{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T18:09:52Z","timestamp":1791828592282,"version":"4.3.4"},"reference-count":46,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,2,22]],"date-time":"2026-02-22T00:00:00Z","timestamp":1771718400000},"content-version":"vor","delay-in-days":21,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T00:00:00Z","timestamp":1769904000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["physoc.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Physiological Reports"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    People with glycogen storage disease type III (GSDIII\u2010p) have a remarkably reduced exercise tolerance. Aim of this study was to analyze the oxygen transport\u2010utilization chain strategies adopted by GSDIII\u2010p during exercise. Nine GSDIII\u2010p (39.4\u2009\u00b1\u200910.0\u2009year, 33% female) and 11 healthy controls (CTRL), age and gender matched, underwent an incremental cardiopulmonary exhaustion test (CPET) to assess peak heart rate (HR), blood lactate [La]p and vastus lateralis O\n                    <jats:sub>2<\/jats:sub>\n                    fractional extraction (\u0394HHb\/isch) using near\u2010infrared spectroscopy. Patterns of breathing (PBr) were assessed accordingly by analyzing pulmonary O\n                    <jats:sub>2<\/jats:sub>\n                    uptake (V\u0307O\n                    <jats:sub>2<\/jats:sub>\n                    ), tidal volume (Vt), respiratory frequency (Rf), end\u2010tidal CO\n                    <jats:sub>2<\/jats:sub>\n                    (PETCO\n                    <jats:sub>2<\/jats:sub>\n                    ) and alveolar ventilation (V\u0307A). GSDIII\u2010p exhibited significantly (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.05) lower peak values of V\u0307O\n                    <jats:sub>2<\/jats:sub>\n                    , pulmonary ventilation (V\u0307E)\u00a0[La] and \u0394HHb\/isch compared to CTRL (1.7\u2009\u00b1\u20090.7 vs. 3.2\u2009\u00b1\u20091.1\u2009L\/min, 50.5\u2009\u00b1\u200919.8 vs. 113.6\u2009\u00b1\u200940.4\u2009L\/min, 1.8\u2009\u00b1\u20090.7 vs. 7.6\u2009\u00b1\u20093.0\u2009mmol\/L and 39.1%\u2009\u00b1\u20099.9% vs. 74.8%\u2009\u00b1\u200936.6%, respectively). The range of peak V\u0307O\n                    <jats:sub>2<\/jats:sub>\n                    values for GSDIII\u2010p, compared to the predicted values for age and sex, was between 79% and 35%. Both GSDIII\u2010p and CTRL were arbitrarily divided into 4 groups according to individual V\u0307E values. GSDIII\u2010p with exercise intolerance relied on increased Rf with inadequate Vt adaptation to maintain V\u0307E and reduce PETCO\n                    <jats:sub>2<\/jats:sub>\n                    , with low V\u0307A values and low to moderate workloads tolerance. Reduced exercise tolerance in GSDIII\u2010p is related to respiratory and skeletal muscle inefficiencies. GSDIII\u2010p strong heterogeneity evaluated throught CPET provides insights into clinical management.\n                  <\/jats:p>","DOI":"10.14814\/phy2.70771","type":"journal-article","created":{"date-parts":[[2026,2,23]],"date-time":"2026-02-23T04:40:07Z","timestamp":1771821607000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Cardiopulmonary and skeletal muscle strategies underlying exhaustive exercise in adults with glycogen storage disease type III"],"prefix":"10.14814","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5777-0821","authenticated-orcid":false,"given":"F.","family":"Lanfranconi","sequence":"first","affiliation":[{"name":"Centro Maria Letizia Verga, Fondazione Monza e Brianza per il bambino e la sua mamma  Monza Italy"},{"name":"AMSD Lecco, Federazione Medico Sportiva Italiana  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Peli","sequence":"additional","affiliation":[{"name":"Centro Maria Letizia Verga, Fondazione Monza e Brianza per il bambino e la sua mamma  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Pollastri","sequence":"additional","affiliation":[{"name":"Centro Maria Letizia Verga, Fondazione Monza e Brianza per il bambino e la sua mamma  Monza Italy"},{"name":"AMSD Lecco, Federazione Medico Sportiva Italiana  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Ferri","sequence":"additional","affiliation":[{"name":"Institute for Health and Sport (IHES) Victoria University  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Tremolizzo","sequence":"additional","affiliation":[{"name":"Neurology Department Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"},{"name":"School of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Conti","sequence":"additional","affiliation":[{"name":"School of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Pieruzzi","sequence":"additional","affiliation":[{"name":"School of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"},{"name":"Nephrology Department Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Miserocchi","sequence":"additional","affiliation":[{"name":"School of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Beretta","sequence":"additional","affiliation":[{"name":"School of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Marzorati","sequence":"additional","affiliation":[{"name":"Istituto Tecnologie Biomediche, Consiglio Nazionale delle Ricerche, LITA  Segrate Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W.","family":"Zardo","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Unit Fondazione IRCCS Istituto Nazionale Tumori  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Gasperini","sequence":"additional","affiliation":[{"name":"Pediatrics Department Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Pretese","sequence":"additional","affiliation":[{"name":"Pediatrics Department Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Paci","sequence":"additional","affiliation":[{"name":"ASST Santi Paolo e Carlo\u2010Presidio San Paolo  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Capelli","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology and Transplantation University of Milano  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Mariani","sequence":"additional","affiliation":[{"name":"Internal Medicine Department, Rare Diseases Unit Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Cattoni","sequence":"additional","affiliation":[{"name":"School of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"},{"name":"Pediatrics Department Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. C.","family":"Balduzzi","sequence":"additional","affiliation":[{"name":"School of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"},{"name":"Pediatrics Department Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Parini","sequence":"additional","affiliation":[{"name":"Internal Medicine Department, Rare Diseases Unit Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"},{"name":"San Raffaele Telethon Institute for Gene Therapy (SR\u2010Tiget)  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,2,22]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1177\/1479972309345927"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.00184.2021"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1183\/20734735.008116"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12355"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1177\/26330040221076497"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11914\u2010013\u20100160\u20105"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1113\/EP092644"},{"key":"e_1_2_12_9_1","volume-title":"GeneReviews\u00ae","author":"Dagli A.","year":"2010"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0735-1097(94)00540-7"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9149(86)90771-X"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2019.01537"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/cphy.c100045"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20708"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1249\/MSS.0000000000002056"},{"key":"e_1_2_12_16_1","doi-asserted-by":"crossref","unstructured":"Grassi B. Porcelli S. &Marzorati M.(2020).Metabolic Myopathies: \u201cHuman Knockout\u201d Models and Translational Medicine.https:\/\/doi.org\/10.3389\/fphys.2020.00350","DOI":"10.3389\/fphys.2020.00350"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1117\/1.JBO.21.9.091313"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005572906807"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010022\u201002184\u20101"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12302"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.1079\/BJN19780152"},{"key":"e_1_2_12_22_1","doi-asserted-by":"publisher","DOI":"10.1249\/00005768-198023000-00009"},{"key":"e_1_2_12_23_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e3181e655b6"},{"key":"e_1_2_12_24_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40478\u2010019\u20100815\u20102"},{"key":"e_1_2_12_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598\u2010017\u201002811\u2010z"},{"key":"e_1_2_12_26_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2020.00968"},{"key":"e_1_2_12_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2005.10.005"},{"key":"e_1_2_12_28_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.129.5.1322"},{"key":"e_1_2_12_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/8904_2011_20"},{"issue":"1","key":"e_1_2_12_30_1","first-page":"72","article-title":"Clinical, biochemical and genetic features of glycogen debranching enzyme deficiency","volume":"26","author":"Lucchiari S.","year":"2007","journal-title":"Acta Myologica"},{"key":"e_1_2_12_31_1","unstructured":"Miserocchi G.(2009).Testo atlante di fisiologia umana. Fisiologia e Fisiopatologia respiratoria. Casa Editrice Ambrosiana. Distribuzione esclusiva Zanichelli."},{"key":"e_1_2_12_32_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00421\u2010022\u201005066\u20103"},{"key":"e_1_2_12_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.resp.2014.12.017"},{"key":"e_1_2_12_34_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00943.2011"},{"key":"e_1_2_12_35_1","doi-asserted-by":"publisher","DOI":"10.1152\/japplphysiol.00782.2009"},{"key":"e_1_2_12_36_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545\u2010014\u20109771\u2010y"},{"key":"e_1_2_12_37_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000001518"},{"key":"e_1_2_12_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2013.02.008"},{"key":"e_1_2_12_39_1","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12224"},{"key":"e_1_2_12_40_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.03.00038503"},{"key":"e_1_2_12_41_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI118799"},{"key":"e_1_2_12_42_1","doi-asserted-by":"publisher","DOI":"10.1249\/00005768-199108000-00006"},{"key":"e_1_2_12_43_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26483"},{"key":"e_1_2_12_44_1","doi-asserted-by":"publisher","DOI":"10.1159\/000441916"},{"key":"e_1_2_12_45_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545\u2010011\u20109388\u20103"},{"key":"e_1_2_12_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2010.06.011"},{"key":"e_1_2_12_47_1","doi-asserted-by":"publisher","DOI":"10.1113\/JP273950"}],"container-title":["Physiological Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/physoc.onlinelibrary.wiley.com\/doi\/pdf\/10.14814\/phy2.70771","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/physoc.onlinelibrary.wiley.com\/doi\/full-xml\/10.14814\/phy2.70771","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/physoc.onlinelibrary.wiley.com\/doi\/pdf\/10.14814\/phy2.70771","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T10:37:47Z","timestamp":1777718267000},"score":1,"resource":{"primary":{"URL":"https:\/\/physoc.onlinelibrary.wiley.com\/doi\/10.14814\/phy2.70771"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2]]},"references-count":46,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,2]]}},"alternative-id":["10.14814\/phy2.70771"],"URL":"https:\/\/doi.org\/10.14814\/phy2.70771","archive":["Portico"],"relation":{},"ISSN":["2051-817X","2051-817X"],"issn-type":[{"value":"2051-817X","type":"print"},{"value":"2051-817X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2]]},"assertion":[{"value":"2025-11-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-01-28","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-02-22","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70771"}}