Comprehensive Treatment Strategy for Intestinal Failure-Associated Liver Disease

Abstract

Purpose: The aim of this study is to investigate the treatment of intestinal failure-associated liver disease (IFALD). Patients and Methods: Six patients with IFALD were hospitalized between January 2006 and February 2010. Patients were 4 males and the median age was 28 months. Underlying liver pathology was cholestasis and hepatocellular injury in 1 patient, non-alcoholic steatohepatitis (NASH) in 3 and hepatic fibrosis associated with portal hypertension in 1. Median HH15 to LHL15 ratio (H/L15) by technetium 99m-DTPA-galactosyl human serum albumin liver scintigraphy was 0.86. The treatment protocol comprised cyclic parenteral nutrition (PN), a decrease in intravenous calories, infusion of amino acids and lipid, and avoidance of fasting. Furthermore, we introduced intestinal transplantation (ITx), serial transverse enteroplasty (STEP) and Omegaven^[○!R] in 2007, 2008 and 2009, respectively. Treatment outcomes were reviewed retrospectively. Results: Four patients were alive recovering from liver damage at a median follow-up of 13 months. Only 1 patient who had undergone ITx was weaned from PN. Two patients who did not recover from liver damage died of liver failure. Patients with severe hepatic fibrosis or a high value for H/L15 were refractory to treatment. Conclusions: For patients with intestinal failure, nutritional supply should be adjusted strictly to liver condition. If a patient develops IFALD, a proper combination of treatments including careful management of parenteral nutrition, STEP and administration of Omegaven^[○!R] should be promptly initiated. By the severity of intestinal failure and IFALD, an indication for isolated ITx or liver and ITx should be considered.