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For some asymptomatic long\u2010chain defects, long\u2010chain fat restriction plays a minor role, and a normal diet may be introduced. For patients presenting only with myopathic symptoms, e.g., during exercise, treatment may be adapted to energy demand. As a consequence, patients with exercise\u2010induced myopathy may be able to return to normal activity when provided with medium\u2010chain triglycerides (MCT) prior to exercise. There is no need to limit participation in sports. Progression of retinopathy in disorders of the mitochondrial trifunctional protein complex is closely associated with hydroxyacylcarnitine accumulation. A strict low\u2010fat diet with MCT supplementation is recommended to slow or prevent progression of chorioretinopathy. Additional docosahexanoic acid does not prevent the decline in retinal function but does promote nonspecific improvement in visual acuity and is recommended. There is no evidence that L\u2010carnitine supplementation is beneficial. Thus, supplementation with L\u2010carnitine in a newborn identified by screening with either a medium\u2010chain or long\u2010chain defect is not supported. With respect to the use of the odd\u2010chain medium\u2010chain triglyceride triheptanoin in myopathic phenotypes, randomized trials are needed to establish whether triheptanoin is more effective than even\u2010chain MCT. With increasing pathophysiological knowledge, new treatment options have been identified and are being clinically evaluated. These include the use of bezafibrates in myopathic long\u2010chain defects.<\/jats:p>","DOI":"10.1007\/s10545-010-9188-1","type":"journal-article","created":{"date-parts":[[2010,9,9]],"date-time":"2010-09-09T08:09:29Z","timestamp":1284019769000},"page":"555-561","source":"Crossref","is-referenced-by-count":108,"title":["Current issues regarding treatment of mitochondrial fatty acid oxidation disorders"],"prefix":"10.1002","volume":"33","author":[{"given":"Ute","family":"Spiekerkoetter","sequence":"first","affiliation":[{"name":"Department of General Pediatrics University Children's Hospital Moorenstr. 5 Duesseldorf 40225 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean","family":"Bastin","sequence":"additional","affiliation":[{"name":"Facult\u00e9 Necker\u2010Enfants Malades CNRS FRE Paris 3210 France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melanie","family":"Gillingham","sequence":"additional","affiliation":[{"name":"Oregon Health &amp; Science University Portland OR USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Morris","sequence":"additional","affiliation":[{"name":"Willink Biochemical Genetics Unit, Genetic Medicine St Mary's Hospital Manchester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frits","family":"Wijburg","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bridget","family":"Wilcken","sequence":"additional","affiliation":[{"name":"Children's Hospital at Westmead and University of Sydney Sydney Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,9,10]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2000.108398"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc0806334"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI113978"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/labinvest.2009.86"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1203\/01.PDR.0000083001.91588.BB"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2004-1936"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0420.2007.01121.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1096-7192(03)00073-8"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2005.06.001"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2006.06.004"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1086\/522375"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.22284"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02535868"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-006-0355-3"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2002.123282"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2007-971921"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-005-5262-5"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-007-0527-9"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2009.01217.x"},{"key":"e_1_2_8_21_1","doi-asserted-by":"crossref","unstructured":"NasserM JavaheriH FedorowiczZ NooraniZ. 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