华南地区布—加综合征的临床研究
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摘要
第一部分98例广东省籍布-加综合症患者临床流行病学的初步研究
研究背景以及目的
Budd-Chiari综合征(Budd-Chiari syndrome,BCS)是由于肝静脉或/和下腔静脉阻塞导致肝静脉或/和下腔静脉血液回流障碍而产生的门静脉高压或/和下腔静脉高压的一系列症候群,病因复杂,且确切的原因尚未明。从世界范围看欧美国家报道较少,中国、印度、尼泊尔等发展中国家较多。我国有关BCS病例报道也较多,目前报道的病例分布主要集中在黄河和淮河地区。较少资料总结华南地区,尤其是广东省籍BCS患者的流行病学特点情况。广东省目前作为中国人口第一大省,拥有一亿以上的人口,却尚无BCS的临床流行病学分析报道。而其实现实工作中,广东省籍的BCS患者也不在少数,为填补此项空白,本研究收集了98例BCS广东省籍患者的相关资料采用临床住院资料(非人群的流行病学结果),对其进行临床流行病学特征行初步调查,旨在初步了解BCS在本省临床流行病学特征,为本病的病因研究、临床诊断、治疗提供临床流行病学依据。
研究方法
搜集从1995年1月-2012年12月年资料完整的住院病例(非人群的流行病学结果),包括广州南方医院(59例),北京大学深圳医院(3例),深圳市人民医院(2例),中山大学第一、二、三附属医院(分别为2,3,25例),暨南大学第一附属医院(2例),梅州市人民医院(2例),户籍和祖籍均为广东省的原发性BCS病例的共98例。采用李彦豪分型,即以肝静脉为中心的分型。将BCS患者分成四个类型。分析各类型的发病趋势、发病年龄、性别、分布区域、可能的诱因、各种类型BCS的分布、主诉、体征等特征。研究对象纳入标准：本研究定义的BCS患者为肝静脉流出道从肝小静脉到腔静脉或右房之间的阻塞导致的肝静脉回流障碍综合症。所有病例采用金标准F腔静脉造影、肝静脉造影或外科手术确诊为BCS患者,并且户籍和祖籍均为广东省,能收集到详细的随访资料。排除标准：a.排除由于肝脏占位、心功能衰竭,OLT(肝移植)等继发的BCS患者。b.排除非广东省籍患者。
对98例BCS患者的临床流行病学特征及临床症状进行总结分析。制定BCS临床流行病学调查表格输入Excel2003建立数据库。分析广东户籍人口BCS患者特征：包括年龄,性别,职业,,是否从事重体力劳动,地域,主诉,术前术后影像学表现,影像解剖分型,主要体征,合并诊断或可能的致病因素等情况。
结果
收集的98例BCS患省的平均年龄为39.0岁,标准差为13.5。性别分布的统计学分析尚不能认为男性在构成比上占优势。发现广东省籍BCS患者呈逐年增多趋势。成人多发,男女比例为1.62：1。粤西地区为高发地区。导致BCS可能的诱因包括：职业,重体力劳动,血液高凝状态,恶性肿瘤等因素。影像学分型：I型25例(25.5%),Ⅱ型29例(29.6%),Ⅲ型39例(39.8%),Ⅳ型5例(5.10%)。肝静脉受累为主的Ⅱ型+Ⅰ型患者超过Ⅲ型单纯腔静脉阻塞型。腹胀是最常见的主诉,最常见的体征依次为腹水、下肢水肿、胸腹壁静脉曲张。
结论
初步判断广东省籍BCS发病率明显低于黄淮河流域,高发区域为粤西地区,经济文化越发达地区,发病率越低。影像解剖分型以肝静脉受累为最常见。Ⅰ型和Ⅳ型多能找到病因,Ⅱ型和Ⅲ型无明显病因。
第二部分：不同影像技术对BCS诊疗应用价值的对比研究
研究背景以及目的
BCS多数起病隐蔽,与肝硬化门脉高压症等容易混淆,诊断多依赖影像手段。BCS患者的影像分型诊断,对治疗方式的选择以及患者预后的判断意义重大。故所有影像学检查均应以了解肝静脉为中心,必须回答下列问题：肝静脉是否存在狭窄或者闭塞?如果存在下腔静脉狭窄或闭塞,肝静脉是否受到影响和怎样的影响?其次是了解相关伴随的异常征,如有无继发血栓和侧支循环等。当然引起静脉血管狭窄或闭塞的血栓性质也将决定治疗方式的不同。利用不同的影像诊断手段对这些问题进行术前分析具有很高的临床价值。本研究目的即为了对比分析不同影像技术在BCS诊疗巾的应用价值,了解不同影像技术的优势和劣势。
研究方法
采用回顾性分析50例经DSA证实的BCS患者影像特点。研究对象：回顾性研究了2005年1月-2012年12月期间在广州南方医院、北京大学深圳医院进行介入治疗的50名BCS患者的影像资料,术前行US,CT和(或)MRI检查。患者中男28例,女22例,年龄15-83岁,中位年龄37.2岁。所有病例术前经US筛查,30例患者术前进行了CT检查,10例患者术前进行了MRI检查,10例患者术前同时进行了CT和MRI检查,所有患者均经DSA造影证实BCS的诊断。以DSA为金标准,比较分析US、CT、MRI三者在显示BCS患者静脉狭窄或闭塞,判断影像解剖分型；显示肝内外侧枝循环；分析静脉腔内血栓性质等方面表现的差异。分析不同影像技术在BCS诊疗中的应用价值。
结果
1.以DSA标准组为对照,证实US在显示肝左、中、右静脉狭窄与DSA不存在差异,在显示腔静脉狭窄方而与DSA,p值为0.03,也存在统计学差异。CT、MRI在显示肝左、右、肝中静脉、腔静脉狭窄上与DSA无差异。由此可见,在静脉狭窄的判断上仅US在判断静脉狭窄时与DSA存在统计学差异。
2.证实US在显示肝左静脉和下腔静脉闭塞上,与DSA组比较p值分别为0.01,0.02,均小于0.05,存在统汁学左异。CT显示肝左、中、右静脉以及下腔静脉闭塞方面与DSA存在差异,判断闭塞存在的阳性率高过DSA,存在过诊问题。与标准组比较,显示肝静脉闭塞的情况CT误诊率最高。其次为US。对腔静脉闭塞的判断US也存在漏诊问题,CT存在过诊问题。MRI在显示肝左、中、右静脉闭塞、腔静脉闭塞上与DSA无明显差异。
3.与DSA标准组比较,由于US、CT、MRI在显示肝静脉、腔静脉狭窄或闭塞方面表现的不同。所以它们在BCS的病理分型准确性上也存在差异。本组卡方检验,观察每一型中,三种影像方法与DSA的差别,只有Ⅲ型,Ⅳ型的判断上US与DSA组的判断差异有统计学意义,其他无统计学意义。CT、MRI分型情况与DSA基本类似。
4.对血栓的性质判断以DSA结合临床治疗病史组为金标准。US与DSA,MRI与DSA之间无统计学差异，其中US的判断与DSA以及患者的临床情况最接近。对于腔静脉血栓性质的判断US诊断准确性减低(因为体深部,干扰和难度加大),但是US和MRI与与标准组比较对肝静脉和腔静脉血栓的判断均无统计学差异。CT对陈旧性血栓的判断与标准组比较无差异,但是对新鲜血栓的判断与DSA组比较,p=0.03<0.05,认为结果有统计学差异。
结论
1.对于BCS分型诊断、侧枝循环的显示、静脉内血栓性质的判断,敏感性和准确性最高均为MRI；MRI目前是BCS无创检查方法中特异度和灵敏度最接近DSA的一种方法。MRA在显示肝静脉的解剖结构和病变方面与DSA显示相当,在判断总体肝静脉情况方面优于经股静脉肝静脉和经皮肝穿刺肝静脉造影。
2.对血栓性质的判断US强于CT；超声诊断BCS虽然有其局限性,但方便、无创、价廉、还可以大致明确病变类型范围、梗阻程度及侧支循环路径,准确判断血栓的性质等,为临床提供有价值参考资料；不失为筛选BCS的首选方法。
3.CT检查对BCS的分型诊断准确性高于US,侧枝循环的显示CT也优于US。
第三部分131例华南区BCS患者介入诊疗后预后的影响因素分析
研究背景和目的
我国BCS主要的流行地区是黄淮河流域,广东乃至华南地区的BCS患者呈散发趋势。根据资料报道,华南地区总体发病率明显低于黄淮河流域。临床上华南地区患者也表现出与北方BCS患者的一些不同特征。实际工作中我们发现华南地区BCS患者多数预后尚可,但不同类型BCS患者群也存在一些不同点；影响其介入治疗预后的可能因素较多,本研究拟顾性观察分析经DSA证实的华南地区BCS患者介入治疗后的预后情况。为临床诊治BCS患者,改善患者的预后提供帮助。
研究方法
回顾性采集1995年1月至2012年12月131例经DSA或手术证实的BCS患者临床资料,研究对象纳入标准本研究定义BCS为肝静脉流出道从肝小静脉到腔静脉或右房之间梗阻堵塞引起的症状群。a.所有患者结合临床症状,并通过US,CTA或MRI检查,诊断为BCS。b.均采用金标准下腔静脉造影、肝静脉造影或外科手术确诊为BCS的患者。c.有详细影像、实验室检查、介入手术记录,有3个月以上随访记录。研究对象排除标准a.排除由于肝肿瘤、心功能衰竭,OLT(肝移植)等继发性BCS患者。b.排除缩窄性心包炎患者。c.术后无任何随访记录的患者。根据患者症状复发时间,术后满意度、术后存活时间等情况,分为预后好和预后差组。记录与患者预后相关的多个参数,包括：性别、年龄、术前有无合并肝硬化火代偿、发病到确诊时间、影像学分型、侧枝循环、治疗方式、治疗前后肝脏体积变化、肝静脉,腔静脉压力变化、胆红素变化等。用单变量logistic回归分析得出与BCS患者预后显著相关的变量。比较不同类型BCS患者生存曲线的不同。用Cox回归分析影响生存的危险因素。
结果
Logistic回归分析表明接受介入治疗后,影响BCS患者预后的显著相关独立变量包括：初次就诊到确诊时间、治疗方式、术前有无合并肝硬化失代偿、影像解剖分型。治疗前后肝左叶前后径、肝静脉、腔静脉压力变化差异存在统计学差异,胆红素变化值无差异。四种类型的病人生存时间差异存在统计学意义,从生存曲线即可看出生存率从大到小依次为Ⅱ,Ⅰ,Ⅲ,Ⅳ。第Ⅳ型的生存时间均短于其他三型。用术后存活时间做时间变量,1995年至2012年期间Ⅰ、Ⅱ、Ⅲ型患者的生存率均大于50%,故中位生存期无法计算；Ⅳ型生存率为20%,Ⅳ型中位生存期为12个月。分型和治疗方式的不同是影响患者生存时间的主要因素。131例患者1995年1月-2012年12月内累积死亡率33.6%,对应不同类型BCS患者死亡率,Ⅳ型患者为80.0%,死亡率最低为Ⅰ型患者,为22.5%,其次为Ⅱ型24.8%,和Ⅲ型32.3%。
结论
1.影响BCS患者介入治疗后预后的主要因素为：解剖分型、治疗方式、术前有无合并肝硬化失代偿、初次就诊到确诊时间。
2.本组研究中发现Ⅳ型BCS病死率高,预后最差：Ⅱ型患者死亡率最低。
3.介入治疗时机的准确把握,与BCS患者预后紧密相关。
4.介入治疗后个体化抗凝治疗,可降低出血并发症,提高远期生存率。
Objective To understand the patients'clinical epidemiological characteristics with Budd-Chiari syndrome (BCS) in Guangdong Province; to help etiological diagnosis, clinical diagnosis and treatment for Budd-Chiari syndrome. Method98BCS patients with epidemiological data in Guangdong Province were retrospectively analyzed.The hepatic vein was as the center of all patients who were divided into different types of Budd-Chiari syndrome. Analysis incidence trend, onset age, gender, regional distribution of different type, the causes, symptoms and so on. Results It could been found that patients with Budd-Chiari in Guangdong Province is increasing year by year; most of them are adult, the ratio of men to women was1.62, the high incidence area is western region, the causes include:heavy physical labor, occupation, high blood coagulation factors,tumor.Imaging anatomy I type has25cases (25.5%) patients, type Ⅱ29patients (29.6%), Ⅲ type39cases (39.8%) patients, Ⅳ type5cases (5.10%); The patients of hepatic vein type Ⅱ+Ⅰ is more than Ⅲ type. Abdominal distension is the main complaint. The most common signs is ascites, edema, thoracic abdominal varicose veins of lower limbs. Conclusion Preliminary judgment is BCS of Guangdong provincialism, significantly has a lower incidence of huang-huai river area. The high incidence area in Guangdong is the yuexi area.The more developed regions, the lower the incidence of imaging anatomical.Type Ⅰ and type Ⅳ can find the most common cause,but type Ⅱ and type Ⅲ has no obvious cause.
Objective To analysis of50cases of Budd-Chiari syndrome in patients with US, CT, MR1and DSA performance, to evaluation of different imaging techniques for the diagnosis of Budd-Chiari syndrome. Methods A retrospective analysis of50cases confirmed by DSA image characteristics in patients with Budd-Chiari syndrome, as the gold standard DSA angiography results, comparative analysis of US, CT, MRI three display Bucharest in patients with hepatic vein stenosis or occlusion of the inferior vena cava; parting diagnostic imaging anatomy; extrahepatic collateral circulation; the endovenous thrombosis nature of performance.
Results CT, MRI, determine the hepatic vein, inferior vena cava performance close to the DSA, the accuracy rate is better than U.S..Pathological diagnosis and judge the extrahepatic collateral circulation on CT, MRI with high accuracy; compared with CT and MRI. US is in disadvantage.To judge thrombus nature US. MRI have the high accuracy. Conclusion US is cheap, practical, but lower accuracy rate in diagnosis BCS. CT, MRI display vena cava, hepatic vein stenosis or occlusion, compensatory extrahepatic collateral circulation, the imaging anatomy typing diagnostic which are of accuracy rate. US, MRI determine accurate and effective nature of thrombosis.
Object To discuss prognostic factors after interventional treatment for BCS patients after interventional therapy.In order to improve the BCS interventional therapeutic effect and improve the prognosis of patients. Method131BCS patients were retrospectively collected confirmed BCS by DSA or surgery from Jun.1995to Dec.2012.According to symptoms recurrence time, satisfaction, survival time,to divide patients into good and poor prognosis group.To analyse parameters including:gender, age, whether merger decompensated cirrhosis performance, disease to diagnose time, image parting, collateral circulation, treatment method, the liver volume change before and after treatment, hepatic vein, vena cava pressure change, bilirubin change.With single variable logistic regression analysis and BCS prognosis significantly related variables.Comparing different image anatomical parting BCS survival curve of different.Cox regression analysis with affect survival risk factors. Results Logistic regression analysis showed that and accept the interventional therapy of BCS the prognosis of patients with significant independent variables include:the disease to diagnose time, treatment method, decompensated cirrhosis, image anatomical parting.The difference before and after treatment the left lobe of liver diameter, hepatic vein, vena cava pressure change are statistically significant.Bilirubin change value is indifference.Four type of BCS patient there is a difference about survival time.the difference has statistical significance. The fourth type of survival time is shorter than the other three type.With postoperative survival time for as variable, from1995to2012, Ⅰ, Ⅱ,Ⅲ type survival rates were greater than50%.IV type survival rate was20%. the median survival time is12months. The classification and different treatment is the risk factors to the survival of patients.Conclusion The prognosis of patients with BCS after interventional therapy is related with image parting, treatment method, decompensated cirrhosis, and the disease to diagnose time,however the gender, the collateral circulation, age has no relationship. Interventional therapy is suitable for all types of BCS, and has the advantages of high curative effect, less complications.
研究背景以及目的
Budd-Chiari综合征(Budd-Chiari syndrome,BCS)是由于肝静脉或/和下腔静脉阻塞导致肝静脉或/和下腔静脉血液回流障碍而产生的门静脉高压或/和下腔静脉高压的一系列症候群,病因复杂,且确切的原因尚未明。从世界范围看欧美国家报道较少,中国、印度、尼泊尔等发展中国家较多。我国有关BCS病例报道也较多,目前报道的病例分布主要集中在黄河和淮河地区。较少资料总结华南地区,尤其是广东省籍BCS患者的流行病学特点情况。广东省目前作为中国人口第一大省,拥有一亿以上的人口,却尚无BCS的临床流行病学分析报道。而其实现实工作中,广东省籍的BCS患者也不在少数,为填补此项空白,本研究收集了98例BCS广东省籍患者的相关资料采用临床住院资料(非人群的流行病学结果),对其进行临床流行病学特征行初步调查,旨在初步了解BCS在本省临床流行病学特征,为本病的病因研究、临床诊断、治疗提供临床流行病学依据。
研究方法
搜集从1995年1月-2012年12月年资料完整的住院病例(非人群的流行病学结果),包括广州南方医院(59例),北京大学深圳医院(3例),深圳市人民医院(2例),中山大学第一、二、三附属医院(分别为2,3,25例),暨南大学第一附属医院(2例),梅州市人民医院(2例),户籍和祖籍均为广东省的原发性BCS病例的共98例。采用李彦豪分型,即以肝静脉为中心的分型。将BCS患者分成四个类型。分析各类型的发病趋势、发病年龄、性别、分布区域、可能的诱因、各种类型BCS的分布、主诉、体征等特征。研究对象纳入标准：本研究定义的BCS患者为肝静脉流出道从肝小静脉到腔静脉或右房之间的阻塞导致的肝静脉回流障碍综合症。所有病例采用金标准F腔静脉造影、肝静脉造影或外科手术确诊为BCS患者,并且户籍和祖籍均为广东省,能收集到详细的随访资料。排除标准：a.排除由于肝脏占位、心功能衰竭,OLT(肝移植)等继发的BCS患者。b.排除非广东省籍患者。
对98例BCS患者的临床流行病学特征及临床症状进行总结分析。制定BCS临床流行病学调查表格输入Excel2003建立数据库。分析广东户籍人口BCS患者特征：包括年龄,性别,职业,,是否从事重体力劳动,地域,主诉,术前术后影像学表现,影像解剖分型,主要体征,合并诊断或可能的致病因素等情况。
结果
收集的98例BCS患省的平均年龄为39.0岁,标准差为13.5。性别分布的统计学分析尚不能认为男性在构成比上占优势。发现广东省籍BCS患者呈逐年增多趋势。成人多发,男女比例为1.62：1。粤西地区为高发地区。导致BCS可能的诱因包括：职业,重体力劳动,血液高凝状态,恶性肿瘤等因素。影像学分型：I型25例(25.5%),Ⅱ型29例(29.6%),Ⅲ型39例(39.8%),Ⅳ型5例(5.10%)。肝静脉受累为主的Ⅱ型+Ⅰ型患者超过Ⅲ型单纯腔静脉阻塞型。腹胀是最常见的主诉,最常见的体征依次为腹水、下肢水肿、胸腹壁静脉曲张。
结论
初步判断广东省籍BCS发病率明显低于黄淮河流域,高发区域为粤西地区,经济文化越发达地区,发病率越低。影像解剖分型以肝静脉受累为最常见。Ⅰ型和Ⅳ型多能找到病因,Ⅱ型和Ⅲ型无明显病因。
第二部分：不同影像技术对BCS诊疗应用价值的对比研究
研究背景以及目的
BCS多数起病隐蔽,与肝硬化门脉高压症等容易混淆,诊断多依赖影像手段。BCS患者的影像分型诊断,对治疗方式的选择以及患者预后的判断意义重大。故所有影像学检查均应以了解肝静脉为中心,必须回答下列问题：肝静脉是否存在狭窄或者闭塞?如果存在下腔静脉狭窄或闭塞,肝静脉是否受到影响和怎样的影响?其次是了解相关伴随的异常征,如有无继发血栓和侧支循环等。当然引起静脉血管狭窄或闭塞的血栓性质也将决定治疗方式的不同。利用不同的影像诊断手段对这些问题进行术前分析具有很高的临床价值。本研究目的即为了对比分析不同影像技术在BCS诊疗巾的应用价值,了解不同影像技术的优势和劣势。
研究方法
采用回顾性分析50例经DSA证实的BCS患者影像特点。研究对象：回顾性研究了2005年1月-2012年12月期间在广州南方医院、北京大学深圳医院进行介入治疗的50名BCS患者的影像资料,术前行US,CT和(或)MRI检查。患者中男28例,女22例,年龄15-83岁,中位年龄37.2岁。所有病例术前经US筛查,30例患者术前进行了CT检查,10例患者术前进行了MRI检查,10例患者术前同时进行了CT和MRI检查,所有患者均经DSA造影证实BCS的诊断。以DSA为金标准,比较分析US、CT、MRI三者在显示BCS患者静脉狭窄或闭塞,判断影像解剖分型；显示肝内外侧枝循环；分析静脉腔内血栓性质等方面表现的差异。分析不同影像技术在BCS诊疗中的应用价值。
结果
1.以DSA标准组为对照,证实US在显示肝左、中、右静脉狭窄与DSA不存在差异,在显示腔静脉狭窄方而与DSA,p值为0.03,也存在统计学差异。CT、MRI在显示肝左、右、肝中静脉、腔静脉狭窄上与DSA无差异。由此可见,在静脉狭窄的判断上仅US在判断静脉狭窄时与DSA存在统计学差异。
2.证实US在显示肝左静脉和下腔静脉闭塞上,与DSA组比较p值分别为0.01,0.02,均小于0.05,存在统汁学左异。CT显示肝左、中、右静脉以及下腔静脉闭塞方面与DSA存在差异,判断闭塞存在的阳性率高过DSA,存在过诊问题。与标准组比较,显示肝静脉闭塞的情况CT误诊率最高。其次为US。对腔静脉闭塞的判断US也存在漏诊问题,CT存在过诊问题。MRI在显示肝左、中、右静脉闭塞、腔静脉闭塞上与DSA无明显差异。
3.与DSA标准组比较,由于US、CT、MRI在显示肝静脉、腔静脉狭窄或闭塞方面表现的不同。所以它们在BCS的病理分型准确性上也存在差异。本组卡方检验,观察每一型中,三种影像方法与DSA的差别,只有Ⅲ型,Ⅳ型的判断上US与DSA组的判断差异有统计学意义,其他无统计学意义。CT、MRI分型情况与DSA基本类似。
4.对血栓的性质判断以DSA结合临床治疗病史组为金标准。US与DSA,MRI与DSA之间无统计学差异，其中US的判断与DSA以及患者的临床情况最接近。对于腔静脉血栓性质的判断US诊断准确性减低(因为体深部,干扰和难度加大),但是US和MRI与与标准组比较对肝静脉和腔静脉血栓的判断均无统计学差异。CT对陈旧性血栓的判断与标准组比较无差异,但是对新鲜血栓的判断与DSA组比较,p=0.03<0.05,认为结果有统计学差异。
结论
1.对于BCS分型诊断、侧枝循环的显示、静脉内血栓性质的判断,敏感性和准确性最高均为MRI；MRI目前是BCS无创检查方法中特异度和灵敏度最接近DSA的一种方法。MRA在显示肝静脉的解剖结构和病变方面与DSA显示相当,在判断总体肝静脉情况方面优于经股静脉肝静脉和经皮肝穿刺肝静脉造影。
2.对血栓性质的判断US强于CT；超声诊断BCS虽然有其局限性,但方便、无创、价廉、还可以大致明确病变类型范围、梗阻程度及侧支循环路径,准确判断血栓的性质等,为临床提供有价值参考资料；不失为筛选BCS的首选方法。
3.CT检查对BCS的分型诊断准确性高于US,侧枝循环的显示CT也优于US。
第三部分131例华南区BCS患者介入诊疗后预后的影响因素分析
研究背景和目的
我国BCS主要的流行地区是黄淮河流域,广东乃至华南地区的BCS患者呈散发趋势。根据资料报道,华南地区总体发病率明显低于黄淮河流域。临床上华南地区患者也表现出与北方BCS患者的一些不同特征。实际工作中我们发现华南地区BCS患者多数预后尚可,但不同类型BCS患者群也存在一些不同点；影响其介入治疗预后的可能因素较多,本研究拟顾性观察分析经DSA证实的华南地区BCS患者介入治疗后的预后情况。为临床诊治BCS患者,改善患者的预后提供帮助。
研究方法
回顾性采集1995年1月至2012年12月131例经DSA或手术证实的BCS患者临床资料,研究对象纳入标准本研究定义BCS为肝静脉流出道从肝小静脉到腔静脉或右房之间梗阻堵塞引起的症状群。a.所有患者结合临床症状,并通过US,CTA或MRI检查,诊断为BCS。b.均采用金标准下腔静脉造影、肝静脉造影或外科手术确诊为BCS的患者。c.有详细影像、实验室检查、介入手术记录,有3个月以上随访记录。研究对象排除标准a.排除由于肝肿瘤、心功能衰竭,OLT(肝移植)等继发性BCS患者。b.排除缩窄性心包炎患者。c.术后无任何随访记录的患者。根据患者症状复发时间,术后满意度、术后存活时间等情况,分为预后好和预后差组。记录与患者预后相关的多个参数,包括：性别、年龄、术前有无合并肝硬化火代偿、发病到确诊时间、影像学分型、侧枝循环、治疗方式、治疗前后肝脏体积变化、肝静脉,腔静脉压力变化、胆红素变化等。用单变量logistic回归分析得出与BCS患者预后显著相关的变量。比较不同类型BCS患者生存曲线的不同。用Cox回归分析影响生存的危险因素。
结果
Logistic回归分析表明接受介入治疗后,影响BCS患者预后的显著相关独立变量包括：初次就诊到确诊时间、治疗方式、术前有无合并肝硬化失代偿、影像解剖分型。治疗前后肝左叶前后径、肝静脉、腔静脉压力变化差异存在统计学差异,胆红素变化值无差异。四种类型的病人生存时间差异存在统计学意义,从生存曲线即可看出生存率从大到小依次为Ⅱ,Ⅰ,Ⅲ,Ⅳ。第Ⅳ型的生存时间均短于其他三型。用术后存活时间做时间变量,1995年至2012年期间Ⅰ、Ⅱ、Ⅲ型患者的生存率均大于50%,故中位生存期无法计算；Ⅳ型生存率为20%,Ⅳ型中位生存期为12个月。分型和治疗方式的不同是影响患者生存时间的主要因素。131例患者1995年1月-2012年12月内累积死亡率33.6%,对应不同类型BCS患者死亡率,Ⅳ型患者为80.0%,死亡率最低为Ⅰ型患者,为22.5%,其次为Ⅱ型24.8%,和Ⅲ型32.3%。
结论
1.影响BCS患者介入治疗后预后的主要因素为：解剖分型、治疗方式、术前有无合并肝硬化失代偿、初次就诊到确诊时间。
2.本组研究中发现Ⅳ型BCS病死率高,预后最差：Ⅱ型患者死亡率最低。
3.介入治疗时机的准确把握,与BCS患者预后紧密相关。
4.介入治疗后个体化抗凝治疗,可降低出血并发症,提高远期生存率。
Objective To understand the patients'clinical epidemiological characteristics with Budd-Chiari syndrome (BCS) in Guangdong Province; to help etiological diagnosis, clinical diagnosis and treatment for Budd-Chiari syndrome. Method98BCS patients with epidemiological data in Guangdong Province were retrospectively analyzed.The hepatic vein was as the center of all patients who were divided into different types of Budd-Chiari syndrome. Analysis incidence trend, onset age, gender, regional distribution of different type, the causes, symptoms and so on. Results It could been found that patients with Budd-Chiari in Guangdong Province is increasing year by year; most of them are adult, the ratio of men to women was1.62, the high incidence area is western region, the causes include:heavy physical labor, occupation, high blood coagulation factors,tumor.Imaging anatomy I type has25cases (25.5%) patients, type Ⅱ29patients (29.6%), Ⅲ type39cases (39.8%) patients, Ⅳ type5cases (5.10%); The patients of hepatic vein type Ⅱ+Ⅰ is more than Ⅲ type. Abdominal distension is the main complaint. The most common signs is ascites, edema, thoracic abdominal varicose veins of lower limbs. Conclusion Preliminary judgment is BCS of Guangdong provincialism, significantly has a lower incidence of huang-huai river area. The high incidence area in Guangdong is the yuexi area.The more developed regions, the lower the incidence of imaging anatomical.Type Ⅰ and type Ⅳ can find the most common cause,but type Ⅱ and type Ⅲ has no obvious cause.
Objective To analysis of50cases of Budd-Chiari syndrome in patients with US, CT, MR1and DSA performance, to evaluation of different imaging techniques for the diagnosis of Budd-Chiari syndrome. Methods A retrospective analysis of50cases confirmed by DSA image characteristics in patients with Budd-Chiari syndrome, as the gold standard DSA angiography results, comparative analysis of US, CT, MRI three display Bucharest in patients with hepatic vein stenosis or occlusion of the inferior vena cava; parting diagnostic imaging anatomy; extrahepatic collateral circulation; the endovenous thrombosis nature of performance.
Results CT, MRI, determine the hepatic vein, inferior vena cava performance close to the DSA, the accuracy rate is better than U.S..Pathological diagnosis and judge the extrahepatic collateral circulation on CT, MRI with high accuracy; compared with CT and MRI. US is in disadvantage.To judge thrombus nature US. MRI have the high accuracy. Conclusion US is cheap, practical, but lower accuracy rate in diagnosis BCS. CT, MRI display vena cava, hepatic vein stenosis or occlusion, compensatory extrahepatic collateral circulation, the imaging anatomy typing diagnostic which are of accuracy rate. US, MRI determine accurate and effective nature of thrombosis.
Object To discuss prognostic factors after interventional treatment for BCS patients after interventional therapy.In order to improve the BCS interventional therapeutic effect and improve the prognosis of patients. Method131BCS patients were retrospectively collected confirmed BCS by DSA or surgery from Jun.1995to Dec.2012.According to symptoms recurrence time, satisfaction, survival time,to divide patients into good and poor prognosis group.To analyse parameters including:gender, age, whether merger decompensated cirrhosis performance, disease to diagnose time, image parting, collateral circulation, treatment method, the liver volume change before and after treatment, hepatic vein, vena cava pressure change, bilirubin change.With single variable logistic regression analysis and BCS prognosis significantly related variables.Comparing different image anatomical parting BCS survival curve of different.Cox regression analysis with affect survival risk factors. Results Logistic regression analysis showed that and accept the interventional therapy of BCS the prognosis of patients with significant independent variables include:the disease to diagnose time, treatment method, decompensated cirrhosis, image anatomical parting.The difference before and after treatment the left lobe of liver diameter, hepatic vein, vena cava pressure change are statistically significant.Bilirubin change value is indifference.Four type of BCS patient there is a difference about survival time.the difference has statistical significance. The fourth type of survival time is shorter than the other three type.With postoperative survival time for as variable, from1995to2012, Ⅰ, Ⅱ,Ⅲ type survival rates were greater than50%.IV type survival rate was20%. the median survival time is12months. The classification and different treatment is the risk factors to the survival of patients.Conclusion The prognosis of patients with BCS after interventional therapy is related with image parting, treatment method, decompensated cirrhosis, and the disease to diagnose time,however the gender, the collateral circulation, age has no relationship. Interventional therapy is suitable for all types of BCS, and has the advantages of high curative effect, less complications.
引文
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