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Abstract
Intestinal failure associated liver disease (IFALD) can cause liver fibrosis without jaundice in the modern era.1 Liver biopsy may be misleading in assessing the severity owing to patchy hepatic involvement, and does not help in defining the presence or absence of portal hypertension.2
The aim was to evaluate common non-invasive tests for liver fibrosis among a cohort of patients on home parenteral nutrition (HPN) in a tertiary paediatric gastroenterology unit.
We conducted a prospective study between March 22 and Nov 22. Data on abdominal ultrasound, fibroscan and biochemical markers (Enhanced Liver Fibrosis ELF test, AST-to-platelet ratio index APRI, fibrosis-4 score Fib-4)were collected.
15/31 patients on HPN were included. While 16 patients were excluded due to either age or behavioural issues. Median age was10.8 years (range 3y10m-17y), 73% male. The median duration of HPN was 47 months (range 12–189), 67% of patients had short bowel syndrome. Results for fibroscan, APRI, FIB-4 and ELF test are shown in table 1. Abdominal ultrasound showed splenomegaly in five patients. No correlation was identified between Fibroscan score, APRI score, FIB4, ELF test or splenomegaly (r = 0.09,0.1,-0.3,0.12 respectively). 13.3% (2/15) children had splenomegaly and F score of > 7.7 suggestive of progressive fibrosis. Of these, one patient is undergoing intensive rehabilitation to stop HPN and the second patient’s monitoring has been intensified.
Non-invasive modalities for assessment of liver fibrosis maybe helpful in identifying fibrosis at earlier stages in children with IFALD. This would allow different treatment options to be considered in a timely manner before progression to end stage IFALD needing intestinal transplantation.
Results of Fibroscan, APRI, Fib-4 and ELF test
References
Lacaille F, Gupte G, Colomb V, D’Antiga L, Hartman C, Hojsak I, Kolacek S, Puntis J, Shamir R; ESPGHAN Working Group of Intestinal Failure and Intestinal Transplantation. Intestinal failure-associated liver disease: a position paper of the ESPGHAN Working Group of Intestinal Failure and Intestinal Transplantation. J Pediatr Gastroenterol Nutr 2015 Feb;60(2):272–83.
Valamparampil, Joseph, Rachel M. Brown, Charlotte Passingham, Jane Hartley, and Girish L. Gupte. L7 Non-invasive markers for predicting hepatic fibrosis in pediatric intestinal failure-associated liver disease (IFALD). BMJ Journal (2022):A50-A51.