布加综合征与肝癌

布加综合征(Budd-Chiari syndrome,BCS)是由肝静脉流出道阻塞所引起的一组异质性的症候群,阻塞部位可发生于从肝小静脉(hepatic vein,HV)到肝后段下腔静脉(inferior vena cava,IVC)至右心房入口处的任何部位,同时排除由心脏疾病引起的肝静脉流出道阻塞及肝窦阻塞综合征[1].BCS多见于中国、日本、印度、尼泊尔、南非等;在我国,则以黄淮流域为主,如河南、河北、江苏、山东、安徽等地[2-3].     

布-加综合征自愈：个例报道

1病例资料患儿女,4岁,宁夏人。一年前因＂过敏性紫癜＂在当地医院治疗,住院期间出现下肢水肿、蛋白尿,诊断为＂紫癜性肾炎＂,给予口服强的松治疗,症状缓解。一个月前无诱因突发腹胀、上腹疼痛、发热。患儿全腹膨隆,腹壁静脉曲张,主要分布于脐水平以上,血流自下至上,腹围67 cm。腹软无压痛,     

经腹翻肝入路非体外循环直视下布-加综合征根治术——单纯开腹手术根治是否可行？

1临床资料1.1病例男,45岁,主因＂腹胀半年,双下肢水肿伴色素沉着5年＂以布-加综合征入院。查体腹部明显膨隆,胸腹壁、背部可见静脉曲张,血流向上,双下肢指凹性水肿,双下肢静脉曲张,膝以下色素沉着。彩超肝左、肝中静脉近心段闭塞,肝右静脉未显示;     

New approaches to the Budd-Chiari syndrome

Abstract  Recent research has led to an improved understanding of the aetiology of Budd-Chiari syndrome in some patients. Fresh approaches and technical developments within methods of radiological intervention have added more effective options to its treatment. In this editorial we aim to summarize our understanding of the role of new aetiologies and new therapeutic approaches in the Budd-Chiari syndrome.     

布-加综合征的进展与展望

本文叙述了布-加综合征的演变,自1845年和1899年由Budd和Chiari报道的不同肝静脉阻塞引起的门脉高压,在病因和流行病学方面开始研究。我国、印度、南非等国的病变大多涉及下腔静脉,今后以改名为肝腔静脉阻塞综合征更为恰当。在病变分类的基础上,出现了一系列治疗方法。在急性期,纤溶疗法是指征。慢性病例采用的方法：各种腔内治疗法、间接减压术、断流术、各种促进侧枝循环的手术、直接减压术,包括各型肠系膜上静脉或下腔静脉或水母头与右心房或颈内静脉之间的转流术、根治性矫治术和肝移植术,后者指征要严格。今后至少要把下腔静脉隔膜性的病因查清,要做到预防和早期诊断,要对复杂性病变的介入和手术方法加以探讨。     

Budd-Chiari syndrome management: Lights and shadows

Budd-Chiari syndrome (BCS) is a rare disease whose management should follow a step by step strategy.Anticoagulation and medical therapy should be the first line treatment.Revascularization or TIPS are indicated in case of no response to medical therapy.OLT should be indicated as a rescue therapy and anticoagulation be started soon after OLT.However,no clear indica-tion can actually be given about the timing of different treatments.Moreover,there is some concern about treatment of some subgroup of patients,especially regarding the risk of recurrence after liver transplanta-tion.The topic of this paper is to critically review the actual knowledge of BCS management.     

Budd-Chiari like syndrome in decompensated alcoholic steatohepatitis and liver cirrhosis

INTRODUCTIONBudd-Chiari Syndrome(BCS)is a rare,heterogeneous and potentially lethal condition caused by obstruction of the hepatic venous outflow tract[1],situated anywhere between the small hepatic venules until the right atrium[2].In Western countries,t…     

Platelet ultra-structure study in Budd-Chiari syndrome

Abstract: Morphologic characteristics of circulating platelets were studied in 20 patients with primary Budd-Chiari syndrome (BCS) without any known etiology, using transmission electron microscopy (TEM). Significant platelet ultrastructural changes were observed in all the patients (in 10←90% platelets) as compared to 20 normal healthy controls (in up to 4% of their platelets). The prominent changes in the platelets were paucity or absence of alpha granules, hypertrophy of the open canalicular systems (OCS) and clumping and fusion of the granules and other organelles in the centre of platelets. Some other changes observed in platelets were dilated channels of OCS, pseudopodial protrusion of cytoplasm and presence of prominent masses of glycogen particles. Platelets from 20 normal controls processed along with the patients' platelets showed only a few such abnormalities. Most of these changes observed in patients' platelets were akin to the changes observed in platelets undergoing activation. Assay of plasma beta-thromboglobulin showed significantly higher levels in all the patients (p< 0.001) further confirming on-going in vivo platelet activation with morphologic changes most likely reflecting the thrombotic process present in BCS patients.     

再论布-加综合征介入治疗的应用

目的进一步分析布-加综合征（BCS）介入治疗的选择及其疗效。方法对65例患者行球囊扩张或/和支架植入术。术后对患者进行随访,最长随访时间已达10年。结果全部患者恢复顺利,术后1周下肢肿胀明显减轻,肝脾不同程度缩小,胸腹壁静脉曲张明显好转。随访证实患者术后远期疗效均良好。结论介入治疗是治疗BCS的首选,应用过程中应重视适应证选择、手术操作及并发症的预防。     

Budd-Chiari syndrome： Etiology, pathogenesis and diagnosis

Budd-Chiari syndrome is a congestive hepatopathy caused by blockage of hepatic veins. This syndrome occurs in 1/100 000 in the general population. Hypercoagulable state could be identified in 75% of the patients; more than one etiologic factor may play a role in 25% of the patients. Primary myeloproliferative diseases are the leading cause of the disease. Two of the hepatic veins must be blocked for clinically evident disease. Liver congestion and hypoxic damage of hepatocytes eventually result in predominantly centrilobular fibrosis. Doppler ultrasonography of the liver should be the initial diagnostic procedure. Hepatic venography is the reference procedure if required. Additionally liver biopsy may be helpful for differential diagnosis. The prognosis of the chronic form is acceptable compared to other chronic liver diseases.     

Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome

The antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced to a variety of phospholipids determinants of cell membranes or phospholipid binding proteins. There are few reports about association between antiphospholipid antibodies and development of Budd-Chiari syndrome (BCS). We report the case of BCS development in young Russian male with primary APS. The patient underwent orthotopic liver transplantation on August 26, 2012. At present time his state is good, the blood flow in the liver restored and its function is not impaired. We report about the first time the successful use of dabigatran etexilate for prolonged anticoagulation therapy in APS patient with BCS. In addition patient is managed with immunosuppressive drugs.     

3种类型布-加综合征的临床特点分析

[目的]探讨3种类型布-加综合征的临床特征.[方法]回顾性分析3种类型共57例布-加综合征患者的临床资料,对其临床表现、实验室生化检查结果及影像学结果等进行比较.[结果]57例中男38例,女19例,发病率男女之比为2∶1.3种类型中以下腔静脉型最多26例(45.6％)、肝静脉型14例(24.6％)、混合型17例(29....     

原发性Budd-Chiari综合征的介入治疗

目的分析经皮球囊扩张治疗原发性Budd-Chiari综合征下腔静脉闭塞病变的疗效。方法采用Seldinger穿刺血管方法,行下腔静脉及右心房造影,确定下腔静脉闭塞段,用Brockenbrough穿刺针开通闭塞段,球囊导管扩张病变治疗下腔静脉闭塞病变42例。结果成功率为100％,病变区血管直径扩至12～21mm,平均(18±3．1)mm,下腔静脉压自(17±3)mmHg,降至(7．5±1．8)mmHg,疗效显著(P＜0．01),症状体征明显减轻或消失。结论临床观察表明,Brockenbrough穿刺针开通病变血管是安全的,球囊扩张对绝大部分原发性Budd-Chiari综合征下腔静脉闭塞病变的疗效满意。     

Budd-Chiari syndrome due to pacemaker-induced thrombosis

Abstract We report a unique case of Budd-Chiari syndrome caused by pacemaker leads-induced thrombosis. A 34 year old female patient was subjected to a permanent pacemaker insertion because of refractory paroxysmal supraventricular tachycardia attacks related to Wolff-Parkinson-White syndrome. Three years later, another pacemaker was re-implanted because of its dislodgement. Four episodes of skin infections at the implantation site were noted thereafter. The patient developed symptoms of abdominal pain and ascites 5 years after the second pacemaker implantation. Ultrasonography and computerized tomography of the abdomen revealed hepatomegaly with ascites and dilated inferior vena cava. An echocardiogram displayed thrombus formation in the superior vena cava, the right atrium and the inlet of the inferior vena cava into the right atrium. Inferior and superior venacavogram confirmed the above findings. With the impression that Budd-Chiari syndrome was caused by pacemaker-induced thrombus, we removed the pacemaker first and thoracotomy with thrombectomy was then performed. The clinical symptoms resolved after the operation. To our knowledge, this is the first case reported in the literature and this observation supported the thrombosis theory for membranous obstruction of inferior vena cava.     

介入治疗布-加综合征883例体会

目的总结介入治疗布-加综合征（BCS）的临床经验及存在的问题。方法对883例BCS患者行下腔静脉（IVC）造影,确定病变部位、类型,再用硬导丝或Rups100穿刺针穿通阻塞部位、球囊扩张,对短段狭窄或闭塞、扩张后球囊有切迹的隔膜型和长段狭窄型常规放置腔静脉支架。结果本组883例,扩张成功803例,其中IVC747例,肝静脉（HV）56例,放置IVC支架504例,HV支架16例。本组46例需行附加降低门脉高压手术。术后发生急性肾衰8例,肝昏迷2例,急性心衰43例。并发IVC急性血栓形成5例,死亡2例。673例获随访7～124个月,复发31例。结论介入治疗BCS具有安全、有效、恢复快等优点,逐渐成为临床首选。     

肠-颈转流术在布-加综合征中的应用（附2例报道）

1病例资料例1男性患者,26岁,2010年1月因工作期间不慎被机器轧伤,后因全身多发伤入院治疗。既往无肝炎病史。入院诊断：①开放性颅脑损伤;左侧额叶硬膜外血肿;左侧额骨骨折,颅内积气;面部多发骨折;②右侧第2～7肋骨骨折;③肝脏挫伤、包膜下积血;④右侧肩峰骨折;⑤全身多发软组织及皮肤撕脱伤。     

Necessity and indications of invasive treatment for Budd-Chiari syndrome

BACKGROUND:The development of collaterals in Budd-Chiari syndrome has been described and these collaterals play an important role in the presentation of this disease.These collaterals are diagnostic and their use in management strategy has never been evaluated.This study aimed to investigate the indications,feasibility and necessity of invasive treatment for patients with Budd-Chiari syndrome and to determine whether such a strategy is necessary for optimal management.METHODS:Twenty-nine patients who had be...     

Budd-Chiari syndrome and portal vein thrombosis due to essential thrombocytosis

Budd-Chiari syndrome secondary to essential thrombocytosis has been described in a few reports in the English literature. Associated portal vein thrombosis occurs very rarely. Herein, we report a case presented with ascites and finally diagnosed with hepatic and portal vein thrombosis, and essential thrombocytosis. We discussed the therapeutic approaches in the light of pertinent literature.     

肠-颈转流术治疗重症布-加综合征术后18年通畅1例

1病例资料患者女,41岁,手术室护士。因＂发现腹水7年,加重伴呼吸短促1个月＂于1992年2月入院。查体：腹部膨隆,腹围87 cm,胸腹壁可见纵行静脉曲张,肝大,剑下5 cm,双下肢轻度浮肿。B超提示下腔静脉长段阻塞,肝脾肿大,肝静脉扩张,大量腹水,右胸腔积液。血生化示总胆红素68.4μmol/L,直接胆红素47.88μmol/L,HBsAg（-）,