Abstract
BACKGROUND: Intestinal failure associated liver disease (IFALD) is a common complication of intestinal failure with limited treatment options. While Omegaven® can be used as a salvage therapy for IFALD in patients receiving intralipid®, its efficacy in IFALD associated with SMOFlipid® remains unclear. This study aims to assess Omegaven® as a salvage therapy in this setting. METHODS: Retrospective study of children with intestinal failure who developed advanced IFALD (conjugated bilirubin over 50 µmol/L for at least 2 weeks) while receiving SMOFlipid® and converted to Omegaven® (1.0 g/kg/d). Patients were treated with Omegaven® for at least 7 weeks. The primary outcome was conjugated bilirubin (CB) < 34 μmol/l after Omegaven® Initiation(.) RESULTS: Out of 219 patients receiving SMOFlipid® between 2013 and 2024, 13 developed advanced IFALD and were converted to Omegaven®. Two patients did not meet inclusion criteria. All 11 remaining patients had a high parenteral nutrition dependency index (PNDI) at baseline. Following conversion to Omegaven®, 7 (63.6 %) patients achieved CB bellow 34 µmol/L, in a mean time of 12.5 weeks (SD 6.63). Non-responders had a higher incidence of gastrointestinal bleeding secondary to portal hypertension (75 % vs 0 %, p = 0.02), INR (1.8 vs 1.1, p = 0.03), and need for continuous glucose infusion (75 % vs 0 %, p = 0.02). Out of the 4 non-responders, one patient died of liver failure, 2 underwent multivisceral transplantation, and one remains listed for liver-intestine transplantation. CONCLUSIONS: Conversion to Omegaven® from SMOFlipid® reversed advanced IFALD in 63.6 % of patients. Response is less likely in children with synthetic liver dysfunction and clinically significant portal hypertension.