{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T21:54:46Z","timestamp":1790805286626,"version":"4.1.0"},"reference-count":47,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2020,9,7]],"date-time":"2020-09-07T00:00:00Z","timestamp":1599436800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2021,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:label\/>\n                    <jats:p>\n                      Glycogen storage disease type IIIa (GSDIIIa) is an inborn error of carbohydrate metabolism caused by a debranching enzyme deficiency. A subgroup of GSDIIIa patients develops severe myopathy. The purpose of this study was to investigate whether acute nutritional ketosis (ANK) in response to ketone\u2010ester (KE) ingestion is effective to deliver oxidative substrate to exercising muscle in GSDIIIa patients. This was an investigator\u2010initiated, researcher\u2010blinded, randomized, crossover study in six adult GSDIIIa patients. Prior to exercise subjects ingested a carbohydrate drink (~66\u2009g, CHO) or a ketone\u2010ester (395\u2009mg\/kg, KE)\u2009+\u2009carbohydrate drink (30\u2009g, KE\u2009+\u2009CHO). Subjects performed 15\u2010minute cycling exercise on an upright ergometer followed by 10\u2010minute supine cycling in a magnetic resonance (MR) scanner at two submaximal workloads (30% and 60% of individual maximum, respectively). Blood metabolites, indirect calorimetry data, and in\u2009vivo\n                      <jats:sup>31<\/jats:sup>\n                      P\u2010MR spectra from quadriceps muscle were collected during exercise. KE\u2009+\u2009CHO induced ANK in all six subjects with median peak \u03b2HB concentration of 2.6\u2009mmol\/L (range: 1.6\u20103.1). Subjects remained normoglycemic in both study arms, but delta glucose concentration was 2\u2010fold lower in the KE\u2009+\u2009CHO arm. The respiratory exchange ratio did not increase in the KE\u2009+\u2009CHO arm when workload was doubled in subjects with overt myopathy. In\u2009vivo\n                      <jats:sup>31<\/jats:sup>\n                      P MR spectra showed a favorable change in quadriceps energetic state during exercise in the KE\u2009+\u2009CHO arm compared to CHO in subjects with overt myopathy. Effects of ANK during exercise are phenotype\u2010specific in adult GSDIIIa patients. ANK presents a promising therapy in GSDIIIa patients with a severe myopathic phenotype.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Trial registration number<\/jats:title>\n                    <jats:p>ClinicalTrials.gov identifier: NCT03011203.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jimd.12302","type":"journal-article","created":{"date-parts":[[2020,8,16]],"date-time":"2020-08-16T09:00:45Z","timestamp":1597568445000},"page":"226-239","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease type\n                    <scp>IIIa<\/scp>\n                    are phenotype\u2010specific: An investigator\u2010initiated, randomized, crossover study"],"prefix":"10.1002","volume":"44","author":[{"given":"Irene J.","family":"Hoogeveen","sequence":"first","affiliation":[{"name":"Section of Metabolic Diseases, Beatrix Children's Hospital University of Groningen, University Medical Center of Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Foekje","family":"de Boer","sequence":"additional","affiliation":[{"name":"Section of Metabolic Diseases, Beatrix Children's Hospital University of Groningen, University Medical Center of Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Willemijn F.","family":"Boonstra","sequence":"additional","affiliation":[{"name":"Section of Metabolic Diseases, Beatrix Children's Hospital University of Groningen, University Medical Center of Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline J.","family":"van der Schaaf","sequence":"additional","affiliation":[{"name":"Section of Metabolic Diseases, Beatrix Children's Hospital University of Groningen, University Medical Center of Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrike","family":"Steuerwald","sequence":"additional","affiliation":[{"name":"National Hospital of the Faroe Islands, Medical Center  T\u00f3rshavn Faroe Islands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita J.","family":"Sibeijn\u2010Kuiper","sequence":"additional","affiliation":[{"name":"Neuroimaging Center, Department of Neuroscience University Medical Center Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riemer J. 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Rebecca","family":"Heiner\u2010Fokkema","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Laboratory of Metabolic Diseases, University Medical Center Groningen University of Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kieran C.","family":"Clarke","sequence":"additional","affiliation":[{"name":"Department of Physiology, Anatomy and Genetics University of Oxford  Oxford UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pete J.","family":"Cox","sequence":"additional","affiliation":[{"name":"Department of Physiology, Anatomy and Genetics University of Oxford  Oxford UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7259-1095","authenticated-orcid":false,"given":"Terry G. 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