Acute-on-chronic liver failure: A 20-year retrospective review of a tertiary paediatric liver centre.

AIM: Acute-on-chronic liver failure (ACLF) is an acute deterioration of pre-existing chronic liver disease related to a precipitating event. We characterised paediatric ACLF at Birmingham Children's Hospital (BCH) utilising European Association of Liver Disease CLIF criteria, including prevalence, triggers and outcomes. METHODS: All BCH patients from 2000 to 2020 with CLD who underwent initial liver transplant or died on the transplant waiting list or whilst too unwell to be listed were reviewed. RESULTS: From 2000 to 2020, 24 (4%) children with ACLF were identified. Death occurred in 18 (75%). Transplant occurred in 9 (36%), 3 of which died. ACLF triggers were sepsis organism negative 11 (46%), sepsis organism positive 8 (33%) and GI bleed 5 (17%). Bilirubin at the time of transplant/death in those with ACLF who lived compared with those who died was 529 umol/L (381) versus 665 (210) (p=0.38), creatinine 138 umol/L (147) versus 67 (46) (p=0.41), PT 33 sec (14) versus (32 (15) (p = 0.72), Grade 3, 4 hepatic encephalopathy 1 (17%) versus 10 (56%) (p = 0.17), vasopressor use 1 (17%) versus 17 (94%) (p = 0.001) and ventilation 3 (50%) versus 17 (94%) (p = 0.035). CONCLUSION: Acute-on-chronic liver failure whilst infrequent has high rates of mortality. The use of vasopressors and ventilation is more frequent in those who die from ACLF.

Long-Term Cognitive Outcomes and Associated Quality of Life of Young Adults Who Experienced Liver Transplantation in Early Childhood.

We evaluated long term outcomes in infants born between 1992 and 2002 with cholestatic liver disease (CLD) who underwent successful liver transplantation (LT). A total of 160 children with CLD were identified: 68 had developmental assessments before and after LT of whom 32 were excluded because they were followed up elsewhere; 16/36 consented to complete measures of IQ, anxiety, depression, health related quality of life (HRQoL), and a habits/employment survey. Illness severity and developmental attainment prior to LT were comparable with the 32 excluded and 20 patients who declined to take part. The IQ of young adults after LT (mean score = 91.13, range 75-108, SD 10.4) was not significantly improved compared to pre-LT scores (mean score = 85.7 range 50-111, SD 17), but was inversely correlated with stunting of growth and duration of disease before LT, highlighting the need for timely LT in CLD. HRQoL scores ranged from 22 to 99 (mean 64.5 SD 20.7), comparable to scores in other LT recipients. Five (31%) had mild-moderate depression; 5 (31%) had moderate-severe anxiety associated with reduced HRQoL (P = 0.01 and P = 0.06, respectively); and nine had problematic fatigue which correlated with reduced HRQoL (r 2 = 0.4 P = 0.007). Reduced medication adherence was associated with fatigue (Spearman correlation r 2 = 0.267; P = 0.09) and anxiety (Spearman correlation r 2 = 0.597; P = 0.02). Raised body mass index was also associated with reduced and health-related quality of life scores PeLTQL© (r 2 = 0.379 P = 0.011). Fifteen (94%) were undergoing education or were employed. The long-term neuro-cognitive and psychosocial outcomes of young adults transplanted as babies is encouraging, although anxiety/depression was more common than in the healthy population. Psychosocial questionnaires help identify those young adult LT recipients who may benefit from support.