长期控制肝硬化门静脉高压症的策略

一、肝硬化门静脉高压症的自然史 门静脉高压症是指各种原因引起门静脉系统血流受阻和(或)血流量增加,导致门静脉及其属支血管内压力升高,伴侧支循环形成的一组临床综合征,包括腹水、食管胃底静脉曲张出血(EVB)、肝性脑病、肝肾综合征、肝肺综合征、门静脉高压性胃病等[1].     

内皮素在门脉高压症中的作用研究进展

在肝硬化门脉高压症的发生、发展过程中,常伴有肝星状细胞(hepalic stellate cell,HSC)的活化和多种血管因子的改变。活化的HSC具有收缩性,能调节肝窦血流,并产生大量细胞外基质(extracellular matrix,ECM),是肝硬化门脉高压症形成的中心环节。而内皮素(endothelin,ET)是调节HSC最重要的血管活性物质之一,ET与HSC的相互作用由于     

缬沙坦治疗肝硬化门静脉高压症的临床疗效

近年研究结果发现,内皮素(ET)、一氧化氮(NO)、肿瘤坏死因子(T N F)α等血管活性物质和转化生长因子(TGF)β1等细胞因子在肝硬化门静脉高压症(PHT)发病中发挥着关键作用.本研究观察缬沙坦对肝硬化PHT患者血管活性物质ET、NO、TNF α和肝纤维化指标TGF β 1、甘胆酸(CG)、透明质酸(HA)的影响,并通过彩色多普勒超声仪测量门静脉内径(DPV)和门静脉流速(VPV)的改变,进一步探讨缬沙坦对肝硬化PHT的治疗作用.     

肝内胆管结石合并门静脉高压症的治疗

肝内胆管结石与门静脉高压症都是临床处理上较为复杂的问题。两者合并更增添了处理上的难度。肝内胆管结石合并门静脉高压症的发生率约占肝内胆管结石就诊人数的10％左右,临床上以胆石胆道梗阻和肝硬变门静脉高压为特点。有关肝内胆管结石合并门静脉高压症的治疗的报道甚少,且尚无统一定论。一旦处理不当。常可产生严重后果,轻则影响治疗效果,加重患者的痛苦,重则可能危及患者的生命。肝内胆管结石合并门静脉高压症总的治疗原则是控制胆道感染,解除胆道梗阻,降低门静脉压力,改善肝脏功能。但临床上由于其复杂性,难以决定是先处理胆道疾患还是先处理门静脉高压症,是一次手术同时处理二者还是行分期手术治疗等。举棋不定则有可能延误治疗甚至危及患者的生命。因此,应在充分认识本病治疗中的问题及全面评估患者情况的前提下。选择适当的手术时机和手术方式。     

脾脏在特发性门静脉高压症形成中的作用研究

转发性门静脉高压症（IPH）是病因不明的疾病，本研究的目的是探讨脾大的发生机制及脾脏血流量在IPH形成中的作用。     

经颈静脉肝内门体分流术治疗门静脉高压症

经颈静脉肝内门体分流术(TIPS)是一项专门治疗门静脉高压症的介入治疗新技术.该技术于1969年首先报道,经过30多年的探索与发展,已日臻成熟,现已被广泛用于伴有食管胃底静脉曲张出血,顽固性腹水、Budd-Chiari综合征(BCS)等的门静脉高压症的治疗,并取得了显著疗效.     

卡维地洛治疗肝硬化门静脉高压症的作用机制及应用现状

肝硬化门静脉高压症的形成依赖于肝内血管阻力的增加及血流高动力状态。卡维地洛因其特有的降低肝内血管阻力作用,理论上具有更好的降低门静脉高压的效果。归纳了卡维地洛降低门静脉高压已知及可能的机制,回顾了卡维地洛治疗肝硬化门静脉高压症的最新研究。认为卡维地洛有望成为治疗肝硬化门静脉高压症的核心药物,而他汀类药物可能成为其最佳搭档。     

门静脉高压症对胆石症手术的影响与治疗体会

目的探讨门静脉高压症（PHT）合并胆石症的合理处理方式。方法回顾分析1999年12月至2009年12月64例PHT合并胆石症患者不同方式手术治疗的结果。根据手术方式不同将64例患者分为三组。A组：仅行胆道手术（n=20）;B组：在行PHT手术时对胆囊或胆道结石未作处理（n=24）;C组：在行PHT手术的同时行胆道手术（n=20）。结果病死率：A组10%,B组4.17%,C组25%。并发症：A组20%,B组16.67%,C组50%。结论 PHT合并胆石症无论仅行胆道手术或同期行PHT手术和胆道手术,手术病死率和术后并发症均显著增加,尤以同期手术为明显。根据患者情况选择正确的手术方式,可有效降低手术风险。     

特发性门静脉高压症3例

本院2009年11月-2010年4月收治特发性门静脉高压症(IPH)患者3例,现报道如下. 一、病例资料 A患者,男性,13岁,因发现脾大2个月于2009年入院.既往病史提示:患者于家中接生,剪断脐带时未严格消毒;B患者,男性,19岁,因发现脾大3年于2009年11月入院,2008年2月及2009年9月曾发生上消化道出血,表现为呕血、便血.     

非肝硬化性门静脉高压症研究进展

门静脉高压的定义为门静脉和下腔静脉压力梯度大于5 mmHg.根据门静脉系统阻塞部位,可分为肝前性、肝性和肝后性.根据肝血窦累及情况,肝性门静脉高压又可以细分为窦前性、窦性和窦后性.继发于肝硬化的窦性门静脉高压是最常见的类型,表现为肝静脉压力梯度( HVP G )升高,即肝静脉楔入压与肝静脉游离压的差值升高[1,2].有...     

New insights into the pathobiology of portal hypertension

Portal Hypertension is a frequent complication of cirrhosis and causes significant morbidity and mortality. Increased intrahepatic resistance is the primary factor but portal hypertension is also associated with changes in systemic and porto-sytemic collateral circulation. Cirrhosis is a state of vasoregulatory imbalance with excess vasoconstrictors and less vasodilators in hepatic circulation and the reverse is true for systemic circulation. Multiple pathophysiologic mechanisms including endothelial dysfunction, sinusoidal remodeling and angiogenesis are involved in increasing resistance in hepatic vascular bed. Current evidence suggests that these changes in vasoreactivity contribute to a significant proportion of intrahepatic vascular resistance and that they are reversible, providing an attractive target for therapeutic intervention.     

重视非肝硬化性门静脉高压的诊断和治疗

非肝硬化性门静脉高压(NCPH)是指除肝硬化外多种疾病导致的门静脉高压症。NCPH常见的原因有门静脉血栓形成、先天性肝纤维化和特发性门静脉高压等。这组疾病的主要特点是门静脉高压相关的表现突出,而肝功能储备相对较好,鉴别该类疾病需要临床,影像学和病理学的深入检查。通过适当的内外科治疗,多数患者预后较好。     

肝硬化门静脉高压脾切除术后门静脉血栓形成的研究进展

门静脉血栓形成(PVT)是指门静脉或其分支、脾静脉和肠系膜上静脉内形成的血栓。PVT是门静脉高压症脾切除术后的一种常见并发症,往往可能会导致肝损伤、上消化道出血、肝昏迷,甚至是缺血性肠坏死。因而,早期发现PVT并进行有效干预,对降低PVT患者的病死率有十分重要的意义。对肝硬化脾切除术后PVT危险因素和治疗进行了综述,指出PVT应及早进行有效干预。     

Haemodynamic effects of enalaprilat on portal hypertension in patients with HBsAg-positive cirrhosis

Abstract It has been suggested that enalaprilat inhibits the renin-angiotensin-aldosterone system in plasma and tissue; it may therefore reduce portal vascular pressure owing to secondary hyperaldosteronism in patients with liver cirrhosis. In order to evaluate this concept, 20 patients with hepatitis B surface antigen (HBsAg)-positive liver cirrhosis and portal hypertension received an intravenous infusion of 2.5 mg of enalaprilat. Wedged hepatic venous pressure, free hepatic venous pressure and cardiac index were measured before, immediately after, and then 15 min, 30 min and 1 h after intravenous enalaprilat infusion. The mean pressure gradient between wedged hepatic venous pressure and free hepatic venous pressure was significantly decreased, by 13% immediately after, 18% at 15 min, 23% at 30 min and 13% at 1 h after infusion of enalaprilat. Thirteen patients experienced a decrease of hepatic venous pressure gradient (HVPG) greater than 5 mmHg, another three 3-5 mmHg and the remaining four patients exhibited no significant change in HVPG. Systemic haemodynamic indices, including pulmonary arterial pressure, pulmonary capillary wedge pressure and central venous pressure, decreased significantly at 15 and 30 min after enalaprilat infusion ( P < 0.01). Liver function, renal function and blood routine before and after enalaprilat infusion showed no significant changes. There were no adverse effects during or after enalaprilat infusion. We conclude that enalaprilat infusion can quickly and safely reduce the hepatic venous pressure gradient in patients with HBsAg-positive cirrhosis.     

Modern management of portal hypertension

The development of portal hypertension plays a major role in the pathogenesis of many of the complications of chronic liver disease. In developed countries, most patients with portal hypertension have cirrhosis, and, in this condition, portal pressure is elevated as a result of both an increase in hepatic resistance to portal perfusion and increased mesenteric blood flow. Bleeding from oesophageal varices is a major cause of mortality in patients with significant portal hypertension. This review concentrates on the recognition, prevention and acute management of this life threatening complication of cirrhosis.     

肝血管性疾病门静脉高压的介入性诊断与治疗

目的探讨介入方法对肝血管性疾病门静脉高压的诊断和治疗效果。方法 6例因慢性肝病史临床初诊疑似肝硬化门静脉高压的患者,增强CT及MRI检查发现门静脉显影时间及形态异常。经数字减影血管造影（DSA）确诊4例为肝动脉-门静脉畸形,2例为布-加综合征肝静脉狭窄。造影后分别予以选择性肝动脉栓塞和肝静脉扩张成形治疗。结果所有介入性治疗操作均获得技术性成功,无并发症发生。治疗后血管造影显示肝动脉畸形血管完全消失或大部分已不显影,肝静脉狭窄解除。所有患者介入治疗后近期临床门静脉高压症状明显改善或消失。1例弥漫性肝动脉-门静脉瘘患者2次栓塞后6个月发现门静脉主干血栓形成,行溶栓和经颈静脉肝内门体分流术（TIPS）治疗后症状消失;1例布-加综合征肝静脉狭窄于3年后复发,在外院植入肝静脉支架。结论应用介入方法对肝血管性疾病导致的门静脉高压进行确诊和治疗确切有效,是安全而微创性的诊疗手段     

胰源性与特发性门脉高压症的临床特点分析

目的探讨PPH与IPH的临床特点,加深对二者的认识,提高临床医师的诊治水平。方法对18例PPH与36例IPH患者的临床资料作一回顾分析。结果二者的肝脏形态、功能正常,病毒学指标阴性,超声检查脾静脉迂曲扩张,脾肿大;PPH患者超声检查门静脉正常,胰腺可见炎症、肿瘤、囊肿等表现;IPH患者门静脉及肠系膜上静脉迂曲扩张,但胰腺方面无异常。IPH患者汇管区纤维组织增生和炎性细胞浸润但无肝硬化改变而PPH患者肝脏组织学正常。结论临床中发现肝脏形态、功能正常,病毒学指标阴性,以门脉高压为主要表现而无肝硬化改变的患者,应考虑IPH与PPH的可能。进一步行超声检查门脉系统及胰腺情况,可进一步区分二者。     

非肝硬化性门静脉高压症的研究现状

肝硬化是门静脉高压的最常见原因,但仍有约20%的门静脉高压继发于非肝硬化因素,称为非肝硬化性门静脉高压症(NCPH),在发展中国家发病率较高。NCPH是一组异源性的肝脏血管疾病,临床上多见的是特发性门静脉高压(IPH)、肝外门静脉血管阻塞(EHPVO),以及布加综合征、先天性肝纤维化和结节再生性增生等少见病。此类患者常常具有门静脉高压的证据,如反复发生的静脉曲张出血和脾脏肿大,但肝功能保存尚好。目前尚无诊断NCPH的统一标准,对其诊断仍是一个挑战。临床上往往采用排除性诊断,必要时可行肝穿刺活组织检查来确诊。介绍了IPH和EHPVO的发病机制、病理表现、诊断方法及治疗策略的选择,若能有效控制上消化道出血,NCPH被认为是预后相对良好的一类疾病。     

Long-term favourable outcome of portal hypertension complicating primary systemic amyloidosis

Abstract: An unusual case of systemic amyloidosis complicated by portal hypertension is reported. In this condition, portal hypertension is a rare event associated with poor prognosis. In our patient, severe presymptomatic sinusoidal portal hypertension was demonstrated by hepatic vein catheterization and coincided with abundant perisinusoidal amyloid infiltration. Despite these features and the absence of objective response to 20 courses of melphalan and prednisone, the patient was still in good clinical condition 4 years after initial diagnosis. This observation suggests that in primary amyloidosis, the incidence of sinusoidal portal hypertension might be underestimated and also that it may be associated with a relatively good prognosis.