{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T02:16:32Z","timestamp":1789179392742,"version":"build-2803163510"},"reference-count":19,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2004,10,13]],"date-time":"2004-10-13T00:00:00Z","timestamp":1097625600000},"content-version":"vor","delay-in-days":4272,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[1993,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Plasma carnitine \u201cinsufficiency,\u201d (plasma esterified carnitine to free carnitine ratio above 0.25) was found in 21 48 (43.8%) patients with mitochondrial myopathy, of whom 4 also showed both total and free carnitine deficiencies in plasma. In addition, plasma levels of SCAC and LCAC were higher in patients with mitochondrial myopathy than in controls (<jats:italic>P<\/jats:italic> &lt; 0.001 and <jats:italic>P<\/jats:italic> &lt;0.01, respectively). Patients diagnosed as having plasma carnitine insufficiency or deficiency were treated with L\u2010carnitine (50\u2013200 mg\/kg per day in four daily doses). Muscle weakness improved in 19 of 20 patients, failure to thrive in 4 of 8, encephalopathy in 1 of 9, and cardiomyopathy in 8 of 8 patients. Plasma carnitine \u201cinsufficiency\u201d provides an additional clue to the diagnosis of mitochondrial myopathy and an indication for L\u2010carnitine therapy. \u00a9 1993 John Wiley &amp; Sons, Inc.<\/jats:p>","DOI":"10.1002\/mus.880160205","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T08:09:25Z","timestamp":1104566965000},"page":"150-153","source":"Crossref","is-referenced-by-count":80,"title":["Plasma carnitine insufficiency and effectiveness of <scp>L<\/scp>\u2010carnitine therapy in patients with mitochondril myopathy"],"prefix":"10.1002","volume":"16","author":[{"given":"Yolanda","family":"Campos,","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosa","family":"Huertas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gustavo","family":"Lorenzo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan","family":"Bautista","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo","family":"Gutierrez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuel","family":"Aparicio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis","family":"Alesso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joaquin","family":"Arenas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,10,13]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880130303"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.bi.57.070188.001401"},{"key":"e_1_2_1_4_2","first-page":"982","article-title":"Carnitine deficiency","volume":"335","author":"Chalmers RA","year":"1990","journal-title":"Lancet"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-198412000-00021"},{"key":"e_1_2_1_6_2","first-page":"45","article-title":"Follow\u2010up and treatment of carnitine deficiencies in man","volume":"7","author":"Di Donato S","year":"1987","journal-title":"Persp Inher Metab Dis"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(84)90187-6"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410170602"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.30.8.795"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(90)90414-Z"},{"key":"e_1_2_1_11_2","first-page":"832","article-title":"Carnitine\u2010palmitoyltransferase. 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