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

Abstract

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

Insights into the management of Wilson’s disease

“…8,31 Pregnancy Medical therapy must be maintained throughout pregnancy with cases of acute liver failure being reported from discontinuation of therapy during pregnancy. 93 Satisfactory outcomes have been observed in those pregnant with Wilson's disease treated with penicillamine, trientine and zinc. There are some concerns over the teratogenicity associated with penicillamine, the risks of discontinuing treatment however outweigh those of continuing it.…”

The Liver in Pregnancy

“…Either they or their physicians may be tempted to discontinue therapy during gestation, but, as in nonpregnant patients with Wilson disease, cessation of therapy can have devastating effects and should not be attempted [194,195]. Successful pregnancies without teratogenicity have been reported in patients taking penicillamine or trientine [196][197][198].…”

Pregnancy in a Patient with Wilson’s Disease Treated with <i>D</i>-Penicillamine and Zinc Sulfate

“…Scheinberg and Sternlieb [28] and Shimono et al [30] described complications in 6 patients who reduced the dose of or discontinued D-penicillamine before or during pregnancy. They observed hepatic decompensations, threat of miscarriage and severe hemolytic anemias.…”