Journal of Inherited Metabolic Disease

, Volume 29, Issue 4, pp 589–589

Dietary intervention and oxidative phosphorylation capacity

Authors

    • Department of Pediatrics, Nijmegen Centre for Mitochondrial DisordersRadboud University Nijmegen Medical Centre
  • Richard Rodenburg
    • Department of Pediatrics, Nijmegen Centre for Mitochondrial DisordersRadboud University Nijmegen Medical Centre
  • Heidi Zweers van Essen
    • Department of Dietetics, Nijmegen Centre for Mitochondrial DisordersRadboud University Nijmegen Medical Centre
  • Maaike De Vries
    • Department of Pediatrics, Nijmegen Centre for Mitochondrial DisordersRadboud University Nijmegen Medical Centre
  • Jan Smeitink
    • Department of Pediatrics, Nijmegen Centre for Mitochondrial DisordersRadboud University Nijmegen Medical Centre
Short Report

DOI: 10.1007/s10545-006-0227-x

Cite this article as:
Morava, E., Rodenburg, R., van Essen, H.Z. et al. J Inherit Metab Dis (2006) 29: 589. doi:10.1007/s10545-006-0227-x

Summary

Secondary deterioration of mitochondrial function has been reported in patients with anorexia and cancer-related 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-appropriate 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-generating capacity in patients with primary oxidative phosphorylation defects.

Supplementary material

10545_2006_ESM_227.pdf (189 kb)
Dietary intervention and oxidative phosphorylation capacity

Copyright information

© SSIEM and Springer 2006