High yield on aetiology using a systematic diagnostic approach to paediatric acute liver failure, analysis of a nationwide cohort

Aim Paediatric acute liver failure (P‐ALF) is a rare and devastating condition that leads to death or liver transplantation (LTx) in 40%–60% of cases. Determining the aetiology can enable disease‐specific treatment, aid in prognostication for hepatic recovery and guide the decision‐making for liver transplantation. This study aimed to retrospectively evaluate a systematic diagnostic approach to P‐ALF in Denmark and to collect epidemiological nationwide data. Methods All Danish children aged 0–16 years with P‐ALF diagnosed between 2005 and 2018, and who were evaluated using a standardised diagnostic assessment programme, were eligible for retrospective analysis of clinical data. Results A total of 102 children with P‐ALF were included (presentation at 0 days to 16.6 years of age, 57 females). Aetiological diagnosis was established in 82% of cases, the remainder were indeterminate. Fifty percent of children with P‐ALF of indeterminate aetiology died or underwent LTx within 6 months after their P‐ALF diagnosis, compared to 24% of children with an aetiological diagnosis, p = 0.04. Conclusion Following a systematic diagnostic evaluation programme, made it possible to identify the aetiology of P‐ALF in 82% of cases which is associated with improved outcomes. The diagnostic workup should never be considered complete but rather adapt to ongoing diagnostic advances.