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In 2008, Glycosade\n                      <jats:sup>\u00ae<\/jats:sup>\n                      , an extended-release waxy maize cornstarch, was reported as an alternative to uncooked cornstarch (UCCS) which could prolong the duration of fasting in the GSD population. To date, there has been minimal published experience in (a) young children, (b) the ketotic forms of GSD, and (c) with daytime dosing. The Glyde study was created as a prospective, global initiative to test the efficacy and tolerance of Glycosade use across a broader and more diverse population.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A randomised double-blind cross-over fasting study assessing the tolerance and efficacy of Glycosade compared with cornstarch was performed across disease types and ages. Participants and clinicians chose the product deemed superior, whilst still blinded. Participants were followed for 2 years to assess long-term metabolic control, growth, and quality of life.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Sixty-one participants (age 2\u201362 years; 59% female) were enrolled, and 58 participants completed the fasting studies (28 GSD I; 30 GSD III, VI, IX). Glycosade improved duration of fasting in GSD I and duration of fasting without ketosis in the ketotic forms. Chronic Glycosade use was chosen by 69% of participants. Those treated with Glycosade for the 2-year chronic phase used fewer doses of therapy while markers of metabolic control remained stable.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The Glyde study is the first multi-centre international trial demonstrating the efficacy and tolerance of Glycosade in a large cohort of hepatic GSD patients across a diverse international population. The ability to use fewer doses of therapy per day and avoidance of overnight therapy may improve compliance, safety, and quality of life without sacrificing metabolic control.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13023-024-03274-y","type":"journal-article","created":{"date-parts":[[2024,7,9]],"date-time":"2024-07-09T05:02:12Z","timestamp":1720501332000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Short and long-term acceptability and efficacy of extended-release cornstarch in the hepatic glycogen storage diseases: results from the Glyde study"],"prefix":"10.1186","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1341-9367","authenticated-orcid":false,"given":"DA","family":"Weinstein","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"RJ","family":"Jackson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"EA","family":"Brennan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Williams","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"JE","family":"Davison","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F de","family":"Boer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"TGJ","family":"Derks","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C","family":"Ellerton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B","family":"Faragher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"Gribben","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P","family":"Labrune","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"KM","family":"McKittrick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E","family":"Murphy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"KM","family":"Ross","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"U","family":"Steuerwald","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C","family":"Voillot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"AJM","family":"Woodward","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"HR","family":"Mundy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,7,9]]},"reference":[{"issue":"1","key":"3274_CR1","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1023\/a:1021831621210","volume":"4","author":"JI Wolfsdorf","year":"2003","unstructured":"Wolfsdorf JI, Weinstein DA. 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The study protocols and questionnaire were approved by the Ethical Review Board of the local committee at all participating sites. Written informed consent was obtained from all individual participants included in the study. Eligibility of the participants were at the discretion of the physician. In the United Kingdom (UK) ethical approval was received from the NRES Committee London- Queen Square. Ref: 15\/LO\/0685, IRAS project ID 148762. In France a favourable opinion was received from Comite de Protection des Personnes (CPP) Ile de France XI, CPP ref: 16012. Ethical approval was received from the Institutional Review Board (IRB) of Connecticut Children\u2019s Medical Centre, CCMC IRB# 16\u2013101. In the Netherlands a favourable opinion was received from Universitair Medisch Ethische Toetsingscommissie: University Medical Centre Groningen (METc UMCG). It opened in six international sites and was registered at\n                      \n                      (NCT02318966).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"All participants provided written informed consent before participating in the study. The authors have all approved publication in the <i>Orphanet Journal of Rare Diseases<\/i> and agree to transfer of rights to the publication.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"KMMCK and EAB are employees of Vitaflo International Ltd. All the investigators and sites received grant support for these studies from Vitaflo International, Ltd. DAW also serves as a consultant for Weinstein Rare Disease and Clinical Development Consulting, LLC. JED, MW, PL, EM, KMR, CV, & US have no competing interests. AJMW, JG & CE have received honoraria, research funding and travel expenses from Vitaflo International Limited and Nutricia. HRM has received honoraria, research funding and travel expenses from Vitaflo International Limited and Nutricia. A contract is in place as an advisor with Ultragenyx Pharmaceutical Inc and Kriya Therapeutics for gene therapy studies in GSD. TGJD declares that he has confidentiality agreements with third parties. In the past 36 months, there have been consultation agreements (with Danone, Ultragenyx Pharmaceutical Inc, ModernaTX Inc, and Beam Therapeutics), contracts for financial research support for investigator-initiated research (NCT04311307) and sponsor-initiated research (NCT03517085, NCT03970278, NCT05139316, and NCT05196165), honoraria for lectures or presentations (by MEDTalks, Prelum, and Danone), and participations in a Data Safety Monitoring Board (NCT05095727) and Advisory Boards (Ultragenyx Pharmaceutical Inc, ModernaTX Inc, and Beam Therapeutics). For all private-public collaborations, including this sponsor-initiated clinical trial, contracts are via UMCG Contract Research Desk, and all payments are to UMCG. FdB declares she has confidentiality agreements with third parties. There have been contracts for financial research support for investigator-initiated research (NCT04311307) and sponsor-initiated research (NCT03517085, NCT03970278, NCT05139316). For all private-public collaborations, including this sponsor-initiated clinical trial, contracts are via UMCG Contract Research Desk, and all payments are to UMCG.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"258"}}