05). Glucose, lactate, insulin, and carbohydrate oxidation rates increased, and fatty acid oxidation decreased with sucrose versus placebo (<jats:italic>p<\/jats:italic>\u2009\u2264\u20090.0002). We can conclude that repeated sucrose ingestion is not recommended during prolonged exercise. This finding can prevent excessive caloric intake and reduce the risk of obesity and insulin resistance.<\/jats:p>","DOI":"10.1002\/jimd.12656","type":"journal-article","created":{"date-parts":[[2023,7,11]],"date-time":"2023-07-11T07:18:12Z","timestamp":1689059892000},"page":"1139-1146","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Repeated oral sucrose dosing after the second wind is unnecessary in patients with <scp>McArdle<\/scp> disease: Results from a randomized, placebo\u2010controlled, double\u2010blind, cross\u2010over study"],"prefix":"10.1002","volume":"46","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5494-8484","authenticated-orcid":false,"given":"Nicoline","family":"L\u00f8kken","sequence":"first","affiliation":[{"name":"Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet Copenhagen University Hospital  Copenhagen Denmark"},{"name":"Department of Clinical Medicine University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tahmina","family":"Khawajazada","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet Copenhagen University Hospital  Copenhagen Denmark"},{"name":"Department of Clinical Medicine University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Slipsager","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet Copenhagen University Hospital  Copenhagen Denmark"},{"name":"Department of Clinical Medicine University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicol C.","family":"Voermans","sequence":"additional","affiliation":[{"name":"The Department of Neurology Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen Medical Centre  The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Vissing","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet Copenhagen University Hospital  Copenhagen Denmark"},{"name":"Department of Clinical Medicine University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,7,17]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2021.10.006"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.65.6.786"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa031836"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.59.9.1395"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20881"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12484"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000343002.74480.e4"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.50863"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1249\/00005768-198205000-00012"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469\u20107793.2001.00295.x"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0168\u20108227(11)70014\u20106"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1042\/BCJ20210185"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010020\u201001562\u2010x"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnagi.2014.00334"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/14651858.CD007931.pub2"}],"container-title":["Journal 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