柏-查综合征的病因学探讨  

A study on the etiology of BuddChiari syndrome

作　　者：秦成勇[1] 朱菊人[1] 孙成刚[1] 陆伟君[1] 叶远红[1] 

机构地区：[1]山东省立医院消化科

出　　处：《中华内科杂志》1999年第6期397-398,共2页Chinese Journal of Internal Medicine

摘　　要：目的对柏查综合征的病因进行探讨。方法对１３例柏查综合征患者行手术探查并行直视隔膜切除及下腔静脉重建术。对切除标本进行光镜、电镜及免疫组化观察。结果术中探查肝后段下腔静脉梗阻膜厚度２～２０ｍｍ。有７例合并血栓形成，有３例血栓延伸至静脉根部。光镜观察血管壁呈纤维组织增生、透明或黏液变性，９例有慢性炎性细胞浸润。电镜见大量胶原纤维和少量血管内皮细胞、线粒体肿胀。免疫组化未发现有ＩｇＧ、ＩｇＡ、ＩｇＭ免疫复合物沉积。结论本研究提示局部炎症可能是血栓形成原因之一，膜性梗阻可能是由血栓机化而来，不支持先天性膜形成学说。Objective To investigate the etiology of BuddChiari syndrome (BCS) with finding in surgical operation. Methods Thirteen patients with BCS undertook direct vision membranectomy and reconstruction of inferior vena cava. The specimens were studied with light microscopy,electron microscope and immunohistochemisty. Results Membranous occluding changes,which thickness varying from 2 to 20mm,were found in the posthepatic segment of inferior vena cava during the operation.Thrombi in inferior vena cava were found in 7 cases,3 of them with thrombi evenly extending to the root of hepatic vein .The compromized venous wall showed proliferation of fibrous connective tissue, hyalinization or mucoid degeneration under light microscopy,9 of the 13 cases showed chronic infiltration of inflammatory cells.Copious collagenous fibers, few endothelial cells and mitochondril swelling were found with electron microscope.No immune complex (IgA, IgG, IgM) was observed by immunohistochemistry. Conclusion It is concluded that in these cases of BCS,occluding and stenosing lesions in the inferior vena cava were the outcome of thrombosis and its sequelae and local inflammation might be one of the reasons of thrombosis. There was no evidence of congenital malformation.

关 键 词：肝静脉血栓形成 炎症 病因 

分 类 号：R575.02[医药卫生—消化系统]