{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T16:53:29Z","timestamp":1780592009684,"version":"3.54.1"},"reference-count":0,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2006,6,19]],"date-time":"2006-06-19T00:00:00Z","timestamp":1150675200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2006,8]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Secondary deterioration of mitochondrial function has been reported in patients with anorexia and cancer\u2010related malnutrition. Inadequate nutrition, failure to thrive and feeding problems are also common symptoms in children with primary oxidative phosphorylation defects. As a standard intervention protocol we advise an age\u2010appropriate diet and energy intake in our patients diagnosed with a mitochondrial dysfunction. By comparing the results of the first and the second samples from a group of children who underwent repeated muscle biopsies, we observed biochemical improvement in the mitochondrial function in 7 out of 10 patients following dietary advice and intervention. We suggest evaluating the nutritional state by interpretation of the skeletal muscle biochemistry in patients with a suspected oxidative phosphorylation defect. Since an insufficient dietary intake could play a role in secondary mitochondrial dysfunction, nutritional intervention should be performed prior to the biopsy. On the other hand, our data suggest that optimizing the nutritional and energy intake might also improve the utilization of the residual mitochondrial energy\u2010generating capacity in patients with primary oxidative phosphorylation defects.<\/jats:p>","DOI":"10.1007\/s10545-006-0227-x","type":"journal-article","created":{"date-parts":[[2006,6,19]],"date-time":"2006-06-19T14:44:08Z","timestamp":1150728248000},"page":"589-589","source":"Crossref","is-referenced-by-count":34,"title":["Dietary intervention and oxidative phosphorylation capacity"],"prefix":"10.1002","volume":"29","author":[{"given":"Eva","family":"Morava","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Nijmegen Centre for Mitochondrial Disorders Radboud University Nijmegen Medical Centre Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Rodenburg","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Nijmegen Centre for Mitochondrial Disorders Radboud University Nijmegen Medical Centre Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heidi Zweers","family":"van Essen","sequence":"additional","affiliation":[{"name":"Department of Dietetics, Nijmegen Centre for Mitochondrial Disorders Radboud University Nijmegen Medical Centre Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maaike","family":"De Vries","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Nijmegen Centre for Mitochondrial Disorders Radboud University Nijmegen Medical Centre Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Smeitink","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Nijmegen Centre for Mitochondrial Disorders Radboud University Nijmegen Medical Centre Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,6,19]]},"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-006-0227-x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T12:54:26Z","timestamp":1694782466000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-006-0227-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,6,19]]},"references-count":0,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2006,8]]}},"alternative-id":["10.1007\/s10545-006-0227-x"],"URL":"https:\/\/doi.org\/10.1007\/s10545-006-0227-x","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,6,19]]}}}