Summary

• Rare disease associated with jaundice, coagulopathy, and hepatic encephalopathy.
• The aetiology and the interval from onset of jaundice to the development of encephalopathy have a significant impact on prognosis.
• Aetiology is established by history, serological assays, and exclusion of alternative causes, including acute presentations of chronic liver diseases.
• Treatment involves ICU monitoring, specific therapies based on aetiology, and management of known complications.
• All patients should be considered for possible liver transplantation.
• Prognostic models may be used to assess the probability of spontaneous recovery and are instrumental in selection of patients who should potentially undergo liver transplantation.