Decreased total serum coenzyme-Q10 concentrations: a longitudinal study in children with cystic fibrosis.
OBJECTIVE
To assess total serum levels of coenzyme Q(10) (Co-Q(10)), an important antioxidant, in children with cystic fibrosis (CF) and to investigate an association between Co-Q(10) level and clinical outcome.


METHODS
Co-Q(10) levels were measured annually in a prospective cohort study of 381 children with CF. A total of 1092 serum levels of total Co-Q(10) were obtained by high-performance liquid chromatography and ultraviolet light detection. Associations of Co-Q(10) with demographic variables and clinical outcomes were investigated.


RESULTS
Of the 381 initial total serum Co-Q(10) measurements, 188 were in the deficient range. Low Co-Q(10) was significantly more prevalent in patients with pancreatic insufficiency (PI) (55%) compared with patients with pancreatic sufficiency (PS) (3%); 22% of the patients with PI exhibited persistently low Co-Q(10) levels. Low Co-Q(10) levels were significantly associated with Pseudomonas aeruginosa colonization in patients with PI and CF under age 24 months, but not with subsequent lung function or hospitalization rates. Low Co-Q(10) levels were related to other markers of nutritional status, including total lipids, beta-carotene, and alpha-tocopherol.


CONCLUSIONS
Persistently low total serum Co-Q(10) levels are common in children with CF and PI. A prospective study is indicated to determine whether Co-Q(10) supplementation in CF is beneficial.
Department of Pediatrics, University of Colorado, Denver, School of Medicine, Aurora, CO, USA.
Theresa A
TA
Laguna
Marci K
MK
Sontag
Iris
I
Osberg
Jeffrey S
JS
Wagener
Frank J
FJ
Accurso
Ronald J
RJ
Sokol
D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D002851:Chromatography, High Pressure Liquid; D003550:Cystic Fibrosis / Q000201:enzymology*; D018450:Disease Progression; D045222:Electron Transport Chain Complex Proteins; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D011446:Prospective Studies; D012720:Severity of Illness Index; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000097:blood
D015415:Biomarkers; D045222:Electron Transport Chain Complex Proteins; D014451:Ubiquinone; C024989:coenzyme Q10