Journal
ACTA PAEDIATRICA
Volume 112, Issue 5, Pages 1082-1090Publisher
WILEY
DOI: 10.1111/apa.16746
Keywords
acute liver failure; children; diagnostic workup; outcome; prognosis
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This study retrospectively evaluated a systematic diagnostic approach to paediatric acute liver failure (P-ALF) in Denmark and collected nationwide epidemiological data. The results showed that identifying the aetiology in 82% of cases led to improved outcomes for P-ALF. Therefore, establishing the cause is of great significance for treatment and prognosis.
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.
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