Fickert P, Ramschak H, Kenner L, Hoefler G, Hinterleitner T A, Petritsch W, Klimpfinger M, Krejs G J, Stauber R E
Department of Medicine, Kari Franzens University, Graz, Austria.
Gastroenterology. 1996 Dec;111(6):1670-3. doi: 10.1016/s0016-5085(96)70031-8.
Budd-Chiari syndrome during pregnancy has rarely been reported. This report presents a case of acute hepatic failure in a 20-year-old pregnant woman attributable to Budd-Chiari syndrome with underlying resistance to activated protein C caused by factor V Leiden mutation. The patient delivered a healthy girl by cesarean section in the 31st week of pregnancy. Acute hepatic failure in the 6th week postpartum was successfully 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 attributable to activated protein C resistance was apparently aggravated by the hypercoagulable state of pregnancy leading to acute Budd-Chiari syndrome. Activated protein C resistance should be sought as an etiologic factor in patients with Budd-Chiari syndrome.
妊娠期布加综合征鲜有报道。本报告介绍了一名20岁孕妇因布加综合征导致急性肝衰竭的病例，该综合征潜在病因是由因子V莱顿突变引起的对活化蛋白C抵抗。患者在妊娠31周时剖宫产下一名健康女婴。产后第6周的急性肝衰竭通过紧急肝移植成功治疗，患者及其孩子在8个月随访时情况良好。肝移植挽救了生命；移植肝脏产生的正常因子V纠正了潜在的凝血病。在该患者中，妊娠的高凝状态显然加重了由活化蛋白C抵抗引起的潜在血栓形成倾向，导致急性布加综合征。对于布加综合征患者，应寻找活化蛋白C抵抗作为病因。
