{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T08:27:10Z","timestamp":1769934430298,"version":"3.49.0"},"reference-count":46,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2004,11,3]],"date-time":"2004-11-03T00:00:00Z","timestamp":1099440000000},"content-version":"vor","delay-in-days":2,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Clin Investigation"],"published-print":{"date-parts":[[2004,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Background\u2002<\/jats:bold>Patients with McArdle's disease suffer exercise incapacity as a result of myophosphorylase deficiency, and for a given work rate have excessive circulatory and ventilatory responses. We hypothesized that the rate of increase of oxygen consumption with work rate (\u0394<jats:italic>V<\/jats:italic>O<jats:sub>2<\/jats:sub>\u2010\u0394WR slope) would also be elevated in such patients as a result of these excessive responses.<\/jats:p><jats:p><jats:bold>Patients and methods\u2002<\/jats:bold>Five patients with McArdle's disease and five matched controls carried out a maximal incremental cardiopulmonary exercise test. Controls then carried out a second test matched to the maximal test of a paired patient. Venous blood was sampled at rest, peak exercise and recovery.<\/jats:p><jats:p><jats:bold>Results\u2002<\/jats:bold>During the matched test, the \u0394<jats:italic>V<\/jats:italic>O<jats:sub>2<\/jats:sub>\u2010\u0394WR slope was higher in the patients than in the controls [19\u00b79 (15\u00b70\u201324\u00b76) vs. 11\u00b77 (9\u00b72\u201313\u00b75) mL min<jats:sup>\u22121<\/jats:sup>\u00a0W<jats:sup>\u22121<\/jats:sup>; mean (range);<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7022], and the peak\u2010achieved<jats:italic>V<\/jats:italic>O<jats:sub>2<\/jats:sub>was also greater in the patient group [1201 (890\u20131575) vs. 918 (599\u20131248) mL min<jats:sup>\u22121<\/jats:sup>;<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7003]. A similar pattern was observed for heart rate [173 (165\u2013182) vs. 108 (105\u2013134) b.p.m.;<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7001] and plasma norepinephrine levels [12\u00b76 (9\u00b72\u201319\u00b79) vs. 2\u00b79 (2\u00b72\u20134\u00b79) nmol l<jats:sup>\u22121<\/jats:sup>;<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7003].<\/jats:p><jats:p><jats:bold>Conclusion\u2002<\/jats:bold>There is an increased rate of rise in<jats:italic>V<\/jats:italic>O<jats:sub>2<\/jats:sub>relative to work rate during exercise in patients with McArdle's disease. There is also a greater rise in catecholamines, which may be the result of a physiological response to substrate starvation, and is likely to contribute to the increase in<jats:italic>V<\/jats:italic>O<jats:sub>2<\/jats:sub>.<\/jats:p>","DOI":"10.1111\/j.1365-2362.2004.01423.x","type":"journal-article","created":{"date-parts":[[2004,11,3]],"date-time":"2004-11-03T16:24:24Z","timestamp":1099499064000},"page":"731-737","source":"Crossref","is-referenced-by-count":13,"title":["Oxygen consumption is increased relative to work rate in patients with McArdle's disease"],"prefix":"10.1111","volume":"34","author":[{"given":"C. S.","family":"O'Dochartaigh","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H. Y.","family":"Ong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S. 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