Endoscopically proven case of rapid esophagogastric variceal progression and rupture as a result of portal hypertension with liver sarcoidosis

Sarcoidosis is a multi‐systemic disease of unknown etiology that results in the development of non‐caseating epithelioid granulomas. The liver is the third most frequently involved organ after the lymph nodes and the lungs. Most cases of liver sarcoidosis do not present with symptoms and involve minimal liver dysfunction, but some cases display progression to portal hypertension and liver cirrhosis, and finally to liver failure. The mechanism and the risk of progression in liver sarcoidosis are still unknown because of the diagnostic difficulty associated with this condition, and because follow‐up examinations can only be done in an invasive manner. Here, we present an informative case of liver sarcoidosis with rapid progression of esophagogastric varices. Four months prior to the definitive diagnosis, no signs of varices were observed on endoscopy, and developmentof esophagogastric varices, rapid progression, and eventual rupture occurred in a short period of time. A liver biopsy, carried out after endoscopic sclerotherapy, revealed that granulomas primarily affected the portal area without fibrotic and cirrhotic changes, which is considered a primary cause of portal hypertension and esophagogastric varices. Following the liver biopsy, the patient was given systemic steroids and is currently receiving outpatient care. Thus, we should consider the possibility that liver sarcoidosis, even in the absence of cirrhotic changes, can cause serious events such as esophagogastric variceal rupture following rapid progression as a result of portal hypertension.     

肝结节病出现重度黄疸致肝硬化1例

一、临床资料1．病历摘要：患者女性,68岁,主诉间断乏力、腹胀、尿黄2年,加重半月于2006年5月第2次住院。患者2年前开始有乏力、腹胀、尿黄等不适症状,肝功能轻度异常,以“慢性肝炎”第1次住院,经保肝对症治疗20 d,肝功能好转,病原学检查阴性。其间出现咳嗽、气短症状,无发热,胸片检查提示肺门淋巴结肿大,双肺野可见点片状阴影,[第一段]     

肝硬化门静脉血栓形成的临床分析

目的　探讨肝硬化 (LC)门静脉血栓 (PVT)形成对LC病程发展的影响。方法　检索我院自 1 995至 2 0 0 2年肝硬化PVT形成患者 ,血栓诊断依据彩色多普勒和 (或 )CT。 4 8例肝硬化PVT形成患者入选血栓组 ;同阶段LC门脉高压症的非血栓病例中选择 5 2例作为对照组。对两组患者的肝功能Child Pugh分级、凝血功能、门静脉、脾静脉宽度及脾脏面积、厚度进行比较。行t检验 ,χ2 检验 ,Logistic回归分析。结果　肝硬化PVT形成除继发于脾切除等手术后 ,75 .0 %隐匿发病 ,85 .4 %的血栓发生于门静脉主干 ,脾脏增大与门静脉增宽是PVT形成的危险因素 (P =0 .0 0 3、0 .0 1 0 )。血栓组门静脉及脾静脉宽度分别为 (1 .4 8± 0 .2 6 )cm ,(1 .2 3± 0 .38)cm ,与对照组比较差异有显著性 [(1 .37± 0 .2 2 )cm ,(1 .0 5± 0 .30 )cm ,P =0 .0 37,0 .0 31 ]。血栓组脾面积平均值为 (96 .6 4± 33.4 )cm2 ,脾厚径为 (6 .0 7± 1 .2 0 )cm ,分别大于对照组的 (80 .81± 2 8.9)cm2 ,(5 .2 3± 1 .0 8)cm(P =0 .0 36 ,0 .0 0 1 )。血栓组食管胃底静脉曲张程度重于非血栓组 ,大出血、大量腹水比例高 (P <0 .0 5 )。血栓形成后 1年内死亡率为1 6 .6 % ,较非血栓组增高 (P =0 .0 2 3)。两组肝功能Child Pugh分级、凝血功能、血小板计     

肝硬化食管胃静脉曲张破裂出血的相关危险因素分析

目的探讨可以评估肝硬化患者食管胃静脉曲张破裂出血风险的临床指标。方法回顾性分析572例合并食管胃静脉曲张破裂出血的肝硬化患者(观察组)及704例未发生出血的肝硬化患者(对照组)的临床资料,对两组间存在差异性的指标运用单因素和多因素Logistic回归模型进行分析。结果两组在肝功能分级状况(P0.05)、血清白蛋白(t=5.05,P=0.000)、凝血酶原时间(t=-2.80,P=0.005)、门静脉内径(t=-2.28,P=0.006)、脾脏厚度(t=-2.73,P=0.006)方面比较,差异有统计学意义。单因素非条件Logistic回归分析显示白蛋白(OR=0.944,P=0.000)、凝血酶原时间(OR=1.067,P=0.007)、门静脉内径(OR=3.423,P=0.007)、脾脏厚度(OR=1.276,P=0.007)与出血存在相关性,进一步多因素非条件Logistic回归分析提示白蛋白(OR=0.936,P=0.000)、门静脉内径(OR=4.098,P=0.013)、脾脏厚度(OR=1.275,P=0.007)是出血的独立危险因素。结论白蛋白、门静脉内径、脾脏厚度是肝硬化并发食管胃静脉曲张破裂出血的独立危险因素,对预测食管胃静脉曲张破裂出血的发生有重要的临床价值,改善白蛋白可在一定程度上减少肝硬化并发食管胃静脉曲张破裂出血的风险。     

诱导型和内皮型一氧化氮合酶基因多态性与肝硬化门静脉高压的相关性

目的探讨诱导型一氧化氮合酶(iNOS)和内皮型一氧化氮合酶(eNOS)基因多态性与肝硬化门静脉高压的相关性。方法采用病例对照和聚合酶链反应-限制性片段长度多态性技术,检测106 例乙型肝炎后肝硬化患者(其中门静脉高压症65例)和108名健康对照者iNOS基因启动子-969G→C多态性及eNOS基因第七外显子894G→多态性,比较等位基因及基因型频率,并进行综合分析。结果在iNOS启动子969G→C多态性中,门静脉高压症组C等位基因和GC基因型频率比对照组显著升高(x2- 5.93,P<0.05)。GC基因型启动子的活性比GG基因型活性强。在eNOS的894G→T多态性中,T等位基因频率明显高于对照组(x2-3.91,P<0.05)。采用Logistic多元回归显示iNOS基因启动子-969G→C多态性及cNOS的894G→T多态性是门静脉高压症新的危险因素。结论iNOS启动子969G→C多态性和eNOS的894G→T多态性与门静脉高压症相关,是形成门静脉高压症新的危险因素。iNOS启动子-969G→C多态性可导致该基因启动子功能活性增强。     

Carvedilol vs endoscopic variceal ligation for primary and secondary prevention of variceal bleeding: Systematic review and metaanalysis

BACKGROUND Variceal hemorrhage is associated with high mortality and is the cause of death for 20–30% of patients with cirrhosis. Nonselective β blockers(NSBBs) or endoscopic variceal ligation(EVL) are recommended for primary prevention of variceal bleeding in patients with medium to large esophageal varices.Meanwhile, combination of EVL and NSBBs is the recommended approach for the secondary prevention. Carvedilol has greater efficacy than other NSBBs as it decreases intrahepatic resistance. We hypothesized that there was no difference between carvedilol and EVL intervention for primary and secondary prevention of variceal bleeding in cirrhosis patients.AIM To evaluate the efficacy of carvedilol compared to EVL for primary and secondary prevention of variceal bleeding in cirrhotic patients METHODS We searched relevant literatures in major journal databases(CENTRAL,MEDLINE, and EMBASE) from March to August 2018. Patients with cirrhosis and portal hypertension, regardless of aetiology and severity, with or without a history of variceal bleeding, and aged ≥ 18 years old were included in this review.Only randomized controlled trials(RCTs) that compared the efficacy of carvedilol and that of EVL for primary and secondary prevention of variceal bleeding and mortality in patients with cirrhosis and portal hypertension were considered, irrespective of publication status, year of publication, and language.RESULTS Seven RCTs were included. In four trials assessing the primary prevention, no significant difference was found on the events of variceal bleeding(RR: 0.74,95%CI: 0.37-1.49), all-cause mortality(RR: 1.10, 95%CI: 0.76-1.58), and bleedingrelated mortality(RR: 1.02, 95%CI: 0.34-3.10) in patients who were treated with carvedilol compared to EVL. In three trials assessing secondary prevention, there was no difference between two interventions for the incidence of rebleeding(RR:1.10, 95%CI: 0.75-1.61). The fixed-effect model showed that, compared to EVL,carvedilol decreased all-cause mortality by 49%(RR: 0.51, 95%CI: 0.33-0.79), with little or no evidence of heterogeneity.CONCLUSION Carvedilol had similar efficacy to EVL in preventing the first variceal bleeding in cirrhosis patients with esophageal varices. It was superior to EVL alone for secondary prevention of variceal bleeding in regard to all-cause mortality reduction.     

肝硬化门脉高压食管胃底静脉曲张破裂出血内窥镜治疗失败后再手术时机探讨

目的 探讨肝硬化门脉高压食管胃底静脉曲张破裂出血内窥镜治疗失败后的再手术时机。方法采用回顾性调查的方法,分析2005年1月至2010年12月48例肝硬化门脉高压症食管胃底静脉曲张破裂出血内窥镜治疗失败后再手术患者病例临床资料,根据术前内窥镜治疗次数分为A组21例,术前内窥镜治疗≤3次;B组27例,术前内窥镜治疗〉3次。比较两组病例术中、术后临床指标情况。结果 A组在胃左静脉血栓发生率、手术时间、术中出血量、断流前及断流后门静脉压力、术后并发症发生率、食管胃底静脉曲张复发率明显低于B组,B组行食管下段横断吻合术比例明显低于A组（P均〈0.05）。结论 肝硬化门脉高压食管胃底静脉曲张破裂出血患者在内窥镜治疗3次失败后,应该及时进行手术治疗。     

Attenuation of portal hypertension by natural taurine in rats with liver cirrhosis

AIM： To investigate the inhibitory effect of natural taurine （NTau） on portal hypertension （PHT） in rats with experimentally-induced liver cirrhosis （LC）. METHODS： Experimentally-induced LC Wistar rats （20 rats/group） were treated with either oral saline or oral NTau for 6 consecutive weeks. Evaluation parameters included portal venous pressure （PVP）, portal venous resistance （PVR）, portal venous flow （PVF）, splanchnic vascular resistance （SVR） and mean arterial pressure （NAP）. Vasoactive substance levels including nitric oxide （NO）, nitric oxide synthase （NOS） and cyclic guanosine monophosphate （cGMP） were also measured. Histological investigation of type Ⅰ and Ⅲ collagen （COL Ⅰ and Ⅲ） and transforming growth factor-β1 （TGF-β1） was also performed. RESULTS： Treatment with NTau （1） significantly decreased PVP, PVR and PVF, and increased MAP and SVP; （2） markedly increased the vascular compliance and reduced the zero-stress of the portal vein; （3） markedly decreased the amount of NO and cGMP and activity of NOS; and （4） improved the pathological status of the liver tissue and reduced the expression of COL Ⅰ, COL Ⅲ and TGF-β1. CONCLUSION： NTau inhibited the LC-induced PHT by improving hyperdynamic circulation, morphology of liver and biomechanical properties of the portal vein in experimentally-induced LC rats.     

门静脉血栓形成对肝硬化病程发展影响

目的探讨门静脉血栓（PVT）形成对肝硬化病程的影响。方法回顾我院2003年～2011年肝硬化伴PVT形成的患者资料。18例肝硬化伴PVT形成患者人选血栓组;随机选择同阶段肝硬化门静脉高压症的无门静脉血栓形成患者19例作为对照组,比较两组患者的门静脉宽度、脾脏厚度、食管胃底静脉曲张、腹水及上消化道大出血发生等情况。结果血栓组的门静脉宽度及脾脏厚度大于对照组,差异有统计学意义（P〈0．05）。血栓组食管胃底重度静脉曲张、上消化道大出血和大量腹水比例两组比较,差异有统计学意义（P〈0．05）。结论脾肿大和门静脉增宽是PVT形成的主要危险因素,PVT形成加重门静脉高压的程度,从而增加上消化道出血几率,使腹水难以消退,增加相关并发症发生率并使相关症状加重,预防门静脉血栓形成有助于延缓肝硬化病情发展。     

检测门脉高压程度评估肝硬化严重程度研究进展

<正>肝硬化是由一种或多种原因引起的、肝组织弥漫性纤维化、假小叶和再生结节为组织学特征的进行性慢性肝病,临床以门静脉高压和肝功能减退为特征,常并发上消化道出血、肝性脑病、继发感染等而死亡。门脉高压常导致食管胃底静脉曲张出血、腹水、脾肿大、脾功能亢进、肝肾综合征、肝肺综合征等,被认为是继病因之后的促进肝功能减退的重要病理生理环节,是肝硬化的主要死亡原因之一。     

Correlation of hepatic venous pressure gradient with variceal bleeding, size of esophageal varices, etiology, ascites and degree of liver dysfunction in cirrhosis of liver

An elevated hepatic venous pressure gradient (HVPG) has been associated with risk of variceal bleeding, and outcome and survival after variceal bleeding. In this pilot study, we measured HVPG in 40 patients with liver cirrhosis and studied its relationship with etiology of liver disease, esophageal variceal size, history of variceal bleeding or ascites, biochemical liver tests and Child-Pugh class. There was no procedurerelated complication. The mean (SD) HVPG was similar in patients who had history of variceal bleeding as compared to those who did not (15.4 [2.8] mmHg vs. 13.9 [2.7] mmHg, p=0.1); HVPG had no significant association with etiology of cirrhosis (p=0.4). HVPG levels were significantly higher in patients with larger esophageal varices (grade III/IV vs. I/II: 15.2 [2.7] mmHg vs.13.1 [2.8] mmHg, p=0.04), poorer Child-Pugh class (B or C versus A), and presence of ascites (p=0.04). Thus, HVPG correlated with variceal size, Child-Pugh class, and presence of ascites, but not with variceal bleeding status.     

门脉高压首次出血的预防进展

静脉曲张出血是肝硬化门脉高压的严重并发症,尽管近年首次静脉曲张出血的死亡率有所下降,但仍是主要死亡原因。目前有人提出用非选择性β受体阻滞剂预防曲张静脉的形成和发展,即一级前预防,并受到重视。非选择性β受体阻滞剂和内镜套扎术(EVL)用于一级预防已较明确。此外,血管紧张素受体抑制剂等药物的运用尚需进一步研究。     

Spontaneous bacterial peritonitis： A severe complication of liver cirrhosis

This report presents a survey of current knowledge concerning one of the relatively frequent and severe complications of liver cirrhosis and associated ascitesspontaneous bacterial peritonitis. Epidemiology,aetiology, pathogenesis, clinical manifestation, diagnosis and present possibilities of treatment are discussed.     

Association of liver cirrhosis related IgA nephropathy with portal hypertension

A high incidence of IgA nephropathy has been reported in patients with liver cirrhosis, though, clinically evident nephrotic syndrome is very uncommon. Impaired hepatic clearance of circulating IgA immune complexes and subsequent deposition in renal glomeruli has been considered principally in the pathogenesis of liver cirrhosis associated IgA nephropathy. Here we report on a patient with cryptogenic liver cirrhosis and splenic vein thrombosis, who presented with nephrotic syndrome. Renal biopsy showed findings consistent with IgA nephropathy. Lower endoscopy showed features of portal hypertensive colopathy. Following initiation of propranolol and anticoagulant treatment to reduce portal pressure, a gradual decrease of proteinuria and hematuria to normal range was noted. The potential pathogenetic role of portal hypertension in the development of IgA nephropathy in cirrhotic patients is discussed.     

TIPSS for variceal hemorrhage after living related liver transplantation： A dangerous indication

The introduction of transjugular intrahepatic portal-systemic stent-shunt(TIPSS)has been a majorbreakthrough in the treatment of portal hypertension,which has evolved to a large extent,into a routineprocedure.A 21-year-old male patient with progressivegraft fibrosis/cirrhosis requiring TIPSS for varicealhemorrhage in the esophagus due to portal hypertensionwas unresponsive to conventional measures twoyears after living related liver transplantation(LDLT).Subsequently,variceal hemorrhage was controlled,however,liver function decreased dramatically withconsecutive multi organ failure.CT scan revealedsubstantial necrosis in the liver.The patient underwentsuccessful"high urgent"cadaveric liver transplantationand was discharged on postoperative d 20 in a stablecondition.     

肝硬化门静脉高压症患者断流术后转归的影响因素分析

目的:研究肝硬化门静脉高压症患者断流术后转归的影响因素．方法:回顾性分析行断流术的肝硬化门静脉高压症患者158例．根据术后并发症情况将患者分为高危组和一般组2组．将其作为因变量,将23个临床指标作为自变量,进行Logistic回归分析．结果:影单因素分析显示,高危组和一般组凝血酶原时间(PT,P=0．007)、Child分级(P= 0．001)、术中所见肝硬化程度(P=0．002)、B型超声测得盆腔腹水前后径值(P=0．023)、胃镜所见食道胃底静脉曲张程度(P=0．010)有显著性差异．Logistic回归分析显示,Child分级、PT和术中所见肝脏硬化程度在两组间仍有显著差异．结论:肝硬化门静脉高压症患者断流术后转归的影响因素为Child分级、PT和术中所见肝脏硬化程度．     

食管曲张静脉压力是预测肝硬化曲张静脉破裂出血的主要危险因素

目的:前瞻性研究影响肝硬化食管曲张静脉破裂出血的主要危险因素.方法:随访未发生过食管曲张静脉出血的57例肝硬化患者1年.采用内镜下无创性食管曲张静脉气囊测压仪检测曲张静脉压,研究终点为出现食管曲张静脉出血.研究食管曲张静脉内镜下表现、食管曲张静脉压力、肝功能分级、肝硬化病因及腹水指标与食管曲张静脉破裂出血的关系.结果:1年内34例(59.6%)患者发生首次食管曲张静脉破裂出血.单因素分析显示,食管曲张静脉压力(P=0.001)、曲张静脉直径(P=0.006)、内镜下红色征(P=0.012)与出血风险有关.进一步的多因素Logistic回归分析显示,食管曲张静脉压力是预测首次出血最主要的危险因子(OR=2.817,P=0.003),其受试者工作曲线(ROC)下面积为0.98.预测出血的食管曲张静脉压力截值为25.3 mm Hg(1 mm Hg=0.133 kPa),其敏感性与特异性均为91%.结论:食管曲张静脉压力是预测食管曲张静脉破裂出血的主要危险因素.     

肝硬化门静脉高压并发症对预后的影响

目的 了解门静脉高压各并发症在失代偿肝硬化患者的发生情况和各并发症对患者预后的影响.方法 选择失代偿期肝硬化患者的病历资料进行登记和随访,根据随访结果,分析患者门静脉高压并发症的发生情况;利用终末期肝病模型(MELD)公式,计算出MELD值并进行分级,同时计算Child-Turcotte-Pugh(CTP)分级,分别分析CTP分级和MELD分级中门静脉高压并发症发生情况和患者生存状况.利用Kaplan-Meier生存分析方法分析门静脉高压并发症对肝硬化患者生存率的影响.利用x2检验和时序性检验比较生存率差别,Cox比例风险回归分析各个并发症对患者生存影响作用的大小.结果 在符合条件的322例失代偿期肝硬化患者中,发生食管胃底静脉曲张破裂出血、肝性脑病、大量腹水、自发性腹膜炎、肝肾综合征Ⅰ型和Ⅱ型的患者病死率分别是45.9%、79.4%、66.7%、100%、100%和84.6%.各并发症的发生基本按CTP分级和MELD值的增加而逐渐升高.经过Kaplan-Meier生存分析,除少量和中量腹水外,各并发症对患者生存率的影响,P值均<0.01,差异均有统计学意义.由Cox回归过程分析出肝性脑病、自发性腹膜炎、肝肾综合征Ⅰ型和Ⅱ型,食管胃底静脉曲张破裂出血和腹水的回归系数分别为0.973、0.928、0.935、0.866、0.464和0.369. 结论 门静脉高压并发症均能对失代偿期肝硬化患者的预后造成明显影响,其中影响程度最大的是肝性脑病.     

部分脾动脉栓塞术后短期疗效观察

通过对肝炎肝硬化门静脉高压患者行部分脾动脉栓塞术（PSE）治疗,观察短期术后肝功能、血细胞计数和并发症发生情况。方法63例肝炎肝硬化患者术前行B超或（和）CT检查,常规行PSE。结果在术前,患者血清ALT、ALB和TBIL分别为（49.9±9.8） U/L、（35.6±1.6） g/L和（22.47±3.7）μmol/L,而术后8 d则分别为（24.4±3.1） U/L、（33.7±1.7） g/L和（30.2±4.4）μmol/L,均变化显著（P〈0.05）;术前WBC、RBC和PLT分别为（2.36±1.24）×10^9/L、（3.62±0.51）×10^12/L和（45.3±20.2）×10^9/L,术后8 d则分别为（6.32±2.16）×10^9/L、（3.66±0.47）×10^12/L和（154.2±161.2）×10^9/L,白细胞和血小板升高显著（P〈0.05）;所有患者在术后均出现不同程度的发热、脾区疼痛。结论 PSE虽可减轻肝硬化脾功能亢进症患者血细胞减少,但术后短期内黄疸加深,须要加强保肝和退黄治疗。