5例布-加氏综合征彩色多普勒超声诊断分析

目的探索彩色多普勒超声对肝静脉阻塞型布-加氏综合征的诊断价值。方法应用彩色多普勒超声观察5例肝静脉阻塞型布-加氏综合征的彩色多普勒超声的声像图特点。结果下腔静脉型3例,肝静脉型2例。结论彩色多普勒超声可较准确地判定血管狭窄及阻塞的部位和类型。     

5例布-加氏综合征彩色多普勒超声诊断分析

目的 探索彩色多普勒超声对肝静脉阻塞型布-加氏综合征的诊断价值.方法 应用彩色多普勒超声观察5例肝静脉阻塞型布-加氏综合征的彩色多普勒超声的声像图特点.结果 下腔静脉型3例,肝静脉型2例.结论 彩色多普勒超声可较准确地判定血管狭窄及阻塞的部位和类型.     

下腔静脉闭塞段切除人工血管原位移植治疗布-加氏综合征18例报道

自1994年以来,采用下腔静脉闭塞或狭窄段切除人工血管原位移植治疗布-加氏综合征18例。手术适宜于短段闭塞型、伴有明显狭窄的隔膜型或局限性狭窄型而采用经皮球囊扩张失败或以往采用隔膜切除、心包片成形术而术后复发者。经本治疗术后3～36个月随访,所有病例疗效满意,无一例复发。     

布-加综合征的彩色多普勒超声诊断及与肝硬化的鉴别诊断

目的探讨彩色多普勒超声在诊断布-加综合征及与肝硬化的鉴别诊断中的应用价值。方法32例布-加综合征患者（男性19例,女性13例,平均年龄37岁）和40例门脉性肝硬化患者（男性28例,女性12例,平均年龄48岁）,分别接受二维超声、彩色多普勒超声和频谱多普勒超声检查,并对获得的资料进行回顾性分析。所有患者经血管造影和/或病理、CT证实。结果布-加综合征的典型特征是病变主要累及下腔静脉和肝静脉,病变处管腔闭塞或狭窄,其内血流紊乱,并可出现侧支循环和交通支;肝硬化患者主要表现为肝脏缩小、脾大、门静脉增宽、腹水,血流动力学改变主要以门静脉和肝静脉为主。结论彩色多普勒超声对布-加综合征、肝硬化的诊断及鉴别诊断符合率较高,为诊断布-加综合征和肝硬化提供有效手段,对选择合理治疗方案及评价治疗效果具有指导意义。     

布-加氏综合征的治疗进展

布-加氏综合征(Budd-Chiari Syndrome)是由于肝静脉或邻近的下腔静脉阻塞而产生的一系列临床综合征。随着血管外科的发展，对这种疾病的认识有了进一步提高。由于其病理类型的不同，对于该病征的治疗方法亦较多，约有30余种。本综合近年国内献，对目前常用的布-加氏综合征的治疗方法作一评述。     

布-加氏综合征根治性术式的探讨

目的：统计布-加氏综合征直视下根治术的45例资料，并探讨三种根治性手术的优缺点及适应症。方法：隔膜切除或病变的下腔静脉段切开、心包片扩大成形17例；闭塞或狭窄的下腔静脉段切除、人工血管原位移植24例；肝静脉流出道成形6例。同时行肝静脉至右心房的下腔静脉人口处人工血管间置移植4例。结果：因术后并发症死亡2例；复发4例．均为心包片扩大成形术后的病人。结论：心包片成形术适用于隔膜型，人工血管原位移植适用于短段闭塞或狭窄型．肝静脉流出道成形可用于长段下腔静脉闭塞型。     

肝右静脉与下腔静脉位置对布-加综合征产生机制的影响

目的探讨不同肝右静脉与下腔静脉夹角变化对布-加综合征患者下腔静脉隔膜发生机制的影响。方法运用Mimics软件对临床磁共振成像血管造影下腔静脉和主要肝静脉图像进行三维实体模型重建,测得正常模型下腔静脉与肝右静脉夹角为56°。在保持模型基本结构不变基础上,分别构建肝右静脉与下腔静脉夹角为30°和120°的模型,并数值模拟计算3个模型的壁面剪切力、壁面压强、速度分布。结果 3个模型壁面压强、壁面剪切力差异显著。与正常人56°模型相比,30°模型有较高的壁面压强和较低的血液流速,120°模型有较低的壁面剪切力和血液流速,并伴随有涡流的出现,这些血流动力学因素的改变更易于血栓的形成。56°模型血管内血流流速最快。结论对下腔静脉与肝右静脉血液流场的数值模拟有助于了解布-加综合征的发病机制,提高下腔静脉阻塞隔膜形成风险的预测,为治疗提供理论依据。     

腔内隔绝术在主动脉瘤外科治疗中的应用

目的：探讨腔内隔绝术治疗主动脉夹层动脉瘤、腹主动脉瘤的操作方法及近期疗效。方法：采用腔内隔绝术治疗主动脉夹层动脉瘤3例、腹主动脉瘤2例。结果：5例主动脉瘤腔内隔绝术均获得成功，术后3例主动脉夹层动脉瘤的假腔立刻消失，2例腹主动脉瘤瘤体成功隔绝。无支架移位；术中发生内漏1例，经及时处理后消失；2例术后出现移植后综合征，经对症处理后缓解。随访3-21个月，无移植物移位及器官缺血，假腔内见血栓形成。结论：腔内隔绝术治疗主动脉瘤创伤小．安全、恢复快，有着较好的近期效果。     

纤支镜介导注药并插管铱192腔内放射治疗气道肿瘤的疗效和安全性观察

目的为了探讨经纤支镜介导局部注射药物并插后装管进行腔内放射治疗气道肿瘤的临床应用价值。方法22例气道肿瘤患者均为中心型晚期肺癌，经纤支镜用专用注射针向腔内肿瘤局部注射5-Fu MMC和／或乙醇，并经纤支镜插后装管进行铱192腔内近距离放射治疗，比较治疗前后腔内肿瘤的消退，气道梗阻和肺不张的缓解及患者生存质量的改善情况及不良反应。结果治疗后15例肺不张，2例完全或部分张开，17例咯血均停止，病员自觉症状缓解率91％，全部病员生存质量提高，气管内肿瘤完全消退（CR）8例；消退在50％以上（PR）11例；有效率（CR＋PR）86．4％，无一例恶化。3例治疗后短期发热（38．5℃以下），1例胸痛不适，无严重并发症。结论经纤支镜注射药物加插管腔内放疗能有效地缓解气道梗阻，对不能手术的气道肿瘤引起的呼吸困难，肺不张，阻塞性肺炎及手术后残喘病，均有明显疗效，可改善患者生存质量，是综合治愈肺癌和姑息治疗的良好手段，其并发症少。     

下腔静脉闭塞段切除人工血管原位移植治疗布一加氏综合征18例报道

自1994年以来，采用下腔静脉闭塞或狭窄段切除人工血管原位移植治疗布一加氏综台征18例。手术适宜于短段闭塞型、伴有明显狭窄的隔膜型或局限性狭窄型而采用经皮球囊扩张失败或以往采用隔膜切除、心包片成形术而术后复发。经本治疗术后3-36个月随访，所有病例疗效满意，无一例复发。     

置管溶栓联合球囊扩张治疗布加氏综合征合并下腔静脉血栓

目的:探讨置管溶栓联合分期球囊扩张治疗布加氏综合征合并下腔静脉血栓的临床应用.方法:全组15例,均经彩色多普勒超声证实为布加综合征合并下腔静脉血栓,同时行CT检查7例,造影检查8例.先经股静脉入路置入溶栓导管于下腔静脉内,抗凝和溶栓治疗7～10 d,然后行病变段开通和分期球囊扩张治疗.结果:15例血栓基本消失或明显缩小,其中2例一期球囊扩张成功,13例行分期球囊扩张治疗.术后15例随访6～24个月,平均13.1个月.13例症状和体征完全消失,2例明显改善,无肺栓塞、血管撕裂、心包填塞等并发症发生.结论:置管溶栓联合分期球囊扩张治疗布加氏综合征合并下腔静脉血栓安全有效,具有临床可行性.     

Primary Leiomyosarcoma of the Inferior Vena Cava with Budd-Chiari Syndrome

A 44 year old Japanese woman with leiomyosarcoma of the inferior vena cava is reported. She presented with Budd Chiari syndrome and died of hepatic failure about 3 months after the onset of symptoms. The tumor arose from the middle segment of the inferior vena cava, occluded the inferior vena cava and projected into the right atrium. A total of 28 cases of Budd-Chiari syndrome due to primary leiomyosarcoma of the inferior vena cava are reviewed.     

Phlegmonous inflammation of gastrointestinal tract autopsy study of three cases.

Three cases of Phlegmonous inflammation of gastrointestinal tract detected at necropsy are described. Predisposing factors were seen in all three cases. These were chronic alcoholism with submissive hepatic necrosis (HbsAg and HbcAg positive) in Case 1, Indian Childhood cirrhosis in Case 2 and acute on chronic Budd Chiari syndrome in Case 3. In case 1 and 3 the inflammation was limited to the large intestine where as in Case 2 it was seen both in the stomach and large intestine. In two of the three cases blood culture grew Staphylococcus aureus (Case 1) and gram negative organisms (Case 2).     

Budd Chiari syndrome from myosarcoma of right atrium

A 50-year-old man presented with abdominal pain followed by marked liver and renal dysfunction. Although liver scan appearances were not diagnostic, at laparotomy a tense, enlarged liver was found with thrombosis of the inferior vena cava. Despite full supportive measures the patient died within a month of laparotomy. Subsequent necropsy confirmed the diagnosis of Budd Chiari syndrome and revealed the primary cause to be a myosarcoma of the right atrium, an occurrence hitherto unreported.     

血管腔内介入治疗下肢动脉硬化闭塞症40例

目的：探讨下肢动脉硬化闭塞症腔内治疗的临床经验。方法：采用血管腔内介入方法治疗下肢动脉硬化性闭塞症（ASO）患者40例（44条肢体）,包括髂动脉病变18例,其中单纯球囊扩张术（PTA）6例,内置支架术（PTA＋stenting）12例;股浅动脉病变13例,其中PTA5例,PTA＋stenting8例;腘动脉病变2例,行PTA治疗;膝下动脉病变7例,单纯deep球囊扩张,不放置支架。结果：本组无手术死亡。髂动脉18例手术全部获得成功;股浅动脉13例,11例获得成功,1例致血栓形成取栓治疗成功,1例介入失败转为人工血管转流手术;腘动脉2例获得成功;膝下动脉7例中5例获得成功。结论：血管腔内介入治疗下肢ASO,操作简单,短期效果良好,值得临床推广应用;     

Endomyocardial fibrosis as a cause of Budd–Chiari syndrome and fatal pulmonary embolism

A 24-year-old-man had right-sided heart failure of 3 months' duration. A Doppler echocardiogram revealed atrium and right ventricular enlargement, obliteration of the right ventricular apex, and a mass with an echolucent center measuring 20×21 mm in the right ventricular outlet. He died of pulmonary embolism. At autopsy, a huge organized thrombus obliterating the right ventricular apex passing through the tricuspid valve to the right atrium and then extending to the inferior vena cava up to the suprahepatic veins was seen. Histologically, an intense fibrotic thickening of the endomyocardium extending into the myocardium was observed. Cardiac thrombosis associated with endomyocardial fibrosis should be added to the list of causes of Budd–Chiari syndrome.     

Laparoscopic duodenojejunostomy for management of superior mesenteric artery syndrome: two cases report and a review of the literature

Superior mesenteric artery(SMA) syndrome is rare disorder, which is caused by a reduction in the aortomesenteric angle causing a duodenal obstruction. It is usually occurs after a period of weight loss, nausea, and vomiting by a partial obstruction of the third portion of the duodenum. If conservative management fails then a laparotomy with a duodenojejunostomy is indicated. Recently, a minimally invasive or laparoscopic approach to the retroperitoneum or duodenal detachment was introduced. Although the role of a laparoscopy in managing SMA syndrome is not clearly defined, a laparoscopic duodenojejunostomy may be an alternative approach to the surgical treatment of SMA syndrome cases. Two cases of superior mesenteric artery syndrome that were treated laparoscopically after medical therapy failure are described. The 4-port procedure was performed. A dilated bowel on the third portion of the duodenum was observed below the transverse mesocolon and to right of the superior mesenteric artery. A proximal loop of the jejunum was anastomosed to the duodenum using an endoscopic GIA stapler. The surgery time and hospital length of stay were acceptable. No complications were encountered in this study. A laparoscopic duodenojejunostomy is a feasible alternative option for treating SMA syndrome. It provides the benefits of being a definitive and minimally invasive surgical technique in a duodenal obstruction.     

Obstructive lesions of the lower urinary tract in the prune belly syndrome

The prune belly syndrome is a well-recognized entity consisting of deficient abdominal musculature, cryptorchid testes, and urinary tract abnormalities most consistent with an obstructive phenomenon. However, an obstructive lesion has not been consistently identified in previously reported cases. Retrospective review of the autopsy files of The Johns Hopkins Hospital, Baltimore, identified 18 cases of prune belly syndrome occurring since 1945. In 13 cases, obstructive lesions in the lower urinary tract had been described grossly. A single case, in which the lower urinary tract had been preserved intact and subsequently serially sectioned as 8-micron sections taken at every 120 micron, demonstrated an obstruction consisting of two overriding urethral lumens connected only by a narrow channel. Reconstruction of the sections suggested that this represented a "kink" in a short segment of the prostatic urethra just above the membranous urethra, leading to the obstructive changes of bladder hypertrophy and dilatation, hydroureter, hydronephrosis, and renal dysplasia seen higher in the urinary tract. The results suggest that examination of the lower urinary tract in cases of prune belly syndrome by serial histologic sectioning of the intact unopened prostatic and penile urethra may be required to demonstrate the presence or absence of an obstructive lesion.     

Controlled trial of methylprednisolone and chlorambucil in idiopathic membranous nephropathy

Sixty-seven adults with idiopathic membranous nephropathy and the nephrotic syndrome were randomly assigned to symptomatic treatment only or to a six-month course of methylprednisolone alternated with chlorambucil every other month. Patients were followed for one to seven years. At the end of follow-up (mean of 31.4 +/- 18.2 months for the treated group and 37.0 +/- 22.0 for the control group) 23 of 32 treated patients were in complete or partial remission, as compared with 9 of 30 control patients (P = 0.001). Twelve of the treated patients were in complete remission, as compared with only two of the controls. In the treated group there were no changes in renal function during follow-up, whereas in the control group the reciprocal of the plasma creatinin level, which is proportional to the creatinine clearance, decreased significantly (P = 0.00017) after two years of follow-up. Side effects were minimal in all treated patients except two, who were dropped from the study because of peptic ulcer and gastric intolerance to chlorambucil. We conclude that steroid and chlorambucil treatment for six months favors remission of the nephrotic syndrome in adults with idiopathic membranous nephropathy and can preserve renal function for at least some years.