P. Fickert et al., ACUTE BUDD-CHIARI SYNDROME WITH FULMINANT HEPATIC-FAILURE IN A PREGNANT WOMAN WITH FACTOR-V LEIDEN MUTATION, Gastroenterology, 111(6), 1996, pp. 1670-1673
Budd-Chiari syndrome during pregnancy has rarely been reported. This r
eport presents a case of acute hepatic failure in a 20-year-old pregna
nt woman attributable to Budd-Chiari syndrome with underlying resistan
ce to activated protein C caused by factor V Leiden mutation, The pati
ent delivered a healthy girl by cesarean section in the 31st week of p
regnancy, Acute hepatic failure in the 6th week postpartum was success
fully treated by emergency liver transplantation, and the patient and
her child were doing well at 8-month follow-up, Liver transplantation
was lifesaving; normal factor V production by the transplant corrected
the underlying coagulopathy. In this patient, latent thrombophilia at
tributable to activated protein C resistance was apparently aggravated
by the hypercoagulable state of pregnancy leading to acute Budd-Chiar
i syndrome, Activated protein C resistance should be sought as an etio
logic factor in patients with Budd-Chiari syndrome.