布-加综合征相关问题的探讨

Discussion of issues related to Budd-Chiari syndrome

布-加综合征(B-CS)是一种由肝静脉流出道梗阻引起的少见疾病,其病因复杂,目前尚无定论。当前研究认为血管发育异常、肠道微生物及微量元素失调等可能与B-CS的发病有关,高通量测序技术的发展可能会有助于明确B-CS的发病机制。B-CS的症状不具有特异性,主要依靠影像学方法确立诊断,因此亟需寻找新的无创性生物诊断标志物。B-CS的病理分型较多且标准不一,多不能充分反映B-CS患者的病理生理变化并指导临床治疗,因此建议依据B-CS的血流动力学变化和侧支循环代偿进行病理生理学分型,并据此分型方案制定与传统的早诊断、早治疗所不同的分期管理的个性化治疗策略,本文就以上内容进行总结和讨论。

Budd-Chiari syndrome(B-CS)is a rare disease caused by hepatic vein outflow obstruction,and its etiology is complex and inconclusive.Current studies suggest that vascular dysplasia,gut microbiota and trace element imbalance may be related to the pathogenesis of B-CS,and the development of high-throughput sequencing technology may help to clarify the exact pathogenesis of B-CS.The symptoms of B-CS are not specific and rely mainly on imaging methods to establish the diagnosis,so there is an urgent need to find new noninvasive biological diagnostic markers.In addition,there are many pathological types and different criteria of B-CS,which mostly can′t fully reflect the pathophysiological changes of B-CS patients and guide clinical treatment.Therefore,we recommend pathophysiological classification according to the hemodynamic changes and collateral circulation compensation of B-CS,and then develop personalized treatment strategies for stratified management different from the traditional early diagnosis and treatment protocols.This article summarizes and discusses the above contents.