Abstract

Background: Direct toxic effects on hepatocytes and bile duct occlusion by protoporphyrin aggregates result in liver damage in Eryhthropoetic Protoporphyria(EPP).Fulminanat hepatic failure in EPP has been described but no definite trigger has been found. Effects of liver resection in EPP are also not known.We describe the first case of liver resection in a patient of EPP which led to acute fatal porphyria hepatopathy of previously normal liver.

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