Ludwig J, Hashimoto E, McGill D B, van Heerden J A
Division of Pathology, Mayo Clinic, Rochester, MN 55905.
Mayo Clin Proc. 1990 Jan;65(1):51-5. doi: 10.1016/s0025-6196(12)62109-0.
Severe hepatic venous outflow obstruction and its manifestations often are recorded under the label "Budd-Chiari syndrome." Unfortunately, this label is ambiguous; it does not clearly identify the site of the lesion (hepatic veins versus inferior vena cava), its morphologic features (thrombotic versus nonthrombotic), or its cause. In the literature, implied or expressed definitions vary. Use of a standardized topographic and pathogenetic classification of hepatic venous outflow obstruction would enable investigators to group patients with comparable conditions, as required for therapeutic trials, prognostic evaluations, and studies of pathogenetic pathways. Review of our own cases revealed that hepatic venous outflow obstruction involving large hepatic veins is usually thrombotic and that isolated obstruction of the inferior vena cava or of small hepatic veins is usually nonthrombotic. Application of such a classification seems feasible and may yield useful results.
严重的肝静脉流出道梗阻及其表现通常被记录在“布加综合征”这一名称之下。不幸的是，这个名称具有歧义性；它并未明确病变部位（肝静脉与下腔静脉）、形态学特征（血栓形成性与非血栓形成性）或病因。在文献中，隐含的或明确表达的定义各不相同。采用标准化的肝静脉流出道梗阻地形学和病因学分类，将使研究人员能够按照治疗试验、预后评估以及致病途径研究的要求，对病情相当的患者进行分组。回顾我们自己的病例发现，累及大肝静脉的肝静脉流出道梗阻通常是血栓形成性的，而下腔静脉或小肝静脉的孤立性梗阻通常是非血栓形成性的。应用这样一种分类似乎是可行的，并且可能会产生有用的结果。
