If I'd known I was going to live this long, I'd have taken better care of myself: Antioxidants and micronutrients in CF

摘要

There have been major advances in the treatment of cystic fibrosis (CF) such that median survival is now above 45 years, and over half the patients are over the age of 18 years in Canada. Increased survival means that care must be given to optimize nutrition for both maximal growth and the prevention of long term complications. CF lung disease is the primary determinant of survival, with nutritional status being an additional independent predictor of survival. The lung disease in CF is characterized by a dysregulated neutrophilic inflammation and persistence of bacterial infection, e.g., with Pseudomonas aeruginosa. This leads to progressive lung damage and, eventually, respiratory failure. The neutrophilic infiltration is accompanied by an overabundance of oxidants. Further, the respiratory epithelium, expressing dysfunctional CFTR, is pro-oxidant and pro-inflammatory. This results in an oxidant-antioxidant imbalance, i.e., oxidative stress. CF is further complicated by intestinal malabsorption, resulting in impaired protein and fat absorption [1]. This not only limits the absorption of macronutrients (protein and lipids), but also results in defective absorption of the fat soluble vitamins (A, D, E, K) and other micronutrients, such as zinc. There is also an essential fatty acid imbalance that is associated with defective CFTR function. This imbalance affects cell function and signaling, further contributing to a pro-inflammatory environment.