Objectives and Study Acute on Chronic Liver Failure (ACLF) is defined as an acute deterioration of pre-existing chronic liver disease usually related to a precipitating event and associated with increased mortality at 3 months due to multisystem organ failure. Within paediatrics, ACLF is infrequently described, with most existing cohorts utilising Asian Pacific Association for Study of Liver (APASL) definitions. We aimed to characterise paediatric ACLF at Birmingham Children’s Hospital (BCH) utilising European Foundation for Study of Chronic Liver Failure (EASL CLIF) criteria: including prevalence, triggers, outcomes and listing patterns.

Methods All BCH patients from 2000–2020 with chronic liver disease (CLD) who underwent initial liver transplant or died either on the transplant waiting list or whilst too unwell to be listed were reviewed. Those with IFALD were included, whilst all other significant extrahepatic disease was excluded. Descriptive statistics was presented as median (range) and CLIF C ACLF score calculated to predict 28-day mortality. Fishers exact test was used for categorical data and unpaired t test (mean, standard deviation) for continuous data (Graph Pad).

Results From 2000–2020 24 (4.2%) children with ACLF were identified out of 576 children meeting review criteria. Age at ACLF onset was 1.75 years (0.25–17) with 9 (38%) males. Death occurred in 18 (75%) children. Aetiology of liver disease, transplant status and CLIF C ACLF scores are outlined in table 1. All deaths in those with IFALD occurred within the years 2000–2012. ACLF triggers were sepsis organism negative 11(46%), sepsis organism positive 8(33%), GI bleed 4(17%) and liver biopsy bleed 1(4%). All with sepsis organism positive died.

Bilirubin at time of transplant/death in those with ACLF who lived compared to those who died was 529 umol/L(381) vs. 665(210) (p=0.27), Creatinine 138 umol/L(147) vs. 63(44) (p=0.06), Prothrombin time 33(13) vs. 32(14) (p=0.95), use of vasopressor 1(17%) vs. 17(94%) (p=0.008), Grade 3,4 hepatic encephalopathy 1(17%) vs. 9 (50%) (p=0.34) and ventilation 3(50%) vs.17(94%) (p=0.04) respectively.

Of those who were listed pre ACLF, 2 (33%) lived compared with 9 (50%) who died. Median time (days) from ACLF onset to listing was 7 (1–13) in those who lived compared with 15 (1–24) in those who died. Median time (days) from ACLF onset to transplant in those that lived compared with time from ACLF onset to death was 19 (2–32) vs. 17.5 (5–60).

Conclusion ACLF whilst infrequent in our cohort when utilising EASL criteria has high rates of mortality with sepsis being a frequent trigger. Those with IFALD are vulnerable, particularly from line sepsis, although death from ACLF in this cohort has become less common in the last decade. Use of vasopressors and ventilation is more frequent in those who die from ACLF despite similar median times to both successful transplant and death from ACLF onset. Larger studies are needed to determine protective factors in children with ACLF who are transplanted and live.