Assessment of liver fibrosis in children with intestinal failure and prolonged parenteral nutrition

BACKGROUND: Children with intestinal failure (IF) often necessitates prolonged parenteral nutrition (PN) use, which predisposes them to IF-associated liver disease (IFALD) and fibrosis. The aim of the study is to evaluate the prevalence and severity of liver fibrosis using transient elastography (TE) on children with IF who were on prolonged PN. METHODS: Patients with IF requiring PN for more than 3 months between Jan 2000 to December 2024 were included in the study. Liver fibrosis was assessed by TE and defined by a fibrosis score (FS) > = 6.8 kPa. Baseline demographics, diagnosis, operation details, PN characteristics, and outcomes were compared between the fibrosis and non-fibrosis groups. RESULTS: 37 children suffered from IF requiring prolonged PN were identified, and TE was performed in 19. 17 patients (89.5%) had massive bowel resection, and 2 (10.5%) had visceral myopathies. Necrotizing enterocolitis was the commonest cause of IF in this cohort (14, 73.7%). 8 (42.1%) remained dependent on PN at the time of TE. Median PN duration was 16 months (3-144 months). 4 patients (21.1%) had liver fibrosis on TE. The fibrosis and non-fibrosis groups (15, 78.9%) were similar in terms of baseline demographics, operative details and PN characteristics. There were 2 deaths in the fibrosis group, one from IF related liver failure and the other from respiratory failure with known cholestasis. (50% vs. 0%, p = 0.035). CONCLUSION: With TE measurement, the incidence of liver fibrosis in this cohort was at 21%, which appears to be higher than previously reported. They are associated with higher risk of mortality but not with known risk factors of IFALD. Further prospective studies with larger sample size will be required for proper evaluation.