{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:46:11Z","timestamp":1786977971004,"version":"build-2736575974"},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2020,2,24]],"date-time":"2020-02-24T00:00:00Z","timestamp":1582502400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100003554","name":"Lundbeckfonden","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003554","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2020,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Glycogen storage disease type V (GSDV) is a rare inborn error of carbohydrate metabolism. Patients present with exercise intolerance due to blocked glycogen breakdown in skeletal muscle. Introducing alternative fuel substrates, such as ketone bodies (KBs), could potentially alleviate muscle symptoms. This pilot study investigates which of three different modified ketogenic diet regimes is optimal for GSDV\u2010patients to follow in a future large\u2010scale study. Participants were randomised to follow one of three diet regimes for 3\u2009weeks (#1: 65%\/15%\/20%; #2: 75%\/15%\/10%, or #3: 80%\/15%\/5%, fat\/protein\/carbohydrate). The primary outcome was exercise tolerance assessed by heart rate (HR) changes during constant load cycling. Secondary outcomes included levels of ketosis, and changes in perceived exertion and indirect calorimetry measures during exercise. Ten GSDV\u2010patients were included. Eight completed the study. The other two were excluded. Diet #3 showed the highest average KB level (1.1\u2009mmol\/L) vs #2 (0.5\u2009mmol\/L) and #1 (0.3\u2009mmol\/L). Five patients reported subjective symptom relief, all of whom were on diets #2 and #3. All diet regimes seemed to improve fatty acid oxidation rates and exercise capacity as indicated by a small decrease in HR and perceived exertion. The results of this open\u2010label pilot study show that diets #2 and #3 induce ketosis and improve symptoms and exercise capacity in GSDV\u2010patients. Diet #2 had the highest acceptability score and was superior or equal to diet #3 in all other parameters, except level of ketosis. Based on this, we suggest testing diet #2 in a large\u2010scale, placebo\u2010controlled study in GSDV.<\/jats:p>","DOI":"10.1002\/jimd.12223","type":"journal-article","created":{"date-parts":[[2020,2,15]],"date-time":"2020-02-15T04:10:15Z","timestamp":1581739815000},"page":"778-786","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":28,"title":["Titrating a modified ketogenic diet for patients with McArdle disease: A pilot study"],"prefix":"10.1002","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5494-8484","authenticated-orcid":false,"given":"Nicoline","family":"L\u00f8kken","sequence":"first","affiliation":[{"name":"Copenhagen Neuromuscular Center, Rigshospitalet, Copenhagen University Hospital  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kit K.","family":"Hansen","sequence":"additional","affiliation":[{"name":"The Institute of Neurology, National Hospital for Neurology and Neurosurgery  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jesper H.","family":"Storgaard","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center, Rigshospitalet, Copenhagen University Hospital  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mette C.","family":"\u00d8rngreen","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center, Rigshospitalet, Copenhagen University Hospital  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ros","family":"Quinlivan","sequence":"additional","affiliation":[{"name":"The Institute of Neurology, National Hospital for Neurology and Neurosurgery  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Vissing","sequence":"additional","affiliation":[{"name":"Copenhagen Neuromuscular Center, Rigshospitalet, Copenhagen University Hospital  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,2,24]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2009.195040"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410170216"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa031836"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20565"},{"key":"e_1_2_12_6_1","unstructured":"IAMGSD. 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