{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T11:36:35Z","timestamp":1793878595651,"version":"4.7.0"},"reference-count":78,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,5,31]],"date-time":"2025-05-31T00:00:00Z","timestamp":1748649600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["physoc.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Experimental Physiology"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Glycogen storage disease III (GSD\u2010III) is caused by an inherited deficiency of the glycogen debranching enzyme. Affecting the liver, muscle and heart, GSD\u2010IIIa is the most common GSD\u2010III subtype. We evaluated the feasibility and safety of a physical exercise intervention in patients with GSD\u2010IIIa and its effects at the multisystem level. Nine patients (four female; five children and four adults; 60% of all diagnosed Spanish patients) participated in a 12\u00a0week home\u2010based, remotely supervised programme combining moderate\u2010intensity endurance and resistance exercises. Training tolerance\/adaptation (fatigue severity scale, wellbeing questionnaire) and safety were assessed throughout the intervention period. The following outcomes were determined at baseline and postintervention: Health\u2010related quality of life; muscle, liver, kidney and cardiac damage\/function biomarkers; dual X\u2010ray absorptiometry\u2010determined fat\/muscle\/bone mass; echocardiography\u2010determined cardiac dimensions and left ventricular global longitudinal strain; endurance capacity indicators during cardiopulmonary exercise testing; and muscle strength. The patients completed a median of 88% (interquartile range, 87%\u2013100%) of the prescribed sessions. The intervention was safe and well tolerated, with no changes in wellbeing over time and with a decrease in perceived fatigue severity during the intervention (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.002). Barring alanine aminotransferase, there were no changes in blood biomarkers, and the following significant effects were found at postintervention: Decrease in visceral adipose tissue (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.038); healthier cardiac geometry [from concentric to normal remodelling, with decreases in relative (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.015) and posterior (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.042) wall thickness]; and increases in peak oxygen uptake (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.033), peak power output (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.017) and knee\u2010extension isometric strength (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.012). Although more research is needed, the present findings suggest that exercise should be considered in GSD\u2010IIIa management.\n                  <\/jats:p>","DOI":"10.1113\/ep092644","type":"journal-article","created":{"date-parts":[[2025,5,31]],"date-time":"2025-05-31T09:54:48Z","timestamp":1748685288000},"page":"153-166","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Physical exercise intervention in glycogen storage disease IIIa: Feasibility and multisystem benefits"],"prefix":"10.1113","volume":"111","author":[{"given":"Asunci\u00f3n","family":"Bustos\u2010Sellers","sequence":"first","affiliation":[{"name":"Department of Sport and Computer Science, Section of Physical Education and Sports, Faculty of Sport Universidad Pablo de Olavide Sevilla 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B.","family":"Alejo","sequence":"additional","affiliation":[{"name":"Department of Sports Sciences, Faculty of Medicine, Health and Sports Universidad Europea de Madrid Madrid Spain"},{"name":"Research Institute of Hospital \u201812 de Octubre\u2019 (\u2018imas12\u2019) Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Barranco\u2010Gil","sequence":"additional","affiliation":[{"name":"Department of Sports Sciences, Faculty of Medicine, Health and Sports Universidad Europea de Madrid Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pedro L.","family":"Valenzuela","sequence":"additional","affiliation":[{"name":"Research Institute of Hospital \u201812 de Octubre\u2019 (\u2018imas12\u2019) Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nerea","family":"L\u00f3pez\u2010Maldonado","sequence":"additional","affiliation":[{"name":"Health Center CAP Fundaci\u00f3n Asistencial M\u00fatua Terrasa Barcelona Spain"},{"name":"Spanish Association of Patients With Glycogenosis (AEEG) Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roser","family":"Ferrer\u2010Costa","sequence":"additional","affiliation":[{"name":"Clinical Biochemistry Department Vall d'Hebron University Hospital Barcelona Spain"},{"name":"Clinical Biochemistry, Drug Delivery and Therapy Research Group Vall d'Hebr\u00f3n Research Institute Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Javier","family":"Esparcia\u2010Estrela","sequence":"additional","affiliation":[{"name":"Clinical Biochemistry Department Vall d'Hebron University Hospital Barcelona Spain"},{"name":"Clinical Biochemistry, Drug Delivery and Therapy Research Group Vall d'Hebr\u00f3n Research Institute Barcelona 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