特发性门脉高压1例

<正>患者男,22岁,因反复呕血、黑便1年余,再发1d就诊我院。患者1年余于饮酒后出现呕鲜红色血液,约400ml,伴解暗红色血便,无头晕、黑曚、晕厥,无腹痛、腹胀等不适,未重视,未特殊化诊治自行好转。入院前1年无明显诱因再次出现呕血、解暗红色血便,外院查腹部超声提示门静脉、脾静脉增宽,脾大,胃镜提示食管静脉曲张(重度、红色征阳性)。予降门脉压、抑酸、补液支持等对症治疗后好转出院。出院后患者无眼黄、尿黄,无腹胀、尿少、下     

特发性门脉高压症一例

患者男性,19岁,因乏力、皮下瘀斑5月余入院。2007年4月无明显诱因感乏力,皮肤轻碰后出现暗红色瘀斑。查体：发育正常,皮肤无黄染,巩膜稍黄染,未见肝掌、蜘蛛痣,无瘀点、瘀斑。     

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。     

免疫机制在特发性门脉高压症发病中的作用

特发性门脉高压症临床上较少见,主要表现为脾大、贫血和门脉高压,无肝硬化和肝外门静脉及肝静脉阻塞,肝功能基本正常.肝脏组织学变化主要为汇管区和门脉周围纤维化及炎性细胞浸润.血流动力学可发现脾门脉血流量增加.此文就免疫机制在其发病中的作用做简要综述.     

特发性门脉高压症研究现状

特发性门脉高压症（Ｉｄｉｏｐａｔｈｉｃｐｏｒｔａｌｈｙｐｅｒｔｅｎｓｉｏｎ，ＩＰＨ）系指门脉高压伴有脾肿大、脾机能亢进而没有肝硬化和肝外门静脉阻塞的一组临床综合征。ＩＰＨ病名最早在本世纪６０年代由Ｂｏｙｅｒ提出。同时代的印度学者Ｒａｍａｌｉｎｇａｓｗａｍｉ经过临床及病理研究发现了不伴肝硬化的脾脏肿大并称之为非硬化性门脉纤维化。１９６５年，Ｍｉｋｋｅｌｓｅｎ等研究了３６例不伴肝硬化的门脉高压患者，证明这些患者有肝内外门静脉硬化，因此称为肝内门静脉硬化症（ＨＰＳ）。现代学者经研究证实，上述三种病名可…     

特发性门脉高压症1例

1 病例摘要 患者,女性,44岁,因乏力、食后上腹饱胀2年余,于2011年4月26日入院.既往体健,无高血压病、糖尿病、冠心病等内科病史,无手术外伤史,无输血及血制品史,无结核、伤寒等病史及密切接触史,近期无疫区旅居及疫水接触史,无放射线及毒物接触史,无烟酒嗜好,否认家族肝病史.     

21例特发性门脉高压临床及病理特点分析

目的分析特发性门脉高压(idiopathic portal hypertension,IPH)的临床及病理特点。方法回顾性分析2012年1月—2016年12月在解放军第三〇二医院住院治疗(资料完整)的21例IPH患者的临床及病理特点。结果 21例IPH患者中,男女比例6∶15,平均发病年龄(38.1±12.7)岁,临床以门脉高压症表现为主,肝功能无明显减退,主要并发症为上消化道出血及腹水。21例肝组织病理主要表现为肝细胞板排列基本正常,无假小叶形成,汇管区扩大,门静脉周围纤维化,门脉周围有不同程度的细胞浸润,血管紊乱,中央静脉及小叶间静脉扩张,肝窦扩张,窦周纤维化。结论 IPH患者门脉高压和肝功能损害不平行,门脉高压表现较重,确诊仍须病理学检查,治疗以防治并发症为主。     

特发性门静脉高压症研究进展

自20世纪60年代以来,许多学者研究发现,某些原因不明的脾肿大和门静脉高压是由于门静脉纤维化使门静脉血流受阻、门静脉压升高而继发脾肿大,但一般并不发展成肝硬化,1962年印度学者Ramalingas Wami称之为非硬化性门脉纤维化(Non cihotic portal fibrosis NCPF),英国也沿用这一名称[1,2],1965年美国Mikkelsen称之为肝内门静脉硬化症(Hepatoportal sclerosis HPS),而在日本则称为特发性门静脉高压症(Idiopathic portal hypertension IPH)[1].     

免疫机制在特发性门脉高压症发病中的作用

特发性门脉高压症临床上较少见,主要表现为脾大、贫血和门脉高压,无肝硬化和肝外门静脉及肝静脉阻塞,肝功能基本正常。肝脏组织学变化主要为汇管区和门脉周围纤维化及炎性细胞浸润。血流动力学可发现脾门脉血流量增加。此文就免疫机制在其发病中的作用做简要综述。     

Study of portal vein thrombosis in patients with idiopathic portal hypertension in Japan.

BACKGROUND/AIMS: The aim of this study was to elucidate the incidence and clinical manifestations of portal vein thrombosis (PVT) in patients with idiopathic portal hypertension (IPH) in Japan during long-term follow-up. PATIENTS AND METHODS: Twenty-two patients with IPH were examined for PVT by sonography during a follow-up of 12+/-6 years. Clinical manifestations and patient outcome related to PVT were studied. Seventy patients with liver cirrhosis were examined by sonography as an incidence control of thrombosis. RESULTS: Nine IPH patients had portal thrombosis (9/22, 41%), a higher incidence than in liver cirrhosis patients (7/70, 10%). Those with thrombosis showed ascites, marked hypersplenism, and low serum albumin. Four patients with thrombosis died. Patients without thrombosis showed less clinical problems after long-term follow-up. Plasma antithrombin III and protein C activity decreased in almost half of the patients. However, there were no differences in these parameters between patients with and without thrombosis. CONCLUSIONS: In Japan, IPH patients had a high incidence of portal thrombosis, a significant factor for poor prognosis. Whether the management of PVT contributes to an improvement of a clinical course of IPH or not should be clarified in further study.     

老年高血压病患者凝血纤溶指标变化及药物干预的评价

目的　研究老年高血压病患者凝血纤溶功能的变化及血管紧张素转换酶抑制剂 (ACEI)与血管紧张素Ⅱ受体 1(AT1)拮抗剂对高血压病患者凝血纤溶功能的影响。方法　测定 4 0例老年高血压病患者及 2 0例与高血压病患者年龄性别相匹配的正常对照组血浆纤维蛋白原 (Fbg)、假性血友病因子 (vWF)、P 选择素、凝血酶原激活物抑制剂 1(PAI 1)。随后 4 0例老年高血压病患者随机分成 2组 ,依那普利组 2 0例 ,10～ 2 0mg ,每日口服 ;氯沙坦组 2 0例 ,5 0～ 10 0mg ,每日口服 ,治疗 8周 ,治疗后测Fbg、vWF、P 选择素、PAI 1。结果　老年高血压病组治疗前Fbg(P <0 .0 1)、vWF(P <0 .0 0 1)、P 选择素 (P <0 .0 5 )、PAI 1(P <0 .0 5 )显著高于对照组 ,药物治疗后依那普利组、氯沙坦组较用药前Fbg、vWF、P 选择素、PAI 1均降低 ,有显著性差异 (P <0 .0 5 ) ,而两组间凝血纤溶指标降低无显著性差异。结论　老年高血压病患者存在高凝状态 ,依那普利与氯沙坦抗高血压治疗时可改善其高凝状态     

Analysis of adhesion molecules in patients with idiopathic portal hypertension

BACKGROUND: The aetiology of idiopathic portal hypertension (IPH) is unknown. However, some evidence of immunological abnormalities in IPH patients has been reported. METHODS: As adhesion molecules are important in the interaction between lymphocytes and accessory and target cells, the expression and release of the soluble form of vascular cell adhesion molecule-1 (VCAM-1) and intercellular adhesion molecule (ICAM-1) were examined in this study. RESULTS: In IPH patients, the serum level of soluble VCAM-1 was found to be increased, compared with that of healthy subjects, fatty liver patients and chronic hepatitis patients. The level of soluble ICAM-1 of IPH patients was found to be slightly increased, compared with that of healthy subjects; however, it was not different from the level in patients with other diseases. The expression of VCAM-1 was observed in the sinusoidal lining cells and endothelial cells around the liver vessels of several IPH patients. In contrast, ICAM-1 was weakly expressed in sinusoidal lining cells and hepatocytes in the liver tissue of only one of four IPH patients. CONCLUSIONS: This differential pattern of VCAM-1 and ICAM-1 was found in IPH patients and it was suggested that VCAM-1 might be an important molecule in the occurrence of IPH.     

Splenomegaly, hypersplenism and coagulation abnormalities in liver disease

Splenomegaly is a frequent finding in patients with liver disease. It is usually asymptomatic but may cause hypersplenism. Thrombocytopenia is the most frequent manifestation of hypersplenism and may contribute to portal hypertension related bleeding. A number of therapies are available for treating thrombocytopenia due to hypersplenism including splenectomy, partial splenectomy, partial splenic embolization, TIPS etc. None is entirely satisfactory. Hypersplenism usually improves following liver transplantation. Therapy with cytokines such as thrombopoietin may offer hope for the future. Patients with liver disease also have abnormalities in coagulation. This is not surprising as all coagulation proteins (except for von willebrand factor vWF) and most inhibitors of coagulation are synthesized in the liver. Genetic or acquired abnormalities of coagulation may predispose to thrombosis of the hepatic or portal veins with significant clinical sequelae. An understanding of the mechanisms involved in coagulation and thrombosis is valuable in choosing from the increasing treatment options available. These include clotting factors, haemeostatic drugs and newer therapies such as recombinant factor VIIa. Splenic artery aneurysms are the most common visceral artery aneurysms in man. Rupture is frequently catastrophic. These aneurysms are being increasingly recognized in liver transplant patients and require treatment before or during transplant surgery.     

肝硬化患者血栓前状态分子标志物对门静脉血栓形成的监测意义

目的探讨肝硬化患者及肝硬化合并门静脉血栓患者血栓前状态分子标志物的变化。方法将32例河南省濮阳市油田总医院2011年-2013年住院的肝硬化合并门静脉血栓的患者设为血栓组(PVT组),40例肝硬化非门静脉血栓的患者设为非血栓组(非PVT组),采用ELISA法检测血小板颗粒膜蛋白-140(GMP-140)、血管性假性血友病因子(v WF:Ag)、血栓调节蛋白(TM)、D二聚体(DD)的含量并进行分析。计量资料组间比较采用t检验。结果 PVT组GMP-140、TM、v WF:Ag、DD含量分别为(20.68±1.49)μg/L、(47.24±1.36)μg/L、(194.32±7.68)%、(0.86±0.12)mg/L,均明显高于非PVT组(13.05±0.97)μg/L、(34.05±5.03)μg/L、(136.21±3.68)%、(0.42±0.08)mg/L,两组比较差异均有统计学意义(P值均0.01),PVT组伴中重度食管静脉曲张患者血浆GMP-140、TM、v WF:Ag、DD水平分别为(19.68±1.29)μg/L、(45.24±1.26)μg/L、(196.32±6.68)%、(0.79±0.12)mg/L,显著高于轻度食管静脉曲张患者(12.05±1.07)μg/L、(35.05±4.83)μg/L、(141.21±3.45)%、(0.36±0.08)mg/L,差异均有统计学意义(P值均0.01);PVT组伴消化道出血患者血浆GMP-140、TM、v WF:Ag、DD水平分别为(18.98±1.18)μg/L、(46.78±1.35)μg/L、(197.32±6.39)%、(0.81±0.14)mg/L显著高于无出血患者(11.98±1.12)μg/L、(36.02±4.78)μg/L、(138.21±4.12)%、(0.35±0.12)mg/L,差异均有统计学意义(P值均0.01)。结论血栓前状态分子标志物水平可能对肝硬化门静脉血栓形成有监测作用。     

A defective autologous mixed lymphocyte reaction in patients with idiopathic portal hypertension

The aetiology of idiopathic portal hypertension or hepatoportal sclerosis is unknown. In view of the indirect evidence for underlying immunologic abnormalities 14 patients (all middle-aged females) were studied. Various auto-antibodies were demonstrated in seven patients and high levels of serum immunoglobulins, either IgG, IgM or IgA were present in ten patients. T cell responsiveness to stimulation with either autologous or allogeneic non-T cells was examined in nine of 14 idiopathic portal hypertension patients and compared with responsiveness in patients with posthepatitic cirrhosis and splenomegaly, and healthy controls. Patients with cirrhosis had levels of T cell responsiveness which were not significantly different from those in healthy controls in both autologous and allogeneic mixed lymphocyte reactions. A distinctly reduced autologous mixed lymphocyte reaction was observed in all idiopathic portal hypertension patients. These data indicate that, like many other autoimmune diseases, abnormal serological features and impaired autoreactive T cell responsiveness exist in patients with idiopathic portal hypertension.     

高血压患者胰岛素抵抗与凝血系统功能的关系

目的:研究高血压(EH)患者胰岛素抵抗(IR)与凝血系统功能的关系。方法:按75g葡萄糖口服负荷法(OGTT)结果,69例EH患者被分为糖耐量正常(NGT)组(43例)例和糖耐量异常(IGT)组(26例)。另正常对照组31例。测定各组纤维蛋白原(Pg)、纤溶酶原激活物抑制因子(PAI-1)含量,同时作血胰岛素(INS)测定, 用HOMA指数作为胰岛素抵抗指标。结果:高血压两组与正常对照组相比HOMA指数、Fg、PAI-1水平明显异常(P<0.05～<0.01).高血压IGT组的这些指标较高血压NGT组更恶化(P<0.05～<0.01)。HOMA与PAI-1、Fg 之间具有正相关性,其r分别为0.635,0.832。结论:高血压病人存在胰岛素抵抗和凝血异常,当高血压合并IGT时, 胰岛素抵抗更严重,血液促凝血状态加重。     

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。