The development of intestinal failure-associated liver disease (IFALD) in pediatric and adult patients on parenteral nutrition is usually multifactorial in nature due to nutritional and non-nutritional causes. The role of lipid therapy as a contributing cause is well-established with the pathophysiological pathways now better understood. The review focuses on risk factors for IFALD development, biological effects of lipids, lipid emulsions and the mechanisms of lipid toxicity observed in laboratory animals followed by a synopsis of clinical studies in pediatric and adult patients. The introduction of fish oil-based lipid emulsions that provide partial or complete lipid replacement therapy has resulted in resolution of IFALD that had been associated with soybean oil-based therapy. Based on case reports and cohort studies in pediatric and adult patients who were at risk or developed overt liver disease, we now have more evidence that an early switch to partial or complete fish oil-based lipid therapy should be implemented in order to successfully halt and reverse IFALD.
Keywords: essential fatty acid deficiency (EFAD); fish oil lipid emulsions (FO IVLE); intestinal failure-associated liver disease (IFALD); intravenous lipid emulsions (IVLE); medium-chain triglycerides (MCT); mixed fish oil-containing lipid emulsions (MFO IVLE); parental nutrition-associated liver disease (PNALD); parenteral nutrition (PN); parenteral nutrition-associated cholestasis (PNAC); polyunsaturated fatty acid (PUFA); soybean lipid emulsions (SB IVLE).