Budd-Chiari综合征、肝小静脉闭塞病与肝硬化的鉴别

Budd-Chiafi综合征（BCS）是由肝静脉或其开口以上的下腔静脉阻塞性病变引起的以下腔静脉阻塞、门静脉高压为特点的综合征，诊断依靠肝静脉血管造影，治疗重点是解除梗阻。肝小静脉闭塞病是肝小叶中央静脉和小叶下静脉损伤导致管腔狭窄或闭塞而产生的门静脉高压症，临床表现类似于BCS，诊断依靠肝组织活检。这两种疾病的治疗和预后均与肝实质性病变导致的肝硬化不同．临床上需要注意对这三种疾病的鉴别。     

布-加综合征的临床观察及护理

布-加综合征是由肝静脉和／或膈段下腔静脉阻塞导致门静脉高压和／或下腔静脉高压为表现的一种综合征，目前临床相对少见。我科近2年运用球囊扩张及血管内支架置入术成功治疗18例，临床效果显著。     

布—加综合征的诊断与治疗概述

布 -加综合征 (简称 B- CS)是由肝静脉阻塞和 (或 )其开口以上段下腔静脉阻塞性病变引起的 ,以伴有下腔静脉高压为特点的一种肝后性门脉高压症。 1845年和 1899年 ,英国内科医师 George Budd和奥地利病理医师 Hans Chiari分别叙述了由不同部位肝静脉阻塞引起的门脉高压症 ,由肝静脉阻塞引起的门静脉高压症被称为 B- CS。190 6年 ,Yamagiwa报道 6例肝静脉阻塞患者 ,其中 3例伴有肝后下腔静脉阻塞性病变。笔者综合分析了 Pleasants(1911)报道的 18例、并复习文献报道的 314例下腔静脉阻塞 ,发现分别有 6例 (33.3% )和 6 2例 (2 1.3% )属…     

布-加综合征模型的构建及进展

布-加综合征是由肝静脉或其开口以上的下腔静脉阻塞引起的门静脉高压伴或不伴有下腔静脉高压的临床综合征,中国人群主要以下腔静脉隔膜形成和(或)阶段性合并肝静脉梗阻为特征。目前人们对布-加综合征的病因和发病机制所知甚少,所做研究多为诊治策略方面的临床研究,针对其发病机制的实验探索相对稀缺,而稳定模型往往是疾病机制研究的必要条件。就目前布-加综合征模型的构建方法、各种模型的特点和研究进展作一综述和分析,以便为该病病因和发病机制的探索提供可靠的实验基础。     

布-加综合征的诊断与治疗

布-加综合征(简称B-CS)是由肝静脉阻塞和(或)其开口以上段下腔静脉阻塞性病变引起的,以伴有下腔静脉高压为特点的一种肝后性门脉高压症.1845年和1899年,英国内科医师George Budd和奥地利病理医师Hans Chiari分别叙述了由不同部位肝静脉阻塞引起的门脉高压症,由肝静脉阻塞引起的门静脉高压症被称为B-CS.1906年,Yamagiwa报道6例肝静脉阻塞患者,其中3例伴有肝后下腔静脉阻塞性病变.     

布-加综合征疑难病例的介入治疗

介入治疗已成为布一加综合征首选治疗方法。回顾了布-加综合征的影像学诊断以及6种疑难类型,即肝静脉阻塞、下腔静脉长段闭塞、下腔静脉闭塞合并血栓形成、下腔静脉闭塞近心端盲端缺如或很短、广泛肝静脉闭塞和下腔静脉支架放置后阻塞肝静脉的诊治经验。介绍了此6种类型的介入治疗技术要点和注意事项。分析表明,只要充分评估病情,选择合理的治疗方案,将有助于提高疑难布一加综合征病例的介入治疗成功率,进一步提高我国布一加综合征的整体诊疗水平。     

布─加综合征经皮球囊成形术治疗（摘要）

布─加综合征经皮球囊成形术治疗（摘要）大连医学院附属第一医院心脏科方唯一，于文信，旅朝霞，李新明，部德秀布－加综合征是指肝静脉流出道－肝静脉或肝段下腔静脉阻塞引起的门静脉或下腔静脉高压所产生的综合征。在下腔静脉和右心房之间膜样堵塞物形成按Ｈｉｒｏｏｋ...     

布-加综合征64例临床分析

布-加综合征（Budd—Chiari syndrome，BCS）是由肝静脉（hepatic veins，HV）或下腔静脉（inferior vena cava，IVC）阻塞性病变引起的以IVC、肝后性门静脉高压为特点的临床综合征。BCS的定义可分为广义和狭义两种，狭义的BCS是指单纯HV狭窄，主要表现为门静脉高压；而各种原因导致的HV和（或）HV开口及以上水平各种类型的IVC阻塞称为广义BCS。由于以往对该病的认识不足，其临床表现复杂多变而无特异性，常误诊为其他原因导致的肝硬化。随着临床治疗手段的进步，特别是介入治疗的引入，使本病早期接受治疗患者的预后得到了改善，所以对BCS临床特点的熟悉及早期诊断成为影响本病预后的重要因素。     

超声在肝窦阻塞综合征中的诊断价值

目的探讨肝窦阻塞综合征超声声像图特征及评估超声在肝窦阻塞综合征中的诊断价值。方法回顾性分析8例由我院临床诊断肝窦阻塞综合征(HSOS)的患者超声声像图,观察肝脏大小形态及内部回声特点、肝静脉、门静脉、肠系膜上静脉及下腔静脉内径及血流情况、脾脏大小、胆囊壁厚度及腹腔积液量。结果 8例患者的超声表现为肝脏体积增大,回声减低,分布不均匀。8例肝静脉内径变细,4例流速减低。6例出现门静脉增宽,5例血流速度减低。2例出现门静脉及肠系膜上静脉血栓。2例患者出现脾脏体积增大。8例患者伴有腹腔积液。8例出现胆囊壁水肿增厚。4例患者下腔静脉肝后段变细。结论彩色多普勒超声对肝窦阻塞综合征有很重要的诊断及鉴别诊断价值。     

吡咯烷生物碱导致的肝窦阻塞综合征的MRI特点

[目的]探讨吡咯烷生物碱导致的肝窦阻塞综合征的MRI特点。[方法]回顾性分析2010-09——2017-12期间共43例吡咯烷生物碱导致的肝窦阻塞综合征患者的基本信息、临床资料及MRI资料;MRI资料由2位经验丰富的影像科医师采用独立盲法阅片获得。[结果]吡咯烷生物碱导致的肝窦阻塞综合征患者的MRI特点为:平扫期腹水43例,肝肿大28例,胆囊壁水肿39例,门静脉区水肿41例,胸腔积液24例,脾大10例;门静脉期43例患者均可见肝脏不均一低信号病变,其中32例表现为沿肝静脉分布的"爪形"强化;平衡期27例肝右静脉狭窄,16例肝右静脉显影不清,42例下腔静脉狭窄。[结论]吡咯烷生物碱导致的肝窦阻塞综合征具有特征性的MRI表现,因此MRI可以为吡咯烷生物碱导致的肝窦阻塞综合征提供一种有效的诊断方法。     

Tusanqi and hepatic sinusoidal obstruction syndrome

Hepatic sinusoidal obstruction syndrome (HSOS), characterized by hepatomegaly, ascites and hyperbilirubinemia, is caused by toxic injury to hepatic sinusoidal endothelial cells. One major etiology of HSOS in China is the intake of products containing pyrrolizidine alkaloids (PA) such as Tusanqi. The manifestations of patients with HSOS are usually non‐specific, presenting with abnormal liver function and portal hypertension. Diagnosis of the disease depends mostly on liver histopathology when clinical and imaging data are not sufficient. A history of Tusanqi intake is mostly important for the diagnosis. Due to a lack of effective, evidence‐based treatments for HSOS, avoiding the mistaken use of PA‐containing products including Tusanqi is important for the prevention of HSOS.     

Japanese case of Budd-Chiari syndrome due to hepatic vein thrombosis successfully treated with liver transplantation

A 22‐year‐old Japanese woman was found to have severe esophageal varices and then suffered from hepatic encephalopathy. She was diagnosed with Budd‐Chiari syndrome (BCS) due to hepatic vein (HV) thrombosis accompanied by portal vein thrombosis without inferior vena cava (IVC) obstruction. Latent myeloproliferative neoplasm (MPN) lacking the JAK2‐V617F mutation was considered to be the underlying disease. Liver transplantation was strikingly effective for treating the clinical symptoms attributable to portal hypertension. Although thrombosis of the internal jugular vein occurred due to thrombocythemia, which manifested after transplantation despite anticoagulation therapy with warfarin, the thrombus immediately disappeared with the addition of aspirin. Neither thrombosis nor BCS has recurred in more than 4 years since the amelioration of the last thrombotic event, and post‐transplant immunosuppression with tacrolimus has not accelerated the progression of MPN. In Japan, IVC obstruction, which was a predominant type of BCS, is suggested to have decreased in incidence with recent improvements in hygiene. The precise diagnosis of BCS and causative underlying diseases should be made with attention to the current trend of the disease spectrum, which fluctuates with environmental sanitation levels. Because the stepwise strategy, including liver transplantation, has been proven effective for patients with pure HV obstruction in Western countries, this strategy should also be validated for utilization in Japan and in developing countries where HV obstruction potentially predominates.     

Management of hepatic vascular diseases

Primary damage to hepatic vessels is rare. (i) Hepatic arterial disorders, related mostly to iatrogenic injury and occasionally to systemic diseases, lead to ischemic cholangiopathy. (ii) Hepatic vein or inferior vena cava thrombosis, causing primary Budd-Chiari syndrome, is related typically to a combination of underlying prothrombotic conditions, particularly myeloproliferative neoplasms, factor V Leiden, and oral contraceptive use. The outcome of Budd-Chiari syndrome has markedly improved with anticoagulation therapy and, when needed, angioplasty, stenting, TIPS, or liver transplantation. (iii) Extrahepatic portal vein thrombosis is related to local causes (advanced cirrhosis, surgery, malignant or inflammatory conditions), or general prothrombotic conditions (mostly myeloproliferative neoplasms or factor II gene mutation), often in combination. Anticoagulation at the early stage prevents thrombus extension and, in 40% of the cases, allows for recanalization. At the late stage, gastrointestinal bleeding related to portal hypertension can be prevented in the same way as in cirrhosis. (iv) Sinusoidal obstruction syndrome (or venoocclusive disease), caused by agents toxic to bone marrow progenitors and to sinusoidal endothelial cells, induces portal hypertension and liver dysfunction. Decreasing the intensity of myeloablative regimens reduces the incidence of sinusoidal toxicity. (v) Obstruction of intrahepatic portal veins (obliterative portal venopathy) can be associated with autoimmune diseases, prothrombotic conditions, or HIV infection. The disease can eventually be complicated with end-stage liver disease. Extrahepatic portal vein obstruction is common. Anticoagulation should be considered. (vi) Nodular regenerative hyperplasia is induced by the uneven perfusion due to obstructed sinusoids, or portal or hepatic venules. It causes pure portal hypertension.     

布加综合征与妊娠

布加综合征是一种少见的肝脏疾病,其与妊娠之间相互影响。系统论述了布加综合征与妊娠的关系,并指出了在疾病诊断和治疗中的注意事项。其一,妊娠是布加综合征的危险因素。对于妊娠期间出现门静脉高压和(或)下腔静脉高压表现的患者,应高度怀疑合并布加综合征。其二,布加综合征患者妊娠具有一定的风险。经治疗病情稳定的患者可以妊娠,但孕期和产后须严密监查,以防止血栓复发。其三,布加综合征可导致女性不孕。原因不明的不孕患者应行腹部超声及CT检查,以排除布加综合征的可能。     

肝窦阻塞综合征八例临床分析

目的探讨肝窦阻塞综合征（SOS）的临床诊治方法。方法回顾性分析8例SOS的临床资料,并进行随访。结果8例SOS主要临床表现为腹胀（8例）、肝区疼痛（7例）、腹水征（8例）及肝肿大（7例）等。8例的肝功能损害程度大多较轻,血清-腹水白蛋白梯度均大于11．1g／L,血清与腹水CA125均显著升高。超声检查均见肝脏肿大、胆囊壁水肿或增厚、门静脉增宽且流速缓慢、肝静脉变细以及腹腔积液等;MRI表现为门脉期及延迟期肝实质不均匀片状强化,肝叶、段静脉腔内造影剂充盈不良。经皮肝穿刺活检均见肝窦扩张、淤血及肝细胞变性、坏死,3例发现小静脉管腔狭窄、管壁增厚伴纤维组织增生。8例中1例行肝移植术后痊愈,4例经内科治疗后逐渐康复,3例死亡。结论SOS的临床表现以突出的门脉高压症为特点,CA125常显著升高,超声及MRI对本病的诊断与鉴别诊断有重大价值,而经皮肝穿刺活检的价值有限,联合应用影像学方法与病理活检可提高诊断正确率。早期应用抗凝药物是治疗本病的关键,严重病例可行肝移植术。     

Liver cirrhosis in hepatic vena cava syndrome (or membranous obstruction of inferior vena cava)

Hepatic vena cava syndrome (HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction (HVOO) that is complicated by high incidence of liver cirrhosis (LC) and moderate to high incidence of hepatocellular carcinoma (HCC). In the past the disease was considered congenital and was included under Budd-Chiari syndrome (BCS). HVCS is a chronic disease common in developing countries, the onset of which is related to poor hygienic living condition. The initial lesion in the disease is a bacterial infection induced localized thrombophlebitis in hepatic portion of inferior vena cava at the site where hepatic veins open which on resolution transforms into stenosis, membrane or thick obstruction, and is followed by development of cavo-caval collateral anastomosis. The disease is characterized by long asymptomatic period and recurrent acute exacerbations (AE) precipitated by clinical or subclinical bacterial infection. AE is managed with prolonged oral antibiotic. Development of LC and HCC in HVCS is related to the severity and frequency of AEs and not to the duration of the disease or the type or severity of the caval obstruction. HVOO that develops during severe acute stage or AE is a pre-cirrhotic condition. Primary BCS on the other hand is a rare disease related to prothrombotic disorders reported mainly among Caucasians that clinically manifest as acute, subacute disease or as fulminant hepatic failure; and is managed with life-long anticoagulation, porto-systemic shunt/endovascular angioplasty and stent or liver transplantation. As epidemiology, etiology and natural history of HVCS are different from classical BCS, it is here, recognized as a separate disease entity, a third primary cause of HVOO after sinusoidal obstruction syndrome and BCS. Understanding of the natural history has made early diagnosis of HVCS possible. This paper describes epidemiology, natural history and diagnosis of HVCS and discusses the pathogenesis of LC in the disease and mentions distinctive clinical features of HVCS related LC.     

Enfermedades vasculares del hígado. Guías Clínicas de la Sociedad Catalana de Digestología y de la Asociación Española para el Estudio del Hígado

Despite their relatively low prevalence, vascular diseases of the liver represent a significant health problem in the field of liver disease. A common characteristic shared by many such diseases is their propensity to cause portal hypertension together with increased morbidity and mortality. These diseases are often diagnosed in young patients and their delayed diagnosis and/or inappropriate treatment can greatly reduce life expectancy.This article reviews the current body of evidence concerning Budd-Chiari syndrome, non-cirrhotic portal vein thrombosis, idiopathic portal hypertension, sinusoidal obstruction syndrome, hepatic vascular malformations in hereditary haemorrhagic telangiectasia, cirrhotic portal vein thrombosis and other rarer vascular diseases including arterioportal fistulas. It also includes a section on the diagnostic imaging of vascular diseases of the liver and their treatment from a haematological standpoint (study of thrombotic diathesis and anticoagulation therapy). All recommendations are based on published studies extracted from PubMed. The quality of evidence and strength of recommendations were rated in accordance with the GRADE system (Grading of Recommendations, Assessment Development and Evaluation). In the absence of sufficient evidence, recommendations were based on the opinion of the committee that produced the guide.     

经颈静脉肝内门体分流术及肝静脉成形术治疗肝静脉型Budd-Chiari综合征的临床意义

目的分析经颈静脉肝内门体分流术(TIPS)及经皮肝或经颈静脉途径肝静脉成形术等介入手段治疗肝静脉型Budd-Chiari综合征(BCS)的临床意义。方法回顾性分析2000年5月至2012年10月收治的32例肝静脉型BCS的临床资料。其中男15例,女17例;平均年龄(38±6)岁;肝功能Child-Pugh评分(9.6±2.2)分;肝静脉近段闭塞8例,肝静脉闭塞合并下腔静脉狭窄4例,全肝静脉闭塞11例,肝小静脉闭塞9例;急性3例,亚急性或慢性29例。患者主要临床表现为顽固性腹水和食管静脉曲张破裂出血,采用彩色多普勒超声、CT血管造影或MRI血管造影、上消化道钡餐及内镜检查明确诊断。治疗方法包括:TIPS 9例,改良TIPS 11例,单独经皮肝或经颈静脉途径肝静脉成形术8例,经皮肝肝静脉成形术联合下腔静脉成形术4例。结果所有患者均成功完成介入或手术治疗,其中TIPS或改良TIPS术后,患者出血控制,腹水逐渐消退,肝功能明显好转;门静脉压力由(42±8)cm H2O(1 cm H2O=0.098 kPa)下降至(27±5)cm H2O(t=20.20,P=0.001),门静脉血流速度由(18±6)cm/s增加至(52±10)cm/s(t=15.02,P=0.001)。住院期间因肝功能不全死亡1例,肝门分流道急性阻塞1例。术后随访3~241个月,平均随访(102±26)个月,分流道狭窄性扩张内支撑2例,肝静脉狭窄再扩张2例。结论经皮肝或经颈静脉途径肝静脉成形术与TIPS均是治疗肝静脉型BCS的有效方法,可根据患者病情酌情选择治疗方法。     

Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review

Budd-Chiari syndrome(BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt(TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.