Neonatal presentation of coenzyme Q10 deficiency

J Pediatr. 2001 Sep;139(3):456-8. doi: 10.1067/mpd.2001.117575.


We report a neonate with ubiquinone deficiency. Skeletal muscle analysis revealed markedly diminished mitochondrial complex II + III activity that could be restored by addition of a ubiquinone analogue. Ubiquinone deficiency was confirmed by high-performance liquid chromatography. Oral ubiquinone therapy was not associated with clinical improvement; the patient died at 2 years.

Publication types

  • Case Reports

MeSH terms

  • Chromatography, High Pressure Liquid
  • Coenzymes
  • Fatal Outcome
  • Humans
  • Infant, Newborn
  • Male
  • Ubiquinone / analogs & derivatives
  • Ubiquinone / deficiency*
  • Ubiquinone / therapeutic use*


  • Coenzymes
  • Ubiquinone
  • coenzyme Q10