Intestinal failure-associated liver disease (IFALD) is characterized by either liver steatosis or cholestasis and may develop in patients on long-term home parenteral nutrition for chronic intestinal failure. The pathogenesis of IFALD is multifactorial and includes gastrointestinal disease-related, parenteral nutrition-related, and systemic-related factors. Alteration of bile acid enterohepatic circulation, gut microbiome, and intestinal permeability, seem to be the main mechanisms. Patients forced to a total oral fasting regimen are at greater risk. Parenteral nutrition overfeeding and/or of soybean-based lipid emulsion may be contributing factors. Prevention and treatment are based on avoiding and promptly treating all the risk factors.
Keywords: Cholestasis; Intestinal failure; Lipid emulsion; Liver disease; Parenteral nutrition; Short bowel syndrome; Steatosis.
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