Summary

  • Rare disease associated with jaundice, coagulopathy, and hepatic encephalopathy.
  • The etiology and the interval from onset of jaundice to the development of encephalopathy have a significant impact on prognosis.
  • Etiology 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 etiology, 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.

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