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Background and Aims: Autoimmune hepatitis (AIH) in children presenting in acute liver failure (ALF) can be fatal and often requires liver transplantation (LTx). This individual patient data meta‐analysis (IPD) aims to examine management and outcomes of this population, given the lack of large cohort studies on paediatric AIH first presenting as ALF (AIH‐ALF). Methods: A systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Review and Meta‐Analyses of IPD statement using PubMed and Excerpta Medica dataBASE, and included English studies published between 2000 and 2020. The study included patients under 21 years of age, diagnosed with type 1 or 2 AIH and presenting with ALF. Data extracted included clinical and biochemical characteristics, interventions, and outcomes. Results: Three hundred and thirty eligible patients from 61 studies were identified, with an additional five patients from our institution. The majority were female (66.8%), with a median age of 10. Overall, 59.7% achieved native liver survival (NLS), 35% underwent LTx, and 5% died before LTx. The use of corticosteroids with non‐steroid immunomodulators increased the likelihood of NLS by 2.5‐fold compared to corticosteroids alone. AIH‐1 was associated with 3.3‐fold odds for NLS, compared to AIH‐2. However, on multivariate analysis, only AIH‐1 was identified as an independent predictor for NLS (OR 3.8 [95% CI 1.03–14.2], p =.04). Conclusion: While corticosteroids and non‐steroid immunomodulators treatment may offer enhanced probability of achieving NLS, treatment regimens for AIH‐ALF may need to consider patient‐specific factors, especially AIH type. This highlights the potential for NLS in AIH‐ALF and suggest a need to identify biomarkers which predict the need for combination immunosuppression to avoid LTx. [ABSTRACT FROM AUTHOR]

Background and Aims: Wilson disease (WD) has diverse presentations that frequently mimic other liver diseases. Distinguishing WD from non‐alcoholic fatty liver disease (NAFLD) and autoimmune hepatitis (AIH), can be difficult and has critical implications for medical management. This study aimed to examine the utility of histological features of WD in children compared to those with NAFLD and AIH. Methods: A review of liver biopsy slides was performed in children with a clinical and/or genetic diagnosis of WD, seen at the Hospital for Sick Children between 1981 and 2019 and compared to controls with NAFLD and AIH. 37 children with WD and 37 disease controls (20 NAFLD; 17 AIH) were included. Three pathologists, blind to clinical details and diagnosis, reviewed all liver biopsies to reach consensus. Clinical and histopathologic features were compared between groups. Results: Most WD cases displayed steatosis or steatohepatitis on histology (34/37), active AIH‐pattern in 1 and inactive cirrhosis in 2 cases. Electron microscopy (EM) findings of mitochondrial abnormalities including dilated tips of cristae, pleomorphism, membrane duplication and dense matrix were more frequent in the WD group as compared to disease controls (p < 0.0001). In WD, dilated tips of mitochondrial cristae had a sensitivity of 91% and specificity of 86%, best among EM features. Conclusions: Light microscopic findings display considerable overlap among children with WD, NAFLD and AIH. Ultrastructural findings of mitochondrial abnormalities are important to distinguish WD from NAFLD and AIH. EM examination should be considered essential in the diagnostic work‐up of paediatric liver biopsies. [ABSTRACT FROM AUTHOR]