Pediatric Acute-on-Chronic Liver Failure.

Acute-on-chronic liver failure (ACLF) is characterized by an acute hepatic insult happening in a patient with underlying cirrhosis with compromised hepatic reserve leading to development of systemic inflammation, sepsis, and organ failure resulting in poor outcome in majority. While Asia Pacific Association for Study of Liver Diseases (APASL) emphasizes on early diagnosis before development of organ failure, European Association for Study of Liver Diseases (EASL) mandates the presence of organ failures to define ACLF. There is a lack of consensus definition of pediatric ACLF although recent APASL guidelines have tried to address the issue. While Wilson disease (WD) and autoimmune hepatitis (AIH) are the most common cause of underlying cirrhosis in children, acute viral hepatitis and flares of WD and AIH are the commonest acute precipitating events. Poor outcomes [death and liver transplantation (LT)] ranging from 19 to 59% have been reported. Prognosis in pediatric ACLF is usually better than that in adults due to greater proportion of treatable etiologies, lesser organ failures, comorbidities and better hepatic reserves. APASL ACLF Research Consortium (AARC) score more than or equal to 11 is predictive of poor 28-90 d mortality. Treatment of pediatric ACLF relies mainly on prompt diagnosis and medical management of a potentially treatable etiology of underlying cirrhosis. Bridging therapies, especially high volume plasma exchange can be initiated early as a bridge to LT or native liver recovery. Those with no improvement in 4-7 d should undergo LT before development of sepsis or multi-organ failure.

How to identify the need for liver transplantation in pediatric acute-on-chronic liver failure?

OBJECTIVES: The objectives of the study were to evaluate the prognostic value of APASL ACLF Research Consortium-Acute-on-chronic liver failure (AARC-ACLF) score against the current prognostic models in pediatric ACLF and to assess the role of pediatric modifications of AARC-ACLF score and chronic liver failure-sequential organ failure assessment (CLIF-SOFA) score. METHODS: All children between 1 and 18 years of age satisfying the APASL definition of ACLF were included in the study. All the prognostic scores were calculated retrospectively from hospital records. Outcome was assessed at days 28 and 90. Pediatric modifications of AARC-ACLF and CLIF-SOFA scores were evaluated. RESULTS: Acute-on-chronic liver failure was seen in 86 (13.4%) of 640 children with chronic liver disease. Twenty-five (29.8%) children died, 7 (8.3%) underwent liver transplant and the remaining 52 (61.9%) survived with their native liver. Four prognostic models (AARC-ACLF, AARC-ACLF-Pediatric, CLIF-SOFA and CLIF-SOFA-Pediatric) had an AUROC greater than 0.9 for predicting poor outcome in pediatric ACLF. AARC-ACLF and CLIF-SOFA models were superior to other prognostic scores with a cutoff score of 11 or more predicting poor outcome. Pediatric modifications of AARC-ACLF and CLIF-SOFA scores were not superior to their original scores. Children with poor outcome had rising scores at day 4, whereas the scores were falling in those with good outcome. CONCLUSION: AARC-ACLF and CLIF-SOFA models are superior to other prognostic scores in pediatric ACLF. The scores are dynamic and a patient with either of these scores ≥ 11 at admission and/or a rising score at day 4 has high likelihood of death and needs to be urgently listed for liver transplantation.