Fulminant hepatic failure during perinatal period in a pregnant woman with Wilson's disease

摘要: Wilson’s disease associated with hepatic failure is not common and the underlying mechanism triggering event known at present. We treated a 28-year-old Japanese woman who developed hemolytic crisis just after delivery. She was diagnosed as having 12 years of age, which time she started taking D-penicillamine. had previously delivered two children without difficulty. When found out pregnant this time, stopped D-penicillamine in contrast to it faithfully during her first pregnancies. On day delivery full-term baby, jaundice accompanied severe crisis. Plasma exchanges blood transfusion were performed administration again. gradually recovered apparently following good clinical course. However, on 30 second occurred subsequent liver led death 50. At autopsy cirrhotic showed massive necrosis. Prophylactic oral careful observation are therefore recommended prevent perinatal period.