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布加综合征病因的临床和实验研究初探

Preliminary study on etiology of Budd-Chiari syndrome through clinic and experiment

摘要:

目的 初步探讨布加综合征(BCS)的病因.方法 分析2001年3月至2009年5月109例BCS根治术中所见.对11例隔膜型BCS患者下腔静脉和(或)肝静脉隔膜和8例下肢深静脉血栓形成(DVT)的血栓成分进行比较.免疫组化方法研究两者组织中B型转化生长因子受体(TGFβR)、血小板衍化生长因子受体(PDGFR)、内皮素-1(ET-1)、Ⅷ因子相关抗原(FⅧ-rAg)、α1-抗胰蛋白酶、铁蛋白的表达情况.结果 本组除4例为下腔静脉肿瘤和1例为下腔静脉外纤维条索压迫所致BCS外,其余104例均为下腔静脉和(或)肝静脉隔膜或血栓形成所致.2例原为下腔静脉血栓,根治清除血栓1年和7年后复发,再次手术发现为下腔静脉新生隔膜.3例下腔静脉节段性闭塞者术中可见陈旧血栓向隔膜演变的过程.8例下腔静脉隔膜在肝静脉开口下方.隔膜和血栓组织均含纤维母细胞、中性粒细胞,均有肉芽组织及新生血管等病理改变.相关的细胞因子TGF-βR、PDGFR、ET-1、FⅧ-rAg、铁蛋白在两者组织中的表达率分别为72.3%和50.0%(P>0.05),45.5%和100%(P<0.05),100%和0(P<0.05),90.9%和12.5%(P<0.05),72.3%和100%(P>0.05).结论 BCS患者的隔膜和血栓有着相似的组织细胞成分和细胞因子表达,隔膜和血栓或为病变的不同阶段.

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abstracts:

Objective To investigate the etiology of Budd-Chiari syndrome(BCS)preliminarily.Methods The clinical findings of radical surgery of 109 cases with BCS from March 2001 to May 2009 were analyzed.The pathological components of membranous tissue(MT)from inferior vena cava(IVC)or hepatic vein(HV)of BCS patients were compared with that of thrombus from deep venous thrombosis(DVT),as well as the expression of transforming growth factor β receptor(TGF-βR),platelet derived growth factor receptor(PDGFR),endothelin(ET-1),factor Ⅷ related antigen(FⅧ-rAg),ferritin and α1-antitrypsin in MTs and thrombus through immunohistochemical method.Results One hundred and four cases of BCS were due to IVC and/or HV membrane or thrombosis except that 4 cases due to IVC tumor or 1 case due to compression of fiber trabs.The new-formed IVC membrane was found in 2 recurred cases whose IVC thrombus was excised before 1 year and 7 years.The development from organized thrombus to MT was found in 3 cases of segmental obstruction of IVC.The IVC membrane located below HV outlet was in 8 cases.Both MTs and thrombus had the pathological components such as fibroblast,neutrophil,granulation tissue,newlyformed blood vessels and so on under the light microscope.The expressions of TGF-βR,PDGFR,ET-1,FⅧ-rAg,and ferritin in MTs and thrombus were as follows:MT 72.3%,thrombus 50.0%(P>0.05);MT 45.5%,thrombus 100%(P<0.05);MT 100%,thrombus 0(P<0.05);MT 90.9%,thrombus 12.5%(P<0.05);MT 72.3%.thrombus 100%(P>0.05).Conclusions The membranous tissues and thrombus have the similar homogeneity and cytokines expression.The membrane and thrombus may be different pathological phases.

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