Hypothesis: Vitamin E Complements Polyunsaturated Fatty Acids in Essential Fatty Acid Deficiency in Cystic Fibrosis

Abstract

While several studies have demonstrated essential fatty acid (EFA) deficiency in plasma and tissue lipids of cystic fibrosis (CF) patients, the reasons for this deficiency are not well established. It is believed that reduced EFA intake, malabsorption of fat, altered desaturase/lipase activity and defective cystic fibrosis transmembrane conductance regulator (CFTR) altering utilisation of EFA in epithelial cells contribute to the development of EFA deficiency in CF. It is likely that increased metabolism of arachidonic acid to eicosanoids such as leukotrienes, thromboxane and prostaglandins may also be a contributing factor. Evidence is presented that elevated oxidative damage to EFA and impaired antioxidant defences, in particular vitamin E, may contribute to the development of EFA deficiency in CF. Furthermore, antioxidant supplementation in CF may improve EFA status.

• cystic fibrosis
• vitamin E
• essential fatty acids
• antioxidants
• oxidative stress

Key teaching points:

• EFA deficiency is common in CF, with oxidative stress being one of several possible contributing factors.

• Vitamin E is the key antioxidant responsible for protecting cell membranes from oxidation.

• Vitamin E concentrations are linked to PUFA status in stable CF patients.

Key teaching points:

• EFA deficiency is common in CF, with oxidative stress being one of several possible contributing factors.

• Vitamin E is the key antioxidant responsible for protecting cell membranes from oxidation.

• Vitamin E concentrations are linked to PUFA status in stable CF patients.