Transplant-free Survival in Chronic Liver Disease Presenting as Acute Liver Failure in Childhood

Background in adults, the absence of a preexisting chronic liver disease (CLD) is required to diagnose acute liver failure (ALF). The paediatric classification does not considered this aspect, thus previous studies pooled together children with ALF and children with unknown CLD presenting with acute hepatic decompensation (ALF-CLD). We aimed to compare prevalence, features and outcome of children with ALF-CLD to those with a proper ALF. Methods Patients admitted between 1996-2017 because of ALF defined by PALF criteria (raised transaminases, INR ≥2.0, no history of liver disease) were classified as ALF-CLD if diagnosed with autoimmune hepatitis (AIH), Wilson disease (WD), Budd-Chiari syndrome, HBV reactivation, inborn errors of metabolism (IEM). The others were classified as ALF. Results 74 children [median age 4 years, 1.0-8.8, M/F =36/38] with ALF were found ; 18 of

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