Decreased Total Serum Coenzyme-Q10 Concentrations: A Longitudinal Study in Children with Cystic Fibrosis

Received 12 June 2007; received in revised form 12 December 2007; accepted 18 March 2008. published online 03 June 2008.

Objective

To assess total serum levels of coenzyme Q10 (Co-Q10), an important antioxidant, in children with cystic fibrosis (CF) and to investigate an association between Co-Q10 level and clinical outcome.

Study design

Co-Q10 levels were measured annually in a prospective cohort study of 381 children with CF. A total of 1092 serum levels of total Co-Q10 were obtained by high-performance liquid chromatography and ultraviolet light detection. Associations of Co-Q10 with demographic variables and clinical outcomes were investigated.

Results

Of the 381 initial total serum Co-Q10 measurements, 188 were in the deficient range. Low Co-Q10 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-Q10 levels. Low Co-Q10 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-Q10 levels were related to other markers of nutritional status, including total lipids, beta-carotene, and alpha-tocopherol.

Conclusions

Persistently low total serum Co-Q10 levels are common in children with CF and PI. A prospective study is indicated to determine whether Co-Q10 supplementation in CF is beneficial.

Abbreviations: CF, Cystic fibrosis, CFTR, Cystic fibrosis transmembrane conductance regulator, Co-Q10, Coenzyme-Q10, FEV1, Forced expiratory volume in 1 second, FVC, Forced vital capacity, HPLC, High-performance liquid chromatography, PI, Pancreatic insufficiency, PS, Pancreatic sufficiency

a From the Department of Pediatrics, University of Colorado, Denver, School of Medicine, Aurora, CO

b Department of Preventive Medicine and Biometrics, University of Colorado, Denver, School of Medicine, Aurora, CO

c Mike McMorris Cystic Fibrosis Research and Treatment Center, The Children's Hospital, Denver, CO

d Section of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital, Denver, CO

Reprint requests: Theresa A. Laguna, MD, The Children's Hospital, 13123 East 16th Avenue, B-395, Aurora, CO 80045

Supported by grants from the Cystic Fibrosis Foundation (CFF ACCURS00A1 and CFF LAGUNA06A0) and the National Institutes of Health (1U01HL081335-01 and M01RR00069).

The authors have no potential, perceived, or real conflicts of interest to declare.

PII: S0022-3476(08)00221-7

doi:10.1016/j.jpeds.2008.03.028

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