10例Budd-Chiari综合征的临床分析

Ｂｕｄｄ－Ｃｈｉａｒｉ综合征是由于肝静脉流出道阻塞所引起的肝窦扩张,淤血伴有肝肿大,大量腹水,下肢浮肿的临床综合征［１］。我院４年来先后收治了 １０例,本文作一临床分析,目的是为了提高对Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的认识及诊断水平。 临床资料 一、病例选择１０例均为住院患者,时间从１９９５年６月一１９９８年２月,部分患者曾多次住院,其中男性５例,女性５例,年龄２６－７５岁,平均５３．１０岁,入院后被误诊为肝硬化或其他疾病者３例。所有病例经临床表现,彩色多普勒超声,特别是磁共振血管显影（ＭＲＡ）等明…     

姐弟同患Budd-Chiari综合征

例 1　女 ,36岁 ,以反复腹胀、下肢水肿 8年就诊。 8年前于妊娠中出现腹胀 ,双下肢水肿。 6年前因“脾大”、下肢水肿、腹水 ,经腹部超声及化验等检查拟诊为“Ｂｕｄｄ Ｃｈｉａｒｉ”综合征。 1年前经螺旋ＣＴ检查示下腔静脉血栓形成。入院体检 :贫血貌 ,营养较差 ,面部及下肢皮肤色素沉着 ,巩膜无黄染 ,未见蜘蛛痣 ,颈静脉无怒张 ,胸壁及腹壁见多条粗大曲张静脉 ,心肺未见异常 ,肝肋下未触及 ,脾肋下 5ｃｍ ,质稍硬 ,无压痛 ,腹水征 (- ) ,双下肢轻度凹陷性水肿。实验室检查 :白细胞 5 .4× 10 9/Ｌ ,红细胞 4 .0 6× 10 12 /Ｌ ,血红蛋白…     

肝硬化并门静脉血栓形成、Budd-Chiari综合征1例报告

患者男 ,5 1岁 ,因右上腹疼痛 3天 ,呕血、黑便伴头晕 6小时急诊入院。平时健康 ,否认肝炎、结核等病史 ,无血吸虫病史及手术史。查体 :中年男性 ,发育、营养正常 ,神志清 ,中度贫血貌 ,皮肤粘膜苍白 ,未见肝掌、蜘蛛痣。浅表淋巴结无肿大 ,巩膜无黄染 ,唇白 ,颈静脉无怒张 ,心、肺检查无异常。腹软 ,未见腹壁静脉怒张 ,无压痛 ,肝肋下未及 ,脾肋下约3 cm,移动性浊音 ( + ) ,双下肢浅表静脉曲张 ,轻度凹陷性浮肿。门诊化验检查 :WBC11.2× 10 9/ L,RBC2 .78× 10 9/ L,Hb91g/ L ,PL T13 7× 10 9/ L ,MCV94.6fl,MCHC3 4.6g/ L ;大…     

原发性肝癌合并Budd-Chiari综合征的影像诊断与介入治疗

目的探讨原发性肝癌合并Budd-Chiari综合征(BCS)的影像诊断与血管内介入治疗价值.方法对42例原发性肝癌合并BCS者进行回顾性分析,所有患者均行超声和CT检查,18例行MRI检查,17例行肝动脉造影,9例行下腔静脉造影.7例行下腔静脉内介入治疗.结果通过超声、CT、MRI和血管造影几种影像学检查相互印证而诊断为原发性肝癌合并BCS者42例,其中下腔静脉癌栓者36例,下腔静脉狭窄者6例.7例原发性肝癌合并BCS者成功地施行了下腔静脉内介入治疗,腔静脉压力阶差由术前的(2.5±1.2)kPa降为(0.8±0.2)kPa.术后患者症状明显缓解,无严重并发症发生.结论超声、CT、MRI和血管造影对原发性肝癌合并BCS有较高的诊断价值,其相互补充有助于本病的正确诊断.血管内介入治疗是原发性肝癌合并BCS的有效治疗方法.     

叶酸对Budd-Chiari综合征患者支架术后血浆同型半胱氨酸浓度及再狭窄的影响

目的 探讨叶酸对Budd-Chiari综合征（BCS）患者支架术后血浆同型半胱氨酸（Hcy）浓度的干预效果及其对下腔静脉或肝静脉再狭窄的影响.方法 选择接受过腔内支架置入术随访复查的56例Budd-Chiari综合征患者为研究对象,将56例患者分为叶酸组（30例）和对照组（26例）2组.高效液相色谱法分析术后1周、3个月、半年血浆Hcy水平,在相同的时间段复查彩色多普勒评估血管再狭窄情况,化学发光法测量治疗前后血浆叶酸的浓度.结果 对Budd-Chiari综合征患者应用叶酸治疗后血浆Hcy浓度明显低于治疗前,差异有显著性（P＜0.01）,术后半年30例叶酸组患者中有3例发生再狭窄,再狭窄率为10.0%;对照组常规治疗后血浆Hcy浓度较治疗前无明显变化（P＞0.05）,有10例发生了再狭窄,再狭窄率为38.5%,与叶酸组比较差异有显著性（P＜0.05）.结论 叶酸可降低Bucld-Chiari综合征患者血浆Hey浓度,血浆Hcy水平与血清叶酸水平呈负相关（r=-0.833）;叶酸有降低Budd-Chiari综合征患者支架术后下腔静脉或肝静脉早期（术后半年）再狭窄率的作用.     

Budd—Chiari综合征18例回顾分析

Budd-Chiari综合征（BCS）临床表现较为复杂,常与肝硬化门静脉高压症相混淆而导致误诊。现对18例确诊的BCS病例进行回顾性分析,以期提高对该病的认识和诊断水平。材料与方法一、一般资料近5年上海第二医科大学附属仁济医院和上海市策一人民医院消化内科共收治BCS患者18例,男7例,女11例,平均年龄54．5岁（26～75岁）。从出现较典型症状至确诊的时间：＜1个月1例,1个月～1年8例,＞1年9例。6例死亡患者中,4例死于肝肾综合征伴代谢性酸中毒和高血钾,1例死于感染性休克,1例死于多脏器功能衰竭。二、诊断方法根据《临床肝胆系病学…     

8例柏-查氏综合征患者误诊原因分析

柏-查氏征(B-CS),又称肝静脉阻塞综合征,系指因肝静脉狭窄或阻塞,包括邻近肝脏的下腔静脉部分或完全性阻塞而引起肝静脉和/或下腔静脉回流障碍。临床表现为腹痛,肝脾肿大,腹水,食管静脉曲张、出血以及下肢浅表静脉曲张,色素沉着或溃疡等复杂的症侯群。由于复杂多样,易致误诊误治。现将我院肝科近几年来误诊为肝硬变腹水而收治住院的8例BCS患者情况报告如下,以分析误诊原因。     

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。     

假性Budd-Chiari综合征2例

假性Budd-Chiari综合征多发生于酒精性肝病的患者,其彩超和CT检查类似于Budd-Chiari综合征,而选择性下腔静脉造影无异常发现.本文报告2例患者均因腹胀、乏力入院.入院后进行查体、实验室检查、超声、肝脏增强CT和下腔静脉造影检查,最后确诊为假性Budd-Chiaxi综合征,此病罕见.     

球囊导管成形术治疗膜状Budd—Chiari综合征（附11例报告）

Budd—Chiari综台征(BCS)常与肝硬变门脉高压相混淆，以致延误治疗时机。近年来诊断技术的不断提高，本病的报道逐渐增多，新的方法脱颖而出。我科与放射科近3年来采用PTA治疗BCSll例，报告如下。     

重症布-加综合征的诊断与治疗

目的：探讨重症布－加综合征的诊断标准与治疗方案。方法：对我院治疗的126例重症布－加综合征病人的临床资料进行回顾性分析。介入治疗10例，均行经皮肝穿肝静脉球囊导管扩张成形和内支架置入；手术治疗102例，其中行肠系膜上静脉－下腔静脉人工血管C型架桥术68例，脾静脉－颈内静脉人工血管架桥术33例，肠系膜上静脉－颈内静脉人工血管架桥术1例；介入加常规手术治疗14例，采用Seldinger技术行下腔静脉球囊导管扩张成形和内支架置入后行肠系膜上静脉－下腔静脉人工血管C型超桥术或改良脾－肺固定术。结果：围手术期死亡6例，随访120例，随访时间6个月－7年，效果优者89例（74．2％），良者31例（25．8％）。结论：提出了重症布－加综合征的诊断标准，不同的病变类型应采取不同的治疗方法。     

Possible association between Budd-Chiari Syndrome and gemtuzumab ozogamicin treatment in a patient with refractory acute myelogenous leukemia

Gemtuzumab ozogamicin (GO; CMA-676; Mylotarg) is a chemotherapeutic agent approved for the treatment of CD33-positive acute myelogenous leukemia in patients of age 60 years or older after first relapse. Hepatic veno-occlusive disease has been reported to develop as a late complication of gemtuzumab ozogamicin treatment. A patient who developed Budd-Chiari Syndrome with hepatic vein thrombosis following treatment with GO is presented. This complication has not been previously reported, and it deserves to be considered as a possible adverse effect of gemtuzumab ozogamicin.     

球囊扩张合并支架置入治疗肝静脉阻塞型布-加综合征疗效观察

目的 观察球囊扩张合并支架置入治疗单纯肝静脉阻塞型布-加综合征(Budd-Chiari-syndrome,BCS)的疗效.方法 2001年7月至2006年9月采用多普勒超声引导下经皮经肝穿刺肝静脉并肝静脉造影术、经颈静脉行肝静脉成形术、肝静脉球囊扩张及支架置入术治疗肝静脉阻塞型BCS患者43例.结果 术前43例肝静脉平均压力为32.5 cm H2O(1 cm H2O=0.098 kPa),支架置入后立刻下降为20 cm H2O(t=11.5,P＜0.01),再次肝静脉造影显示肝静脉支架通畅,肝内肝静脉侧支消失.其中38例症状立刻消失,5例部分好转.术后43例随访期间(1～62个月,平均31.5个月),1例出院后1个月因上消化道大出血死亡,其余42例症状体征均无再发,术后多普勒超声检查肝静脉均通畅.治疗成功率为100%,所有患者均未出现严重并发症.结论 多普勒超声引导下经皮经肝穿刺肝静脉和经颈静脉途径开通肝静脉,球囊扩张成形及支架置入治疗单纯肝静脉阻塞型BCS安全、有效.     

Hyperhomocysteinemia and the MTHFR C677T mutation in Budd-Chiari syndrome

Hyperhomocysteinemia (HH) is a factor that predisposes individuals to thrombosis, and the C677T mutation in the 5,10-methylenetetrahydrofolate reductase (MTHFR) is known to give increased plasma homocysteine. However, little is known about their roles in Budd-Chiari syndrome (BCS). This study evaluated the roles of HH and the MTHFR C677T mutation in patients with BCS. We compared 41 BCS patients with 80 sex- and age-matched healthy controls. The mean plasma homocysteine level was significantly higher in patients with BCS (20.15 +/- 5.78 micromol/L) compared with normal controls (15.80 +/- 6.58 micromol/L), P < 0.01. HH (>19.5 micromol/L in men and >15.0 micromol/L in women) was detected in 15 (36.59%) patients and in 14 (17.5%) controls (odds ratio [OR], 2.72; 95% confidence internal [CI], 1.17-6.32). The prevalence of the mutated MTHFR 677TT genotype and the 677T allele in normal controls was 10.0% and 31.3%, respectively. The mutant 677T homozygotes and alleles were more frequent in patients with BCS than in controls (22.0% vs. 10.0%, 0.025 < P < 0.05; 45.1% vs. 31.3%, 0.025 < P < 0.05). The relative risk of BCS among the carriers of 677TT was significantly increased (OR, 3.3; 95% CI, 1.1-10.0). The mutant MTHFR heterozygous 677C/T carriers were not significantly increased in patients with BCS compared with controls (46.3% vs. < 2.5%, P > 0.05). The relative risk OR of BCS among carriers of 677C/T was 1.6 (95% CI, 0.7-3.6). This study suggests that both HH and the homozygous C677T mutation in the MTHFR gene are important risk factors of BCS.     

Pentadecapeptide BPC 157 resolves suprahepatic occlusion of the inferior caval vein,Budd-Chiari syndrome model in rats

BACKGROUND Recently, as a possible therapy resolving solution, pentadecapeptide BPC 157 therapy, has been used in alleviating various vascular occlusion disturbances.BPC 157 was previously reviewed as novel mediator of Robert cytoprotection and endothelium protection in the stomach, and gut-brain axis, beneficial therapy in gastrointestinal tract, with particular reference to vascular recruitment, ulcerative colitis and tumor cachexia, and other tissues healing. Here we raised new hypothesis about BPC 157 therapy in the Budd-Chiari syndrome in rats, rapid bypassing of the suprahepatic inferior caval vein occlusion, and rats recovery with the active and effective pharmacotherapy treatment.AIM To investigate Budd-Chiari syndrome model(inferior caval vein suprahepatic occlusion) resolution, since BPC 157 resolves various rat vascular occlusion.METHODS We assessed the activated bypassing pathways between the inferior and superior caval veins and portocaval shunt, counteracted caval/portal hypertension, aortal hypotension, venous/arterial thrombosis, electrocardiogram disturbances, liver and gastrointestinal lesions(i.e., stomach and duodenum hemorrhages, in particular, congestion). Rats with suprahepatic occlusion of the inferior vena cava by ligation were medicated at 1 min, 15 min, 24 h, or 48 h post-ligation.Medication consisted of 10 μg/kg BPC 157, 10 ng BPC 157 or 5 m L/kg saline,administered once as an abdominal bath or intragastric application. Gross and microscopic observations were made, in addition to assessments of electrical activity of the heart(electrocardiogram), portal and caval hypertension, aortal hypotension, thrombosis, hepatomegaly, splenomegaly and venography.Furthermore, levels of nitric oxide, malondialdehyde in the liver and serum enzymes were determined.RESULTS BPC 157 counteracted increased P wave amplitude, tachycardia and ST-elevation,i.e., right heart failure from acute thrombotic coronary occlusion. The bypassing pathway of the inferior vena cava-azygos(hemiazygos) vein-superior vena cava and portocaval shunt occurred rapidly. Even with severe caval portal hypertension, BPC 157 antagonized portal and caval hypertension and aortal hypotension, and also reduced refractory ascites. Thrombosis of portal vein tributaries, inferior vena cava, and hepatic and coronary arteries was attenuated.In addition, there was reduced pathology of the lungs(severe capillary congestion) and liver(dilated central veins and terminal portal venules),decreased intestine hemorrhagic lesions(substantial capillary congestion,submucosal edema and architecture loss), and increased liver and spleen weight.During the period of ligation, nitric oxide-and malondialdehyde-levels in the liver remained within normal healthy values, and increases in serum enzymes were markedly reduced.CONCLUSION BPC 157 counteracts Budd Chiari syndrome in rats.     

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.     

Prevalence of the JAK2V617F mutation in Chinese patients with Budd-Chiari syndrome and portal vein thrombosis: a prospective study

Background and Aim: Whether routine screening for the JAK2V617F mutation should be performed in Chinese patients with Budd‐Chiari syndrome (BCS) and portal vein thrombosis (PVT) is unclear. Therefore, we aimed to evaluate the prevalence of the JAK2V617F mutation in such patients and to explore the risk factors associated with the mutation. Methods: All consecutive patients with BCS and PVT diagnosed between September 2009 and May 2011 were prospectively enrolled in the observational study and underwent the JAK2V617F mutation detection. Results: Prevalence of the JAK2V617F mutation was 4.3% (4/92) in patients with primary BCS, 26.6% (17/64) in non‐malignant and non‐cirrhotic patients with PVT, and 1.4% (1/71) in cirrhotic patients with PVT. All BCS patients with the JAK2V617F mutation had both platelet count (PLT) of above 100 × 10⁹/L (range, 107–188 × 10⁹/L) and splenomegaly. In non‐malignant and non‐cirrhotic patients with PVT, higher PLT and older ages were the independent predictors of the JAK2V617F mutation. Further, the difference in PLT between the patients with and without the mutation displayed greater significance in the subgroup of patients with splenomegaly (P < 0.0001), but the statistical significance disappeared in the subgroup of patients with splenectomy (P = 0.1312). Conclusions: The low prevalence of the JAK2V617F mutation in patients with BCS suggests that myeloproliferative neoplasm should be an uncommon etiological factor of BCS in China. Routine screening for the JAK2V617F mutation might be recommended in non‐malignant and non‐cirrhotic patients with PVT, but not in cirrhotic patients with PVT. The coexistence of higher PLT and splenomegaly might be closely associated with the JAK2V617F mutation.