Purpose of review: This review evaluates our current knowledge on the association of fatty acid abnormalities in cystic fibrosis with the disease process, and makes a case for a well-designed clinical trial to evaluate the clinical efficacy of long chain n-3 fatty acids.
Recent findings: It has long been known that cystic fibrosis patients exhibit fatty acid abnormalities, but these have not been well investigated in tissues affected by the disease. Recent studies have demonstrated that such tissues do indeed show abnormalities in the proportions of linoleic, arachidonic and docosahexaenoic acids, and have demonstrated alterations in fatty acid and phospholipid metabolism in cystic fibrosis. Work in other areas has identified novel anti-inflammatory actions of long chain n-3 fatty acids that might be relevant to cystic fibrosis. A recent systematic review of fish oil supplementation in cystic fibrosis did not allow firm conclusions to be drawn, but suggested that there may be some benefits.
Summary: The restoration of abnormal fatty acid profiles may be beneficial in cystic fibrosis, but this approach has not been evaluated in studies with a suitable design to permit firm conclusions to be drawn. There is a need for a long-term randomized controlled study to evaluate the therapeutic benefit of fish oil supplementation in cystic fibrosis.