肝硬化合并门静脉血栓致门静脉高压症治疗现状

肝硬化合并门静脉血栓(PVT)并不少见,PVT会进一步加重门静脉高压症,此时针对PVT的治疗可改善肝硬化患者的预后。目前的治疗方式包括抗凝治疗及经颈静脉肝内门体支架分流术(TIPS)。本文就此类患者的治疗现状作一综述。     

Melena-associated regional portal hypertension caused by splenic arteriovenous fistula

Regional portal hypertension is a rare cause of upper gastrointestinal bleeding. We reported an extremely rare case in which regional portal hypertension was associated with both the splenic arteriovenous fistula and chronic pancreatitis. In June 2010, our patient, a 41-year-old man, was admitted to a local hospital due to a sudden melena and dizziness without haematemesis and jaundice. The splenic arteriovenous fistula in this patient was successfully occluded through transcatheter arterial embolization. At the 12-mo follow-up, our patient was in good condition.     

脾静脉血栓形成导致门静脉高压症的诊断

脾静脉血栓形成（splenic vein thrombosis,SVT）引起的门静脉高压症（PH）,只局限于胃脾区,故称区域性PH,又称左侧或左区PH。SVT大多数有孤立性胃静脉曲张（isolated gastric varices,IGV）,少数伴食管静脉曲张（EV）即胃食管静脉曲张（GEV）,多数有脾大和上消化道出血。SVT行单纯脾切除后不再出血,GEV亦消失。SVT是一种完全可治愈的PH,临床少见,易误诊。现就SVT介绍如下。     

阵发性睡眠性血红蛋白尿症致门静脉高压症1例报告

门静脉高压症是指肝静脉压力梯度>10 mm Hg[1]。由于门静脉压力升高将导致腹腔脏器血管压力升高,形成侧支循环,极易发生并发症,严重影响患者预后。门静脉高压症大多由肝硬化引起,少数患者存在肝前性或肝后性因素。现报道1例由肝前性和肝后性因素共同导致的门静脉高压症。     

Portal venous stent placement for treatment of portal hypertension caused by benign main portal vein stenosis

AIM: To evaluate the value of endovascular stent in the treatment of portal hypertension caused by benign main portal vein stenosis. METHODS: Portal vein stents were implanted in six patients with benign main portal vein stenosis (inflammatory stenosis in three cases, postprocedure of liver transplantation in another three cases).Changes in portal vein pressure,portal vein patency, relative clinical symptoms, complications,and survival were evaluated. RESULTS: Six metallic stents were successfully placed across the portal vein stenotic or obstructive lesions in six patients. Mean portal venous pressure decreased significantly after stent implantation from (37.3±4.7) cm H2O to (18.0±1.9) cm H2O.The portal blood flow restored and the symptoms caused by portal hypertension were eliminated. There were no severe procedure-related complications. The patients were followed up for 1-48 mo. The portal vein remained patent during follow-up. All patients survived except for one patient who died of other complications of liver transplantation. CONCLUSION: Percutaneous portal vein stent placement for the treatment of portal hypertension caused by benign main portal vein stenosis is safe and effective.     

部分脾栓塞治疗肝硬化门脉高压及脾亢

目的 进一步观察部分脾栓塞治疗肝硬化门静脉高压及脾功能亢进（简称脾亢）的应用价值。方法 经下腔动脉插管对部分脾动脉用明胶海绵颗粒进行栓塞,观察其治疗作用。结果 本文２２例术后外周血白细胞和血小板计数均回升,其中１９例恢复至正常水平,３例部分缓解,门静脉及脾静脉回缩,食道静脉曲张程度改善,凝血酶原时间（ＰＴ）缩短,凝血酶原活动度（ＰＴＡ）增加,脑病发作次数减少,血氨水平下降,未出现严重并发症。结论     

Hemodynamic mechanisms of emerging portal hypertension caused by schistosomiasis in the hamster

A hamster model of schistosomiasis has provided the first opportunity to sequentially examine the early phases of the development of portal hypertension in a natural model of chronic liver disease. Groups of hamsters were infected with 50 cercariae of Schistosoma mansoni and underwent hemodynamic evaluation at intervals of 5, 8, 12 and 20 wk after infection. A progressive rise in intrahepatic resistance (from 4.0 +/- 0.4 to 8.4 +/- 1.0 mm Hg min.ml-1.gm liver weight [p less than 0.01]) appeared to play a major role in the initial stages of evolving portal hypertension. A gradual decline in portal blood flow (from 2.1 +/- 0.3 to 1.3 +/- 0.1 ml.min-1.gm-1 liver weight [p less than 0.01]) was only partially compensated for by an increase in hepatic arterial flow. Accordingly, by week 20, total hepatic blood flow decreased 23%. Liver weight that increased markedly between 5 and 12 wk after infection, as a result of the acute accumulation of obstructing granulomas, stabilized between wk 12 and 20, while a gradual but progressive rise in hepatic collagen content was seen. Portal pressure increased 75% during the study period. Chronic examination of this natural model should help define the pathogenesis of the complications of portal hypertension and contribute to the basis for effective intervention in this disease process.     

Stability of a rat model of prehepatic portal hypertension caused by partial ligation of the portal vein

AIM： To study the stability of portal hypertension （PHT） caused by partial ligation of the portal vein ligation （PVL） in a rat model.
METHODS： Thirty male adult Wistar rats were divided into two groups： 10 in Group Ⅰ received a sham operation; and 20 in Group Ⅱreceived partial PVL. Portal vein pressure （PVP） was measured at four time periods： before ligation, 2 wk, 6 wk and 10 wk postsurgery. Portal venography, blood sampling and liver and spleen pathological examinations were conducted at 10 wk after surgery.
RESULTS： The PVP was 9.15± 0.58 cmH2O before ligation, and increased to 17.32 ±0.63 cmH2O 2 wk after PVL. By repeat measurement of the PVP in each rat, it was shown to remain elevated for 10 wk. There were no significant differences in the pressure measurements at 2 wk, 6 wk and 10 wk. Varices were found mainly in the mesenteric vein 2 wk after PVL, which were more obvious later, while these manifestations were similar at week 6 and week 10. Portal venography demonstrated the varices and collaterals. There was no significant change in liver pathology. The volume of the spleen was enlarged 2-fold after ligation, and the sinus of the spleen was enlarged due to congestion. Significant sinus endothelial cell proliferation was observed, but no evidence of hypersplenia was found on hemogram and biochemical examination.
CONCLUSION： These findings suggest that a satisfactory prehepatic PHT rat model can be obtained by partial ligation of the portal vein, and this PHT rat model was stable for at least 10 wk.     

Correlation of Doppler ultrasound of the portal system with endoscopic changes caused by portal hypertension in cirrhotic patients

BACKGROUND: The portal hypertension in cirrhotic patients is the main cause of this illness complication, that are clinically translated to visible collateral circulation in the abdominal wall, ascites and esophageal varices. AIM: To evaluate if the portal system echodoppler is able to estimate the presence of esophageal varices, gastric varices and congestive gastropathy in patients with hepatic cirrhosis. PATIENTS AND METHODS: One hundred and eighty six patients of the gastroenterology and hepatology ambulatory of the Clinical Hospital of the Federal University of Paraná, Curitiba, PR, Brazil, had been selected for evaluation. Of those, 145 had completed all the stages of the evaluation and 133 had been enclosed in the final analysis. All had been submitted to high digestive endoscopy for evaluation of esophagogastric varices and congestive gastropathy and then to Doppler ultrasound of the portal system with study of the systolic peak speed of the portal vein, diameter of the portal and splenic vein and spleen size, presence of the umbilical vein recanalization and hepatofugal flow. RESULTS: The patients with esophagogastric varices had significant difference of the spleen size when compared to patients without these change. However, none of the Doppler ultrasound parameters showed good accuracy and specificity in this group of cirrhotic patients. Congestive gastropathy patients had their diagnosis predict with significant manner not only by the portal and splenic vein diameter but also by the spleen size. Similarly to that described above, they do not have a good accuracy and specificity. These evaluations were validated by the construction of ROC (Receiver Operating Characteristic) curves, whose areas below the curves had always been less than 0,8. CONCLUSION: There was not a good correlation of the Doppler ultrasound parameters of the portal system to the presence of the main endoscopic alterations (esophagogastric varices and congestive gastropathy) in patients with hepatic cirrhosis.     

Effect of a portal venous stent for gastrointestinal hemorrhage from jejunal varices caused by portal hypertension after pancreatoduodenectomy

We report herein the case of a 64-year-old man successfully treated by portal venous stent placement for repeated gastrointestinal bleeding associated with jejunal varices. He was admitted to our hospital with melena 8 years after having a pancreatoduodenectomy for carcinoma of the papilla of Vater. From portogram findings showing severe portal vein (PV) stenosis and dilated collaterals through the jejunal vein of the Roux-en-Y loop, jejunal varices resulting from PV stenosis were suspected as the cause of the melena. A metallic stent was placed in the PV following percutaneous transhepatic PV angioplasty. Although the cure of hemorrhagic jejunal varices caused by PV stenosis is difficult in patients who have undergone major abdominal surgery, patency of the stent in this patient has been maintained for 32 months without gastrointestinal hemorrhage. Metallic stent placement is recommended as a useful treatment for PV stenosis that is less invasive than open surgery.     

肝硬化门静脉高压性脾肿大并发脾功能亢进的特点及临床意义

肝硬化门静脉高压性脾肿大患者并发脾亢本身包含了脾肿大,因而,评价肝硬化门静脉高压症患者是否有脾亢主要依据是外周血细胞减少.然而肝硬化门静脉高压性脾肿大患者不一定都有外周血细胞减少,他是肝硬化门静脉高压性脾肿大的并发症,而不是其必然表现.对于该病的治疗,外科手术一方面可以消除巨脾和/或重度外周血细胞减少,还可以止血,因而绝大多数患者均可采用手术治疗,但对肝硬化门静脉高压性脾肿大无外周血细胞减少、无巨脾、无出血史的患者可采用非手术治疗.     

Analysis of factors related to early mortality in digestive hemorrhage caused by portal hypertension

In order to determine immediate criteria of prognosis for patients with portal hypertension hospitalized for digestive hemorrhage, in an intensive care unit, 18 variables were recorded during the 24 hours following admission in 65 patients. Data related to death were age, ascites, hepatic encephalopathy, shock, active hemorrhage, acute pneumonia, decrease in prothrombin time, use of esophageal balloon tamponade, use of mechanical ventilation, number of red blood cell units transfused. Discriminant analysis yielded a linear combination of 4 variables which best separated survivors from non survivors with the following equation: F = 0.330 X hepatic encephalopathy + 0.433 X shock + 0.226 X active hemorrhage + 0.0097 X age - 0.396. The threshold decision of the hemorrhage prognosis index (HPI) was F = 0.57; 80 p 100 of all patients were correctly classified. In order to be validated, HPI was compared with a general (SAPS) and specific (Pugh's classification) scoring system, in a prospective study of 57 episodes of digestive hemorrhage. In this study, sensitivity was better with HPI than with SAPS (0.70 versus 0.45), specificity was higher with HPI than with Pugh's classification (0.86 versus 0.70). Percentage of correctly classified patients was higher using HPI (81 p. 100) than SAPS (77 p. 100) and Pugh's classification (68 p. 100). We suggest that the HPI, determined with 4 easily defined and recorded variables should be used prospectively to compare efficacy of different treatments.     

Transjugular intrahepatic portosystemic shunt as a treatment for protein-losing enteropathy caused by portal hypertension

A case of a 41-year-old man with diarrhea, hypoalbuminemia, and cryptogenic cirrhosis with features of portal hypertension is described. Protein-losing enteropathy was confirmed by analysis of whole-gut lavage fluid, and intestinal inflammation and infection were excluded. Distal duodenal biopsy specimens showed evidence of edematous villi with prominent submucosal vascular and lymphatic vessels. A transjugular intrahepatic portosystemic stent-shunt was inserted, resulting in resolution of both his diarrhea and elevated whole gut lavage fluid protein concentrations. His symptoms recurred and then again improved after shunt thrombosis and parallel shunt placement, respectively. Histological improvement of the villous edema was also noted. This is the first recorded case of protein-losing enteropathy caused by portal hypertension confirmed by successful treatment with transjugular intrahepatic portosystemic stent-shunt. (Gastroenterology 1996 Dec;111(6):1679-82)     

Non-cirrhotic portal hypertension versus idiopathic portal hypertension

Portal hypertension occurs in a number of disorders other than cirrhosis and they are collectively called non-cirrhotic portal hypertension (NCPH). The common causes of NCPH include idiopathic portal hypertension (IPH), non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous thrombosis (EHPVT). Other causes include schistosomiasis, hepatic venous outflow tract obstruction, veno-occlusive disease and congenital hepatic fibrosis. Patients with IPH and EHPVT present with upper gastrointestinal bleeding, splenomegaly, ascites after gastrointestinal bleeding, features of hypersplenism, growth retardation and jaundice due to portal biliopathy. The diagnosis is usually made by abdominal ultrasound, upper gastrointestinal endoscopy, normal liver function tests and normal liver histology. Variceal bleeding in NCPH has lower mortality as compared with cirrhosis because of better liver functions in NCPH. Treatment for NCPH includes primary prophylaxis for variceal bleeding and prevention of repeat bleeding using drugs like beta-blockers, endoscopic sclerotherapy and endoscopic band ligation of varices. In patients with uncontrolled variceal bleeding or symptomatic hypersplenism, porto-systemic shunt surgery or splenectomy are required.     

门静脉闭锁致门脉高压相关性肺动脉高压1例并文献复习

目的:复习罕见门静脉解剖异常导致门脉高压性肺动脉高压(PPHTN)的相关文献,了解这类患者临床特点。方法:回顾分析1例成人门静脉闭锁导致PPHTN患者的临床经过和特点,并以"门静脉闭锁"、"门脉高压性肺动脉高压"为检索词,在万方数据库和中国全文期刊数据库中进行检索,以"atresia of portal vein"、"pulmonary hypertension"为检索词在pubmed全文数据库中进行检索。结果:患者女性,71岁,3年前因反复"肝性脑病"发现门静脉主干闭锁,脾静脉纤细;肠系膜上静脉-左肾静脉门体分流形成。胃底周围见静脉曲张,门腔静脉自发分流形成可能。2年6个月前出现双下肢水肿,1月前活动耐力明显下降,伴夜间阵发性呼吸困难,伴腹胀。UCG示下腔静脉增宽,心室呈"D"型影,s PAP84mm Hg(1mm Hg=0.133kPa)。因患者入院后出现院内感染及急性肾损伤,未能行右心导管检查,结合病史及检查结果,除外肺实质疾病、肺血栓栓塞症、结缔组织疾病及其他可能导致肺动脉高压(PAH)的药物使用或毒物接触史,同时确定患者存在门脉高压导致的胃底静脉曲张,诊断患者为非肝病性门静脉解剖异常导致的PPHTN。共检索中文文献2篇,外文文献24篇,保留较为详细病例资料文献15篇,涉及病例25例。结论:非肝病性门脉高压所形成的门体分流可以导致高动力性PAH,先天性门体分流者多见,继发于成人门静脉闭锁者罕见,早期识别随访,及时针对PAH进行干预治疗,维护右心功能,可能会延迟右心衰竭发生的时间,改善预后。