Decreased Total Serum Coenzyme-Q<sub>10sub> Concentrations: A Longitudinal Study in Children with Cystic Fibrosis
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文摘

ss=""h4"">Objective

To assess total serum levels of coenzyme Q<sub>10sub> (Co-Q<sub>10sub>), an important antioxidant, in children with cystic fibrosis (CF) and to investigate an association between Co-Q<sub>10sub> level and clinical outcome.

ss=""h4"">Study design

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.

ss=""h4"">Results

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.

ss=""h4"">Conclusions

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.

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