Abstract

Liver biopsy is no viable tool to routinely screen for liver fibrosis in children suffering from chronic intestinal failure (IF). We aim to assess the prevalence of liver fibrosis in a cohort of children with chronic IF by non-invasive tests: transient elastography (TE), aspartate-aminotransferase-to-platelet-ratio-index (APRI) and enhanced liver fibrosis (ELF) score. Cross sectional study where patients with chronic IF, receiving parenteral nutrition (PN) for at least 3 months, were enrolled. TE, APRI and ELF score were measured. Using Spearman's rank correlation coefficient and Kruskal-Wallis H test, the correlation between TE, APRI, ELF score and known risk factors for development of intestinal failure-associated liver disease (IFALD) were calculated. 32 patients were included (50% female), median age was 8 years and 4 months, median PN duration was 45 months. Six patients (21%) had TE≥6.5kPa, indicating significant fibrosis. Twelve patients (38%) had APRI ≥.5, indicating fibrosis. ELF score indicated moderate fibrosis in 17 patients (63%) and significant fibrosis in 10 patients (37%). TE and APRI correlated significantly with known risk factors for IFALD, but ELF showed poor correlation with known risk factors for IFALD. In a cohort of pediatric patients suffering from chronic IF, TE measurement, APRI and ELF test show a varying, but substantial proportion of subjects with fibrosis. The diagnostic value of these tests and their role in the management of pediatric IF must be determined in larger cohorts with liver biopsy as reference standard. Academic Medical Center medical ethics committee number: METC 2017_185.

Highlights

  • A value of .5 was used to determine if liver fibrosis was present. This value was chosen since Voutilainen et al reported an optimal cut-off value of .5 to detect significant liver fibrosis (METAVIR!F2) in a cohort of 99 pediatric patients with various liver diseases of whom 31 patients suffered from intestinal failure (IF) [35]

  • This study assessed the prevalence of liver fibrosis in pediatric patients dependent on home parenteral nutrition (PN) using non-invasive fibrosis markers

  • This is the first time transient elastography (TE), APRI and enhanced liver fibrosis (ELF) score have been described in a large pediatric home PN cohort

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Summary

Introduction

The reference standard for the assessment of fibrosis in any liver disease, including IFALD, is histology. Fibrosis status in most liver disorders is commonly graded using the METAVIR scoring system. Liver biopsy is no viable tool to routinely screen for liver fibrosis in children suffering from chronic intestinal failure (IF). We aim to assess the prevalence of liver fibrosis in a cohort of children with chronic IF by non-invasive tests: transient elastography (TE), aspartate-aminotransferase-toplatelet-ratio-index (APRI) and enhanced liver fibrosis (ELF) score. Using Spearman's rank correlation coefficient and KruskaleWallis H test, the correlation between TE, APRI, ELF score and known risk factors for development of intestinal failure-associated liver disease (IFALD) were calculated. Conclusion: In a cohort of pediatric patients suffering from chronic IF, TE measurement, APRI and ELF test show a varying, but substantial proportion of subjects with fibrosis.

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