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

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