{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T15:06:54Z","timestamp":1791558414218,"version":"4.3.4"},"reference-count":22,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,3,3]],"date-time":"2021-03-03T00:00:00Z","timestamp":1614729600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Oxygen uptake efficiency slope (OUES) is a noninvasive cardiopulmonary exercise testing (CPET) measurement based on oxygen uptake (V\u02d9O<jats:sub>2<\/jats:sub>) and minute ventilation (V\u02d9E) and is a marker of the efficiency of oxygen utilization by the body. However, it has not been studied in mitochondrial disorders. We explored noninvasive CPET parameters, including OUES, as a way to reliably diagnose mitochondrial myopathy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We performed cycle ergometer maximal exercise testing on definite and suspected mitochondrial myopathy subjects (MM\u2010D and MM\u2010S) and their age\u2010 and sex\u2010matched controls. OUES was corrected for body surface area (OUES\/BSA) to eliminate the effect of body size.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 40 participants, including 20\u2009MM\u2010D (n = 13; 6 males; aged 14\u201064\u2009years) and 7\u2009MM\u2010S (5 males, aged 11\u201030\u2009years) subjects and 20 controls, completed the study. MM\u2010D subjects showed lower aerobic fitness than controls. OUES\/BSA was lower in MM\u2010D subjects, suggesting inefficient oxygen utilization. Area under the curve (AUC) and 95% confidence interval (CI) for OUES\/BSA (AUC, 0.91; 95% CI, 0.80\u20101.00), peak V\u02d9O<jats:sub>2<\/jats:sub> percent predicted (AUC, 0.95; 95% CI, 0.86\u20101.00), and V\u02d9O<jats:sub>2<\/jats:sub>\/work slope (AUC, 0.94; 95% CI, 0.85\u20101.00) showed excellent ability to diagnose mitochondrial myopathy in MM\u2010D subjects. We applied a diagnostic approach based on the parameters just noted to MM\u2010S subjects and their controls and were able to support or disprove the diagnosis of mitochondrial myopathy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>We proposed and applied an approach based on the aformentioned three CPET parameters to diagnose mitochondrial myopathy reliably and found it to be clinically useful.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27191","type":"journal-article","created":{"date-parts":[[2021,2,5]],"date-time":"2021-02-05T14:31:16Z","timestamp":1612535476000},"page":"715-723","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["A novel exercise testing algorithm to diagnose mitochondrial myopathy"],"prefix":"10.1002","volume":"63","author":[{"given":"Rajeev","family":"Bhatia","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Pulmonology Akron Children's Hospital  Akron Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruce H.","family":"Cohen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Neurodevelopmental Science Center\u2013Division of Neurology Akron Children's Hospital  Akron Ohio"},{"name":"Northeast Ohio Medical University  Rootstown Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil","family":"L. McNinch","sequence":"additional","affiliation":[{"name":"Rebecca D Considine Research Institute Akron Children's Hospital  Akron Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,3,3]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2018-105800"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2014.177"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.164.3.2005110"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awg028"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201701-014FR"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0b013e31826fb946"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.2165\/00007256-199927010-00003"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201611-852FR"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00421-005-1343-2"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(96)00412-3"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2004.07.004"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1161\/JAHA.116.005037"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000033795.17156.00"},{"key":"e_1_2_9_15_1","first-page":"700","article-title":"Normal standards for an incremental progressive cycle ergometer test","volume":"131","author":"Jones NL","year":"1985","journal-title":"Am Rev Respir Dis."},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1164\/arrd.1984.129.2P2.S47"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1249\/MSS.0000000000001314"},{"key":"e_1_2_9_18_1","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1183\/09031936.93.01050445","article-title":"Exercise responses in patients with an enzyme deficiency in the mitochondrial respiratory chain","volume":"1","author":"Bogaard JM","year":"1988","journal-title":"Eur Respir J."},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-7753(00)71216-4"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1249\/MSS.0b013e3181aae96b"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpregu.00001.2011"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(00)00691-4"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1249\/01.mss.0000177341.89478.06"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.27191","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mus.27191","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.27191","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T16:42:52Z","timestamp":1693327372000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.27191"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,3]]},"references-count":22,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2021,5]]}},"alternative-id":["10.1002\/mus.27191"],"URL":"https:\/\/doi.org\/10.1002\/mus.27191","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,3,3]]},"assertion":[{"value":"2020-03-04","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-01-31","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-03-03","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}