胰源性胃食管静脉曲张28例分析

胰源性胃底或食管下段静脉曲张是区域性门脉高压症表现之一,该病发病率低,其原发疾病起病隐匿,故早期诊断较为困难.在常规胃镜发现胃底静脉曲张者,联合B超等影像学检查,对本症的诊断有确诊价值.现就28例胰源性区域性门静脉高压症做一回顾性分析.     

区域性门静脉高压症的临床特征及诊治

区域性门静脉高压症 (RPH )临床较少见 ,其属于肝外型门静脉高压症的特殊类型 ,以往称左侧肝外型门静脉高压症、胰源性门静脉高压症、胃脾区门静脉高压症、节段性门静脉高压症及 RPH,现统称为 RPH。国内外公开医学杂志 1980～ 1999年共报道 75例病因明确的 RPH,1995～ 1999年笔者收治 RPH患者 11例。86例患者中胰源性 6 5例 (以慢性胰腺炎、胰腺癌及胰腺囊肿引起者居多 ) ,脾源性 12例 (脾动静脉瘘引起者 6例 ) ,其它 9例 ,多为术中和术后诊断 ,部分通过经脾间接门静脉造影、彩色多普勒超声等方法术前诊断。86例中有手术记录者 6 6例 …     

胰源性区域性门脉高压症7例临床分析

区域性门脉高压症亦称为左侧门脉高压、局限性门脉高压等,占肝外型门脉高压症的5%,是惟一可治愈的门脉高压症.根据病因,区域性门脉高压可分为胰源性、脾源性和腹膜后源性三类,其中以胰源性最常见.     

胰源性门静脉高压症3例报告

胰源性门静脉高压症３例报告江苏省常州市第二人民医院（２１３００３）王凤洲肝外型门静脉高压症其病变的位置在门静脉主于或脾静脉中，与肝病无关。临床表现类似门脉性肝硬化而常被误诊，但其治疗与预后不同，应引起警惕和重视。本文报道３例胰源性门静脉高压症，并予讨...     

胰源性区域性门脉高压症——附14例报告

本文报告了14例胰源性区域性门脉高压症。本病为肝外型门脉高压症中罕见的一种，对未发现肝脏疾病，而有胃底，食管静脉曲张，脾肿大的病人，应考虑本病。门静脉造影可以确立诊断，经皮脾穿刺门静脉造影是确诊本症的三种简单可行的方法。本病可经脾切除治愈。     

胰源性门静脉高压的诊断与处理

胰源性门静脉高压(PPH)是由胰腺疾病及其并发症导致的脾静脉阻塞、血液回流障碍而引起的区域性门静脉高压,以消化道出血为主要临床表现,预后凶险,应充分了解其发病机制和治疗方法。对于伴有脾肿大但肝功能正常的上消化道出血患者,应考虑PPH的诊断。PPH治疗的关键是处理胰腺原发疾病,内镜介入治疗是可选择的干预措施,脾切除术是最根本、最有效的治疗方法。     

胰源性门脉高压症的诊断和治疗

胰腺疾病是区域性门脉高压症最常见的病因。孤立性胃底静脉曲张、肝功能正常、脾肿大是胰源性门脉高压最典型的临床表现。胰源性门脉高压症并发胃底静脉曲张破裂出血最有效的治疗是脾切除术,但预后主要取决于胰腺原发疾病。     

特发性非肝硬化性门静脉高压症的研究进展

特发性非肝硬化性门静脉高压是一种原因不明的门静脉高压症,无明显肝硬化特征;本病的发病机制尚不清楚,目前认为慢性感染、免疫、毒物接触、凝血机制障碍等均可能参与发病。临床主要表现为门静脉高压征象,如食管胃底静脉曲张或破裂出血、显著脾肿大,而肝功能基本正常,腹水及肝性脑病少见;病理改变主要表现为门静脉闭塞性病变,但无肝硬化改变。临床诊断主要是排他性诊断,有门静脉高压的临床证据,组织病理学检查除外肝硬化,排除引起肝硬化的慢性肝病以及引起非肝硬化性门静脉高压的其他临床疾病即可考虑本病。有关特发性非肝硬化性门静脉高压症研究较少,推荐按照肝硬化所致门静脉高压指南进行治疗。预后主要取决于门静脉高压的严重程度及其并发症的处理,一般优于肝硬化并食管胃底静脉曲张破裂出血。     

抗凝药物在急性胰腺炎相关内脏静脉血栓中的应用进展

AP相关内脏静脉血栓是指在AP基础上发生门静脉、脾静脉和(或)肠系膜静脉等血管静脉血栓。随着辅助检查手段多样化、影像学检查技术的提高和临床医师对该病认识程度的加深, 越来越多的AP患者特别是SAP患者被发现合并内脏静脉血栓, 甚至胰源性门静脉高压症。AP相关内脏静脉血栓的治疗主要包括治疗原发病、抗凝药物的应用及消化道出血的治疗。本文就抗凝药物在AP相关内脏静脉血栓中的应用进行综述。     

腹腔广泛淋巴结结核导致区域性门脉高压一例

区域性门脉高压是一种特殊类型的肝前性门脉高压症，其病因主要为胰源性、脾源性及腹膜后源性，临床相对少见。而腹腔广泛淋巴结结核导致区域性门脉高压在临床中更为罕见，在临床中易漏诊、误诊，故本文以此病例的诊疗过程，结合文献复习作一报道。     

胰源性区域性门脉高压症合并上消化道出血的诊治分析

目的探讨胰源性区域性门脉高压症合并上消化道出血的诊断和治疗方法。方法回顾分析我院2000年1月至2011年2月收治的14例胰源性区域性门脉高压症合并上消化道出血患者的诊疗措施和随访资料。结果14例患者中胰体尾占位6例,胰腺假性囊肿4例,慢性胰腺炎4例。均有呕血或（和）黑便史,其中4例有失血性休克表现。所有患者均无肝硬化、腹水及肝功能异常等表现。胃镜和超声胃镜提示14例患者均有胃底静脉曲张,2例同时合并食管下段静脉曲张。8例患者有脾肿大和脾功能亢进的表现。14例患者均采用手术治疗。9例患者获得随访,曲张静脉明显改善或消失,随访5月～8年均无出血复发。结论孤立性胃底静脉曲张、脾肿大和脾功能亢进、无肝硬化和肝功能正常以及胰腺疾病病史是诊断胰源性区域性门脉高压症的基本要点。该疾病可通过脾切除术或联合胃底周围血管离断术治愈,应同时重视对胰腺原发疾病的治疗。     

Une cause inhabituelle d’hypertension portale segmentaire: un phéochromocytome gauche

Segmental or sinistral portal hypertension is a rare form of extrahepatic portal hypertension. It results from thrombosis or compression of the splenic vein or the union of superior mesenteric and splenic vein which are usually due to pancreatic disease. The originality of our observation comes from the aetiology of segmental portal hypertension which is a left pheochromocytoma. It is to our knowledge the second case in literature.     

胰腺疾病相关性门脉高压症59例临床分析

目的探讨胰腺疾病相关性门脉高压症的临床特点及治疗。方法选择我院1986年1月至2005年4月收治的胰腺疾病相关性门脉高压症患者，回顾性分析其一般资料、受累静脉、临床表现、实验室和影像检查、治疗和结局。结果我院19年共收治本病59例，占同期门脉高压症的4．0％。常见基础胰腺疾病依次为慢性胰腺炎（21例，35．6％）、胰腺癌（20例，33．9％）、急性胰腺炎（8例，13．6％）和胰腺囊肿（3例，5．1％）。40例患者有明确的受累静脉，其中脾静脉阻塞27例（67．5％）、门静脉阻塞16例（40．0％）。脾脏肿大48例（81．4％），为轻、中度肿大，脾功能亢进31例（52．5％），程度较轻，以白细胞减少为主。45例患者（76．3％）有胃、食管静脉曲张（孤立性胃静脉曲张35例），19例有破裂出血（32．2％）。药物治疗可控制急性出血，但不能预防再出血。18例行脾切除术，主要指征是反复发生的消化道出血，术后患者均未再出血（随访8个月～9年）。结论胰腺疾病可累及门静脉主干及其属枝，导致广泛性或区域性门脉高压症。药物治疗可有效控制急性曲张静脉破裂出血，而手术可能是防止再出血的主要措施。     

Sinistral portal hypertension. A case report

Sinistral portal hypertension is a clinical syndrome of gastric variceal hemorrhage in the setting of splenic vein thrombosis due to a primary pancreatic pathology. The distinguishing features from other forms of portal hypertension are preserved liver function and a patent extrahepatic portal vein. The important causes include acute and chronic pancreatitis, pancreatic pseudocysts and pancreatic carcinomas. Benign pancreatic neoplasms only rarely cause sinistral portal hypertension. Splenic vein thrombosis complicates 7-20% of patients having pancreatitis or a pancreatic pseudocyst; however, bleeding occurs in only approximately 5% of patients. The diagnosis of sinistral portal hypertension is achieved by a combination of gastroscopy, liver function tests, ultrasound examination (with Doppler) and/or contrast-enhanced CT scan of the abdomen. A mere demonstration of sinistral portal hypertension does not warrant intervention. An expectant management is justifiable in asymptomatic patients with pancreatitis. However, concomitant splenectomy may be considered in patients undergoing operative treatment of symptomatic chronic pancreatitis if sinistral portal hypertension and gastroesophageal varices are present. In patients presenting with gastric variceal hemorrhage, splenectomy (with treatment for the primary pancreatic pathology, e.g. distal pancreatectomy) is curative with excellent long term results.     

左侧门静脉高压的临床和内镜特征

目的 探讨左侧门静脉高压的临床特点和内镜特征。方法 对手术证实的8例左侧门静脉高压患者的临床资料进行回顾性分析。结果 左侧门静脉高压的临床表现主要为呕血、黑便和脾大、脾功能亢进，患者具有胰腺疾病的特点，而无肝脏疾病的表现和检查异常。内镜下以孤立性胃底静脉曲张为主，占62．5％；食管、胃底静脉同时曲张占37．5％。术前易误诊为血液系统疾病和肝硬化门静脉高压。结论 胰腺疾病可致门静脉高压，孤立性胃底静脉曲张是左侧门静脉高压的的特征性表现之一。     

胰源性门静脉高压症合并上消化道出血的治疗策略

胰源性门静脉高压症(PSPH)的发病机制和肝硬化性门静脉高压症完全不同,是唯一可被治愈的门静脉高压症。PSPH合并胃曲张静脉出血是相对少见的临床表现,但起病凶险,病情复杂,不恰当的治疗决策会延误病情,造成患者死亡。因此探索最优化的PSPH合并上消化道出血救治策略很有必要。脾切除是治疗PSPH继发消化道出血的确定性术式,同时实施针对胰腺原发疾病的术式。对于不适合手术的晚期肿瘤或手术高风险患者,优先选择疗效确切的脾动脉钢圈栓塞止血后再评估是否二期手术。PSPH出血的治疗须个体化决策,有必要开展多中心研究以获得最优化的PSPH出血治疗策略。     

胰源性门脉高压症44例临床分析

目的探讨胰源性门脉高压症(Pancreatogenicportalhypertension)的病因、临床特点及防治措施。方法回顾性分析我院1998年~2005年收治的44例胰源性门脉高压患者的临床资料,并结合1998~2006年中文科技期刊全文数据库累及报道的胰源性门脉高压症164例患者的临床资料进行综合分析。结果208例胰源性门脉高压症患者中脾大者占98.1%,胃底静脉曲张者占87.5%,伴上消化道出血者占80.2%,慢性胰腺炎、胰腺假性囊肿、胰腺肿瘤共占总数的93.1%。结论上述胰腺疾病容易并发胰源性门脉高压症,提高对本病的认识具有重要的临床意义;病变在胰尾的胰源性门脉高压,脾脏切除术是治愈本病的手段。     

Extrahepatic portal venous obstruction of different pathogenesis in pancreatic diseases: reports of 4 autopsy cases with chronic pancreatitis and pancreatic carcinoma

Four autopsy cases of extrahepatic portal venous obstruction associated with pancreatic diseases, 1 case of pancreatitis and 3 cases of pancreatic carcinoma, are presented. The pathogenesis of portal obstruction was different in each case; old thrombosis with recanalization due to chronic pancreatitis with pseudocysts formation in 1 case, fresh thrombosis due to intraportal venous catheterization for pancreatic carcinoma in 1 case, fresh thrombosis probably due to pancreatitis accompanying pancreatic carcinoma in 1 case, and direct invasion of pancreatic carcinoma into the portal vein in the remaining 1 case. Morphologic evidence for portal hypertension was present in each case. In the pancreatitis case and one pancreatic carcinoma case with portal tumor invasion, both of which had chronic portal obstruction, there were many thin-walled vascular channels (cavernous transformation) around the occluded portal vein. Their endothelia were positive for factor VIII-related antigen and Ulex europaeus lectin I, implying that these vessels were hepatopetal blood vascular collaterals. It was shown that pancreatic diseases resulted in portal venous obstruction by several different mechanisms and chronic portal obstruction in pancreatic diseases led to the formation of hepatoperal blood vascular collaterals.     

INTRAHEPATIC SEQUESTERED SEGMENT OF I.IVKR PRESENTING AS FOCAL FATTY CHANGE

Focal fatty change of the liver is a lesion that is often discovered on imaging studies und must he distinguished from space-occupying lesions. The pathogenesis is unknown. W'e report a lesion of focal fatty changes in which the portal supply and biliary drainage were anomalous so that the lesion represents sequestered liver tissue, Because insulin favors the development of steatosis, the pathogenesis of focal fatty change could he explained if the aber-rant portal snpph contained more insulin than the main portal vein, as would occur if the portal supply arose from pancreatic veins via the parabiliary venous plexus of Couinuud. Further-more, focal fatty sparing could be explained if the spared segment was supplied by veins draining from the stomach that carry blood with lower insulin levels than the main portal vein.     

New concepts in the pathogenesis of portal hypertension: hepatic wounding and stellate cell contractility

The pathogenesis of portal hypertension is multifactorial, and appears to result from interplay between fixed and dynamically modulable elements; the stellate cell is a newly recognized example of the latter. This perisinusoidal, pericyte-like cell has contractile features that are most prominent after liver injury, concomitant with their activation. These data imply an exaggerated contractile phenotype in the cirrhotic liver. This cell may contribute to increased intrahepatic portal hypertension via perisinusoidal constriction of the sinusoid or by contraction of fibrous extracellular matrix rich in type I collagen with concomitant disruption of lobular architecture. Endothelins and NO play a major role in the modulation of stellate cell contractility, and are therefore important in the pathogenesis of intrahepatic portal hypertension. These new data provide potential areas for therapeutic intervention in this clinical entity.