Decreased coenzyme Q10 concentration in plasma of children with cystic fibrosis.
OBJECTIVE
Coenzyme Q10 (CoQ10) is an effective lipophilic antioxidant and protects against lipid peroxidation by scavenging radicals. Patients with cystic fibrosis generally have fat malabsorption; thus, we hypothesized that overall plasma CoQ10 concentration in pediatric patients with cystic fibrosis might be diminished. Because these patients have increased oxidative stress due to chronic pulmonary inflammation, we also assumed that the oxidized form of CoQ10 might be relatively increased.


METHODS
The total plasma CoQ10 levels and the oxidized and reduced form were measured by high-performance liquid chromatography in 30 children with cystic fibrosis (mean FEV1 % predicted = 88.5% +/- 18.7%) and 30 age-matched controls.


RESULTS
Total plasma CoQ10 levels were significantly lower in the cystic fibrosis group as compared with the control group (0.87 +/- 0.42 micromol/L and 1.35 +/- 0.39 micromol/L, respectively; P < 0.001). When correcting for the lower serum cholesterol level in patients with cystic fibrosis, this difference remained significant: the CoQ10/cholesterol ratio (micromol/mol) was 268.8 +/- 136.7 and 334.0 +/- 102.9 in patients and controls, respectively (P < 0.05). However, the CoQ10 redox status was identical in patients and controls (86.4% +/- 7.1% and 85.4% +/- 7.3%, respectively).


CONCLUSIONS
We found that the overall plasma CoQ10 concentration is lower in patients with cystic fibrosis, probably because of fat malabsorption. The CoQ10 redox status was not disturbed, indicating that CoQ10 could still be adequately regenerated in this group of patients with cystic fibrosis with mild-to-moderate pulmonary disease.
Oudshoorn
Johanna H
JH
Department of Pediatric Gastroenterology, Wilhelmina Children's Hospital, UMC Utrecht, The Netherlands. pos.oudshoorn@planet.nl
Lecluse
Anne Ly
AL
van den Berg
Robin
R
Vaes
Wouter H J
WH
van der Laag
Johan
J
Houwen
Roderick H J
RH
D000293:Adolescent; D002648:Child; D002784:Cholesterol / Q000097:blood; D002851:Chromatography, High Pressure Liquid; D003067:Coenzymes; D003550:Cystic Fibrosis / Q000097:blood*; D005260:Female; D006801:Humans; D008297:Male; D010084:Oxidation-Reduction; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000097:blood
D003067:Coenzymes; D014451:Ubiquinone; D002784:Cholesterol; C024989:coenzyme Q10