Seki S, Sakaguchi H, Kobayashi S, Kitada T, Nakamura K, Yamada R
Third Department of Internal Medicine, Osaka City University Medical School, 1-4-3 Asahimachi, Abenoku, 545-8585 Osaka, Japan.
Hepatogastroenterology. 2001 Sep-Oct;48(41):1447-9.
The antiphospholipid antibody syndrome is characterized by arterial and venous thrombosis including hepatic veins. Although transjugular intrahepatic portosystemic shunt or liver transplantation have been considered for Budd-Chiari syndrome, treatment options for patients with complete obstruction of three hepatic veins including the junction with the inferior vena cava are limited. We describe a 27-year-old female, who suffered thrombotic obliteration of hepatic veins including the portion confluent with the inferior vena cava (Budd-Chiari syndrome) associated with marked ascites and liver dysfunction. Transjugular intrahepatic portosystemic shunt using a Wall-stent (10 mm in diameter) between inferior vena cava and intrahepatic portal vein was performed. Intrastent coagulation and recurrence of thrombosis were prevented by combination therapy with warfarin potassium and ticlopidine hydrochloride. These treatments induced loss of ascites and improvement of liver function, and she has been able to resume daily life. The portosystemic shunt described above in addition to combination therapy with warfarin potassium and ticlopidine hydrochloride appeared to be one of the options for treating Budd-Chiari syndrome associated with antiphospholipid antibody syndrome.
抗磷脂抗体综合征的特征是动脉和静脉血栓形成，包括肝静脉血栓。尽管经颈静脉肝内门体分流术或肝移植已被用于布加综合征的治疗，但对于包括与下腔静脉连接处在内的三条肝静脉完全阻塞的患者，治疗选择有限。我们描述了一名27岁女性，她患有肝静脉血栓闭塞，包括与下腔静脉汇合部分（布加综合征），伴有明显腹水和肝功能障碍。在肝静脉和肝内门静脉之间使用一枚直径10mm的Wall支架进行了经颈静脉肝内门体分流术。通过华法林钾和盐酸噻氯匹定联合治疗预防了支架内凝血和血栓复发。这些治疗使腹水消失，肝功能改善，她已能够恢复日常生活。上述门体分流术以及华法林钾和盐酸噻氯匹定联合治疗似乎是治疗抗磷脂抗体综合征相关布加综合征的选择之一。
