Knoop M, Lang M, Neumann U, Bechstein W O, Neuhaus P
Chirurgische Klinik und Poliklinik, Virchow Klinikum, Humboldt Universität zu Berlin.
Chirurg. 1998 Dec;69(12):1362-8. doi: 10.1007/s001040050585.
Budd-Chiari syndrome (BCS) with hepatic vein occlusion is a rare disorder that can effectively be treated in advanced stages with orthotopic liver transplantation. We report on 16 patients who received 18 liver grafts and were followed up for at least 2 years. In 7 patients a hematological disorder was confirmed by bone marrow biopsy. One patient died after 4 months due to cytomegalovirus pneumonia; another patient died after 2 years due to progressive liver failure after portal vein thrombosis. The actuarial 5-year survival rate is 87.5% compared to 85.3% in all other 710 orthotopic liver transplantations performed from September 1988 to December 1995 at our institution. Anticoagulation consisted of intravenous heparin and overlapping continuation with dicoumarin. Three patients received hydroxyurea for thrombocytosis, one patient for 1 week only early after the transplantation. Two postoperative abdominal hemorrhages required laparotomy. Two patients had to be retransplanted, one for thrombosis of the hepatic artery and portal vein after discontinuation of dicoumarin due to GI bleeding and one for hepatic vein thrombosis after insufficient dicoumarin intake. Terminal BCS represents a good indication for orthotopic liver transplantation; however, life-long, closely monitored anticoagulation is essential.
布加综合征（BCS）伴肝静脉闭塞是一种罕见疾病，在晚期可通过原位肝移植得到有效治疗。我们报告了16例接受18次肝移植且随访至少2年的患者。7例患者经骨髓活检确诊患有血液系统疾病。1例患者在4个月后因巨细胞病毒肺炎死亡；另1例患者在2年后因门静脉血栓形成后进行性肝衰竭死亡。与1988年9月至1995年12月在我们机构进行的其他710例原位肝移植的85.3%相比，其5年实际生存率为87.5%。抗凝治疗包括静脉注射肝素并与双香豆素重叠持续使用。3例患者因血小板增多症接受羟基脲治疗，1例仅在移植后早期接受了1周治疗。术后发生2次腹腔出血，需要进行剖腹手术。2例患者不得不再次移植，1例因胃肠道出血停用双香豆素后发生肝动脉和门静脉血栓形成，另1例因双香豆素摄入不足发生肝静脉血栓形成。终末期布加综合征是原位肝移植的良好适应证；然而，终身密切监测的抗凝治疗至关重要。
