Co-Q<sub>10sub> levels were measured annually in a prospective cohort study of 381 children with CF. A total of 1092 serum levels of total Co-Q<sub>10sub> were obtained by high-performance liquid chromatography and ultraviolet light detection. Associations of Co-Q<sub>10sub> with demographic variables and clinical outcomes were investigated.
Of the 381 initial total serum Co-Q<sub>10sub> measurements, 188 were in the deficient range. Low Co-Q<sub>10sub> 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<sub>10sub> levels. Low Co-Q<sub>10sub> 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<sub>10sub> levels were related to other markers of nutritional status, including total lipids, beta-carotene, and alpha-tocopherol.
Persistently low total serum Co-Q<sub>10sub> levels are common in children with CF and PI. A prospective study is indicated to determine whether Co-Q<sub>10sub> supplementation in CF is beneficial.