PE-25: Significant Improvement in Catheter Related Infections, Hospitalization Duration and Self-care after the Implementation of a Multidisciplinary Approach in Intestinal Failure

Abstract

Introduction: Acute on Chronic Liver Failure (ACLF) is defined as an acute deterioration of pre-existing chronic liver disease related to a precipitating event with increased mortality at 3 months due to multisystem organ failure. Children with Intestinal Failure Associated Liver Disease (IFALD) are at particular risk of ACLF as they often face a long waiting time for appropriate sized liver small bowel transplant donors and are not able to be categorised as super urgent. We aimed to characterise ACLF in children with IFALD. Methods: All BCH patients from 2000-2020 with IFALD determined to be in ACLF utilising European Foundation for Study of Chronic Liver Failure (EASL CLIF) criteria were included. Descriptive statistics was presented as median (range) and CLIF C ACLF score calculated to predict 28-day mortality. Results: 6 children with IFALD were included. 3 patients had Gastroschisis, 2 Necrotising Enterocolitis (NEC) and 1 total colonic Hirschsprung's. Median age at ACLF onset was 1 year (0.8-2). ACLF triggers were sepsis in 5 and GI bleed in 1. Bilirubin at time of ACLF onset was 600 umol/L (506-870), Creatinine 30 umol/L (14-124), Prothrombin time 22 (15-35), Grade 3,4 hepatic encephalopathy (2/6), ventilation (2/6) and CLIF C ACLF score 31 (28-49). There was 100% mortality (n=6) between 2000-2012. Median time from ACLF admission to death was 29 days (6-60). Transplant listing occurred in 4, 3 pre ACLF with a median of 17 days (10-120) and 1 7 days post ACLF. 2 children were thought to be too unwell to list. Discussion: Children with IFALD are vulnerable to develop ACLF, particularly from triggering factors e.g. line sepsis. Children presenting with ACLF had end stage IFALD and median time to death was short, with not enough time for rescue transplantation. The decrease in ACLF episodes in last decade may be related to less incidence of IFALD due to advances in parenteral nutrition. Conclusion:Children with end stage IFALD can be at risk of developing ACLF especially following line sepsis and thus further prioritisation in those with IFALD at risk of ACLF should be considered.