Antioxidant deficiency in cystic fibrosis: when is the right time to take action?

Little is known about age- and disease-related changes in prooxidant and antioxidant systems in patients with cystic fibrosis (CF). We investigated changes in antioxidant concentrations and oxidative stress in plasma, buccal mucosal cells (BMCs), and breath condensate in patients with CF in relation to age and disease progression. We recruited 22 patients with CF as well as 35 healthy control subjects and conducted a cross-sectional study by dividing the participants into 4 age groups (<6 y, 6-11 y, 12-17 y, > or =18 y). We collected fasting blood samples, BMCs, and breath condensate. Carotenoids, alpha-tocopherol, vitamin C, protein carbonyls, thiobarbituric acid-reactive substances, and F(2)alpha-isoprostane were assessed. In patients with CF, plasma vitamin C concentrations, plasma and BMC alpha-tocopherol concentrations, and forced expiratory volume in 1 s (percentage predicted) decreased significantly with age. Plasma beta-carotene, beta-cryptoxanthin, and total lycopene were significantly lower in patients than in control subjects in all age groups. Furthermore, alpha-tocopherol and vitamin C plasma concentrations as well as alpha-tocopherol concentrations in BMCs were significantly lower in CF patients > or =18 y old, whereas all indicators of oxidative stress assessed were significantly higher than those same indicators in control subjects. Adult patients with CF in particular showed distinct vitamin deficits and elevated indicators of oxidative stress in plasma, BMCs, and breath condensate along with a progression of clinical status. We suggest that early in life dietary habits should be improved and that innovative supplementation strategies should be applied to optimize the antioxidant status of patients with CF.