Raszeja-Wyszomirska Joanna, Mieżyńska-Kurtycz Joanna, Marlicz Wojciech, Lawniczak Małgorzata, Wójcicki Maciej
Liver Unit, Pomeranian Medical University, Szczecin, Poland.
Hepatogastroenterology. 2012 Sep;59(118):1879-82. doi: 10.5754/hge11064.
BACKGROUND/AIMS: Budd-Chiari syndrome (BCS) is recognized as a clinical manifestation of various prothrombotic conditions which may be lethal within 3 years of the onset of symptoms if untreated. This study is a retrospective analysis of patients with BCS managed between 2004 and 2011.
The diagnosis was confirmed with contrast CT-angiography and/or Doppler ultrasound.
BCS was diagnosed in 20 patients (11 females and 9 males), median age 38 years (ranging from 18 to 56). Twelve patients were referred as acute BCS for the liver transplant (LTx) assessment. Thrombosis of the hepatic veins was caused by myeloproliferative disorders (n=8), end-stage liver disease (n=4), protein C deficiency (n=3), paroxysmal nocturnal hemoglobinuria (PNH) (n=1), antiphospholipid syndrome (n=1) and secondary poliglobulia (n=1). In two patients the origin of BCS could not be established despite appropriate screening. Median follow-up was 29 months. Low molecular heparin with subsequent conversion to vitamin K antagonists was routinely applied in all patients. Two patients underwent TIPS procedure with good long term outcome and 10 subjects received LTx; 1 patient was lost to follow-up and 1 died of chest infection 9 years since the diagnosis of BCS was made; 14 patients, including those who received LTx, were alive and well at least one year after BCS diagnosis. All survivors remain stable and are followed-up on a regular basis.
Strict adherence to the diagnostic and therapeutic guidelines plays a crucial role in the management of BCS patients. Our results confirm the efficacy of anticoagulation as well as TIPS and/or OLT in treatment of this rare condition.
背景/目的：布加综合征（BCS）是多种血栓前状态的临床表现，若不治疗，症状出现后3年内可能致死。本研究是对2004年至2011年间诊治的BCS患者的回顾性分析。
通过增强CT血管造影和/或多普勒超声确诊。
确诊BCS患者20例（女性11例，男性9例），中位年龄38岁（18至56岁）。12例患者因急性BCS被转诊进行肝移植（LTx）评估。肝静脉血栓形成由骨髓增殖性疾病（n = 8）、终末期肝病（n = 4）、蛋白C缺乏（n = 3）、阵发性夜间血红蛋白尿（PNH）（n = 1）、抗磷脂综合征（n = 1）和继发性红细胞增多症（n = 1）引起。2例患者尽管进行了适当筛查仍无法确定BCS的病因。中位随访时间为29个月。所有患者均常规应用低分子肝素，随后转换为维生素K拮抗剂。2例患者接受了经颈静脉肝内门体分流术（TIPS），长期效果良好，10例患者接受了肝移植；1例患者失访，1例患者在BCS诊断9年后死于肺部感染；14例患者，包括接受肝移植的患者，在BCS诊断后至少一年存活且情况良好。所有幸存者病情稳定并定期接受随访。
严格遵循诊断和治疗指南对BCS患者的管理至关重要。我们的结果证实了抗凝以及TIPS和/或原位肝移植（OLT）治疗这种罕见疾病的有效性。
