Dietary intervention and oxidative phosphorylation capacity.
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.
Morava
Eva
E
Department of Pediatrics, Nijmegen Centre for Mitochondrial Disorders, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands, E.Morava@cukz.umcn.nl.
Rodenburg
Richard
R
van Essen
Heidi Zweers
HZ
De Vries
Maaike
M
Smeitink
Jan
J
D000255:Adenosine Triphosphate / Q000378:metabolism; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D004032:Diet; D019587:Dietary Supplements; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008931:Mitochondria, Muscle / Q000201:enzymology / Q000378:metabolism; D028361:Mitochondrial Diseases / Q000178:diet therapy* / Q000378:metabolism; D010085:Oxidative Phosphorylation
D000255:Adenosine Triphosphate