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症(INCPH)是一种罕见的血管性肝病,近年来引起了人们新的关注。INCPH在组织学改变、血流动力学特征和临床结果方面与肝硬化不同。重要的是,其病因和发病机制尚不清楚,但已确定与肝内血管疾病的发展有关,并在排除引起门静脉高压的其他原因(如肝硬化以及其他导致门静脉高压的窦前性、窦性、窦后性原因和内脏静脉血栓形成)后做出的。此外,特发性非肝硬化性门静脉高压症的总体预后优于肝硬化患者,但对症治疗(控制胃食管静脉曲张出血和预防血栓形成)仍是主要治疗方法。目前临床上对INCPH的认识相对不足,对该病易发生误诊,本文通过目前对INCPH研究进展的阐述,以提高临床医生对此病的认识。     

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。     

特发性非硬化性门静脉高压诊治进展

特发性非硬化性门静脉高压是一组少见的临床综合征,它的特征是没有肝硬化的组织学表现但有门静脉高压的临床表现,并且排除已知的可以引起门静脉高压的肝内外原因。其病因和发病机制尚不明确,但是可以确定与肝内血管病变的发展有关。目前认为可能的致病机制包括：免疫紊乱、慢性感染、毒物或药物损伤、微血栓形成、基因异常等。最常见的临床表现...     

我国肝硬化门静脉高压症药物治疗现状及进展

门静脉高压症是一组门静脉血流系统异常变化为特点症候群,以食管胃底曲张静脉破裂出血为主要并发症。门静脉高压症的治疗主要是针对出血的治疗,治疗策略分为一级预防、控制急性出血和二级预防,治疗方法较多,合理选择非常重要,其中药物治疗在门脉高压症的治疗中扮演重要的角色,本文旨在介绍我国目前药物治疗现状及进展。     

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...     

肝硬化并门静脉高压及食管-胃静脉曲张的治疗进展

肝硬化(LC)并门静脉高压(PVH)引起的食管静脉曲张(EV)和(或)胃静脉曲张(GV)即EGV及其出血(EGVB)的有关发病机制，特别是治疗方面的报道很多，但在治疗措施上存在不少争论。Garcia-Tsao发表一长篇述评，涉及LC发生PVH血液动力学(HD)的进展和EGV和治疗方案的评定和抉择，时至今日仍具有指导性和权威性。     

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。     

特发性非肝硬化门静脉高压症患者肝脏影像学和病理学特征分析

目的 分析特发性非肝硬化门静脉高压症(INCPH)患者肝脏影像学和病理学特征,并与肝硬化的鉴别要点。方法 2016年1月～2021年7月我院收治的INCPH患者16例和乙型肝炎肝硬化患者28例,常规进行超声、CT和MRI及肝穿刺活检检查。结果 INCPH与肝硬化患者在影像学检查发现的弥漫性结节样改变(0.0%对35.7%)、门静脉直径【9.7(7.2,11.6)mm对13.6(9.2,15.7)mm】、门静脉壁厚【2.6(1.4,4.0)mm对1.4(1.1,1.6)mm】方面比较,差异具有统计学意义(P<0.05);肝组织学检查发现,INCPH与肝硬化患者在门静脉区域纤维化、肝膈膜纤维化、肝小叶间静脉闭塞、肝细胞坏死和肝细胞水肿或脂肪变性方面【分别为100.0%对0.0%、18.7%对92.8%、56.2%对10.7%、0.0%对75.0%和12.5%对89.3%】,差异具有统计学意义(P<0.05)。结论 INCPH仍是一种病因不明的疾病,注意分析影像学和组织病理学特征可以作出与肝硬化的鉴别诊断。     

高度重视门静脉高压症出血治疗中的几个关键问题

门静脉高压症是一种常见的临床综合征,在我国门静脉高压诸多病因中,肝硬化占90%左右,以肝炎后、血吸虫病及酒精性肝硬化为主要原因。门静脉高压症的并发症包括食管和(或)胃静脉曲张破裂出血、腹水、肝性脑病及肝癌等,这些并发症是患者死亡的主要原因,其中最严重的当属食管胃静脉曲张破裂出血。结合我国的临床实际,以下几个关键问题必须引起临床工作者的高度重视。慢性肝病和肝硬化患者的内镜定期检查问题     

特发性非肝硬化性门脉高压1例并文献复习

<正>特发性非肝硬化性门脉高压(idiopathic non-cirrhotic portal hypertension,INCPH)是一种血管性疾病,临床表现为门脉高压的一系列症候,但缺乏肝硬化的病理学表现,约占门脉高压症患者的10%。因缺乏相对特异的诊断标准,易误诊。现报道1例我科诊治的以脾功能亢进症为首发症状的、经13年就医路终确诊的INCPH患者,以期提高临床医师对该病的诊治水平。1病例摘要患者女,68岁。主因“发现脾肿大13年,间断腹泻8月”于2020年9月9日入院。患者于13年前因“急性胆囊炎、胆囊结石”于我院普外科行手术治疗,     

Immunological profile of patients with non-cirrhotic portal fibrosis

The aetiopathogenesis of non-cirrhotic portal fibrosis (NCPF), a common cause of portal hypertension in India, is not known. To study the immune status of NCPF patients and to see whether immunological mechanisms have a role to play, humoral and cell-mediated immunological studies were carried out in 43 patients with NCPF and compared with equal number of matched healthy controls and 31 patients with compensated liver cirrhosis. Serum immunoglobulin A (IgA) and complement (C3, C4) levels were significantly (P less than 0.001) lower in NCPF patients compared with controls and cirrhotics. There was no significant difference between the total or the relative concentration of the immunoglobulins and complements between NCPF patients and healthy controls, but, in patients with cirrhosis, concentration of all the immunoglobulins was higher. The cutaneous response to dinitrochlorobenzene was poorer in patients with NCPF, but the difference between cirrhotics and controls was not significant. A decrease in the suppressor/cytotoxic (T8) phenotype of lymphocytes in the peripheral blood and an increase in the ratio of helper/inducer (T4) and T8 lymphocytes was seen in patients with NCPF and cirrhosis. Although these results indicate definite immunological abnormalities in NCPF patients, their role in the pathogenesis of NCPF remains to be investigated.     

Natural/spontaneous shunts in non-cirrhotic portal fibrosis

Large natural/spontaneous shunts on splenoportovenography were demonstrated in 10 of 93 patients with non-cirrhotic portal fibrosis. There was significantly less bleeding in patients with spontaneous shunt than in those having no shunt. Large oesophageal varices (grade III-IV) were seen more frequently in patients without spontaneous shunt.     

Altered alpha adrenergic vasoresponsiveness in a non-cirrhotic portal hypertension model of E. coli injection

BACKGROUND AND AIM: Portal hypertension is associated with decreased vascular responsiveness to vasoconstrictors, which may contribute to the hyperdynamic circulation in cirrhosis. Animal models of cirrhosis and portal vein ligation have helped in our understanding of portal hypertension. The etiopathogenesis of non-cirrhotic portal fibrosis (NCPF), a common cause of portal hypertension, is still poorly understood. The aim of this study was to investigate the pathophysiology of NCPF in a rabbit model. METHODS: An indwelling cannula was inserted into the gastrosplenic vein of rabbits. Animals were randomly injected with saline (Group I, n = 13) or lipopolysaccharide (Group II, n = 13) from heat killed Escherichia coli at 0, 1, 2, 7, 14 and 28 days. Portal pressure was measured at 3 months and vasoresponsiveness studied in isolated aortic rings in intact and in endothelium-denuded tissues from both groups. RESULTS: In all group II compared with group I animals, the splenic weight (0.89 +/- 0.16 vs 0.62 +/- 0.1 g, P < 0.05) and the portal pressure (14.99 +/- 0.56 vs 7.04 +/- 0.42 mmHg, P < 0.05) were higher at 3 months. The group II animals showed reduced responsiveness to phenylephrine showing maximal contraction of 1.25 +/- 0.08 at 10(-4) mol/L as compared to 2.85 +/- 0.33 g tension in Group I (P < 0.05). Endothelium denudation of aortic rings had no effect on reduced reactivity in Group II animals. Acetylcholine induced an increase in vasorelaxation at lower concentrations in preconstricted aortic rings in Group II compared to Group I animals, but this decreased in higher concentrations. Nifedipine produced comparable vasodilatation in preconstricted rings in both the groups of animals. CONCLUSIONS: Repeated injection of lipopolysaccharide into the gastrosplenic vein leads to the development of portal hypertension. This non-cirrhotic model of portal hypertension is characterized by generalized arterial hyporeactivity to vasoconstrictors akin to other models of portal hypertension.     

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。     

Rabbit model of non-cirrhotic portal fibrosis with repeated immunosensitization by rabbit splenic extract

BACKGROUND: Non-cirrhotic portal fibrosis (NCPF) or idiopathic portal hypertension, a disease of unknown etiology, is a common cause of portal hypertension in developing countries. Attempts to understand the etiopathogenesis of NCPF by developing animal models have been made. We describe a novel approach using repeated injections of rabbit splenic extract that were obtained from a previously primed rabbit, to develop a model of NCPF. METHODS: Twenty-eight rabbits (1.5-2.0 kg) were divided into the control (group I, n = 13) and the experimental (group II, n = 15) groups. The supernatant obtained after centrifugation of a 20% splenic homogenate, containing 6 mg protein/mL, was mixed with Freund's complete adjuvant (1:1 ratio) and injected intramuscularly to the recipient rabbits every 2 weeks for 3 months. Portal pressure was measured by inserting a cannula into the gastrosplenic vein. RESULTS: The mean portal pressure in group II was significantly (P < 0.05) higher than group I at 1 (19.4 +/- 2.9 vs 10.4 +/- 2.2 mmHg), 3 (16.7 +/- 1.1 vs 7.2 +/- 3.6 mmHg), and 6 (20.3 +/- 5.4 vs 10.3 +/- 4.8 mmHg) months. The mean splenic weight in group II was significantly (P < 0.05) greater than group I at 1, 3 and 6 months. Histopathology of spleen showed medullary congestion, hemosidrin laden macrophages and mild fibrosis. Liver showed normal hepatocytes with mild portal lymphocytic infiltrates and Kupffer cell hyperplasia. No significant anomalies were observed in the tests of liver function at 1 and 6 months. CONCLUSIONS: This animal model showed significant splenomegaly, with persistent rise in portal pressure without hepatic parenchymal injury, quite akin to NCPF seen in humans. This study also proposes that repeated immunostimulation may have an important role in the pathogenesis of NCPF.     

Successful treatment of noncirrhotic portal hypertension with eculizumab in paroxysmal nocturnal hemoglobinuria: A case report

BACKGROUND Idiopathic non-cirrhotic portal hypertension(INCPH) is mainly associated with thrombophilia in Western countries. Paroxysmal nocturnal hemoglobinuria(PNH) is a rare hematologic disease that manifests with hemolytic anemia,thrombosis, and peripheral blood cytopenias. Portal and hepatic venous thrombosis were reported in PNH. A rare case of INCPH complicating PNH is described.CASE SUMMARY A 63-year old woman with a 2-year past medical history of PNH without treatment was admitted because of jaundice and refractory ascites requiring large volume paracentesis. Liver histology revealed portal venopathy with portal fibrosis and sclerosis, nodular regenerative hyperplasia, parenchymal ischemic changes, and focal sinusoidal and perivenular fibrosis without bridging fibrosis or cirrhosis, all indicative of INCPH. The flow cytometry confirmed PNH diagnosis and eculizumab treatment was initiated. Her condition was improved gradually, bilirubin was normalized 6 months following initiation of eculizumab,and 1 year later diuretics were stopped.CONCLUSION Eculizumab improved intravascular hemolysis and reversed clinical manifestations of INCPH in a patient with paroxysmal nocturnal hemoglobinuria.