P3.45: Intestinal Failure-Associated Liver Disease (IFALD) in the SMOF Era: What Has Changed?

Introduction: IFALD is the most severe complication of intestinal failure (IF). Since 2009, we have used a composite lipid emulsion containing fish oil, medium chain triglycerides, olive oil and soybean oil (SMOF lipid) in our cohort of IF children. The aim of this study was to assess the hepatic profile of children with IF receiving this lipid emulsion. Methods: This study was a retrospective review of the children followed in our center for intestinal failure from 2009 to 2019 who received at least two years of parenteral nutrition (PN) and SMOF lipid and who were still on home PN on January 2019. The data was collected from charts review, using the latest clinical, biological and radiological data available. Results: One hundred and nine children were included. Of these, 60 (55%) were born after 2009 and received SMOF lipid exclusively. Most children (66%) were followed for short bowel syndrome (whom 17 had extensive Hirschsprung’s disease), 25 (23%) for congenital enteropathy, and 13 (12%) for chronic intestinal pseudo-obstruction. They received PN a mean of 5.4 days/week with SMOF lipid 1.5 g/kg/day. Sixty-six percent of patients had abnormal liver tests, 53% elevated ALT, 48% elevated GGT, 22% elevated bilirubin with 9 of them having elevated conjugated bilirubin; 29 patients had low PT and 20 had low platelets rate. Ultrasound showed splenomegaly in 35 patients (32%). Only 8 patients (7%) had signs of cirrhosis or portal hypertension. Forty-five children underwent liver biopsy, fibrosis was seen in 33, 5 had F4 or cirrhosis, 16 had steatosis. Thirteen patients had both fibrosis and steatosis. In the meantime (2009–2019), 43 other children were followed in our HPN center for more than two years but left the program: 19 were weaned from PN, 5 died from non-digestive complications (sepsis, cancer), 15 were transferred to adult centers, 4 underwent liver- small bowel transplantation. Only one child – born in 2011 - showed a rapid progression to cholestasis and severe portal hypertension and received liver-intestine transplantation for life threatening IFALD. Conclusion: After ten years of using SMOF, children with IF on long term parenteral nutrition rarely evolve to severe and life threatening IFALD. However, most patients still present with abnormal biologic profiles and steatosis/fibrosis on biopsies. Identifying predictive factors for IFALD should help in the prevention of IFALD and the management of these patients.