非肝硬化性门脉高压的临床诊断

门静脉高压症是指各种原因导致的门静脉压力升高,临床表现为脾肿大、脾功能亢进症、食管胃底静脉曲张和腹水等临床症候群。根据病变部位的不同,门脉高压可分为肝前性、窦前性、窦性、窦后性和肝后性门脉高压[1]。引起门脉高压的最常见病因是肝硬化,属于窦性门脉高压,约占我国患者的80%~85%,而非肝硬化性门脉高压(non-cirrhotic portal hypertension,NCPH)是指患者有明显的门脉高压表现,但临床生化、影像学或组织学上无肝硬化证据的一组疾病,包括肝外门静脉闭塞症、先天性肝纤维化、特发性非硬化性门脉高压、肝窦阻塞综合征和布加综合征等[2]。NCPH患者与肝硬化门脉高压症患者在病因、治疗和预后方面有明显的不同,因此应重视对该组疾病的认识。本文对常见的NCPH疾病进行了简述,期待提高临床医生对该组疾病的认识。     

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。     

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。     

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。     

30岁以下人群食管胃静脉曲张患者临床特点分析

目的 分析总结30岁以下食管胃静脉曲张(GOV)患者的临床特点。方法 2015年1月～2020年12月解放军总医院第一医学中心消化内科医学部收治的61例30岁以下GOV患者,提取、分析和总结其临床资料。结果 在61例GOV患者中,肝硬化门静脉高压症27例(44.3%),其中隐源性肝硬化占40.7%,乙型肝炎肝硬化占33.3%,和非肝硬化性门静脉高压(NCPH)34例(55.7%),其中以门静脉海绵样变占61.8%;基于内镜下静脉曲张LDRf分型,在位置方面主要以Le/g型多见(77.1%),在直径方面,D1.0占41.0%,在出血风险方面,Rf1分级占77.1%;针对GOV治疗,以二级预防治疗为主(85.7%),多采用组织胶或硬化剂注射或套扎联合治疗(66.1%);NCPH患者GOV再出血比例为11.8%,显著低于肝硬化组的29.6%(P<0.01)。结论 30岁以下人群GOV患者以NCPH居多,其中以各种原因引起的门脉海绵样变最多见。NCPH患者并发GOV经内镜治疗后再出血发生率显著低于肝硬化患者。     

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。     

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…     

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。     

先天性肝纤维化24例临床分析并文献复习

目的分析先天性肝纤维化的临床特征。方法对24例先天性肝纤维化患者临床症状、体征、实验室化验检查及病理学特点进行回顾性分析。结果 24例患者均有门脉高压表现,8例曾有消化道出血。化验肝功能正常或轻度异常。影像学检查提示7例存在肝肾囊肿。22例病理结果均提示先天性肝纤维化,其中9例合并先天性肝内胆管扩张（Caroli’s）病。结论对于病因不明门脉高压,尤其门脉高压与肝功损害程度不一致的患者应尽量行肝组织活检病理检查以协助诊断。先天性肝纤维化与肝肾囊肿、Caroli’s病常常伴发。     

门脉高压与肝星状细胞

肝硬化伴门脉高压时肝窦的变化使血流阻力增加 ,肝星状细胞 (ＨＳＣ)受ＮＯ的扩张及内皮素 (ＥＴ)的收缩作用 ,导致门脉高压。本文就门脉高压与ＨＳＣ的最新研究动向作一介绍。一、门脉高压与肝窦的血流障碍肝窦是连接末端门静脉分支及中央静脉的枢纽 ,长约450 μｍ ,直径约 1     

先天性肝纤维化——附12例临床分析

目的分析先天性肝纤维化的临床特征。方法对12例先天性肝纤维化患者临床表现、实验室检查及病理组织学特点进行回顾性分析。结果12例患者均有明显临床症状,10例有典型门脉高压症状,11例就诊时肝功能异常,肝脏病理主要表现为：肝组织内呈现宽大致密且炎症不明显的胶原纤维间隔,或纤维束弥漫穿插于固有的肝小叶内,无典型的假小叶结构。结论对于不明原因肝功能异常的门脉高压症患者,应尽可能进行肝组织活检病理检查以协助诊断及指导治疗。     

抗生素治疗原发性腹膜炎对肝硬化门脉血液动力学的影响

目的研究抗生素治疗60例肝硬化原发性腹膜炎患者对肝硬化门脉血流的影响。方法用抗生素治疗肝硬化原发性腹膜炎患者,根据临床资料对患者行Child—Pugh’s分期,用彩色多普勒检测治疗前、后门脉主干内径、门脉主干、门脉左支矢状部血流速度;并检测血浆内毒素水平（LPS）。结果治疗后内毒素水平明显低于治疗前（P〈0．05）;门脉主干及门脉左支矢状部血流速度较治疗前明显增加（P〈0．05）,但门脉内径无明显变化（P〉0．05）。结论抗生素治疗肝硬化原发性腹膜炎可降低内毒素水平,增加门静脉血流,改善肝脏功能,使再出血率降低。     

Follow up in a case of congenital hepatic fibrosis (author's transl)

A case of congenital hepatic fibrosis (CHF) is described. CHF is characterized by hepatomegaly, portal hypertension, extensive portal fibrosis, ectatic bile ducts, and hypoplasia of terminal portal vein branches. In contrast to the severe portal hypertension liver function tests are largely normal. In our case the disease was first detected when the patient was 7 years old. During the following 9 1/2 years three sequential liver biopsies were performed. Each of them showed the same picture and no progression occurred. The characteristic histological picture of CHF includes mature bile ducts without epithelial proliferation, absence of significant intraportal or interlobular inflammatory infiltrates, and small or hypoplastic distal portal vein branches. On the basis of these features the disease can easily be separated from other forms of liver cirrhosis.     

64层螺旋CT门静脉血管造影及重建技术的临床应用

目的评价64层CT门静脉血管造影(MSCTP)及重建技术的临床应用价值。方法 56例门静脉病变患者,采用64层螺旋CT行动脉期、门脉期及平衡期扫描后,运用容积重建法(VR)、多层面重建法(MPR)和最大密度投影法(MIP)对门静脉及其分支进行重建,以评价门静脉病变的范围和程度。结果 MSCTP能满意显示正常和异常的门静脉,VR、MIP均可显示4级以上的门静脉分支,直观地评价门静脉的位置、轮廓、有无门静脉受侵或癌栓形成,了解门静脉高压侧枝循环的分布范围和程度。MPR能较直观地显示病变整体形态和范围。结论螺旋CT门静脉造影是门静脉无创性检查的可靠方法,具有很高的临床应用价值。     

Non-cirrhotic portal fibrosis: current concepts and management

Non-cirrhotic portal hypertension (NCPH) comprises diseases having an increase in portal pressure (PP) due to intraheptic or prehepatic lesions, in the absence of cirrhosis. The lesions are generally vascular, either in the portal vein, its branches or in the perisinusoidal area. Because the wedged hepatic venous pressure is near normal, measurement of intravariceal or intrasplenic pressure is needed to assess PP. The majority of diseases included in the category of NCPH are well-characterized disease entities where portal hypertension (PHT) is a late manifestation and, hence, these are not discussed. Two diseases that present only with features of PHT and are common in developing countries are non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal vein obstruction (EHPVO). Non-cirrhotic portal fibrosis is a syndrome of obscure etiology, characterized by 'obliterative portovenopathy' leading to PHT, massive splenomegaly and well-tolerated episodes of variceal bleeding in young adults from low socioeconomic backgrounds, having near normal hepatic functions. In some parts of the world, NCPF is called idiopathic portal hypertension (IPH) or 'hepatoportal sclerosis'. Because 85-95% of patients with NCPF and EHPVO present with variceal bleeding, treatment involves management with endoscopic sclerotherapy (EST) or variceal ligation (EVL). These therapies are effective in approximately 90-95% of patients. Gastric varices are another common cause of upper gastrointestinal bleeding in these patients and these can be managed with cyanoacrylate glue injection or surgery. Other indications for surgery include failure of EST/EVL, and symptomatic hypersplenism. The prognosis of patients with NCPF is good and 5 years survival in patients in whom variceal bleeding can be controlled has been reported to be approximately 95-100%.     

Non-cirrhotic portal fibrosis

Non-cirrhotic portal hypertension (NCPH) comprises of diseases having an increase in portal pressure (PP) due to intraheptic or prehepatic lesions, in the absence of cirrhosis. The lesions are generally vascular, either in the portal vein, its branches or in the perisinusoidal area. Because the wedged hepatic venous pressure (WHVP) is near normal, measurement of intravariceal or intrasplenic pressure is needed to assess portal pressure. The majority of the diseases included in the category of NCPH are well characterized disease entities where portal hypertension (PHT) is a late manifestation and hence, these are not discussed. Two diseases which present only with features of PHT and are common in developing countries are NCPF and extra-hepatic portal vein obstruction (EHPVO). Non-cirrhotic portal fibrosis is a syndrome of obscure etiology, characterized by 'Obliterative portovenopathy' leading to PHT, massive splenomegaly, repeated well tolerated episodes of variceal bleeding and anemia in young adults from low socio-economic strata of life. The hepatic parenchymal functions are nearly normal. Jaundice, ascites and hepatic encephalopathy are rare. Management of variceal bleeding remains the main concern as nearly 85% of patients with NCPF present with variceal bleeding. Endoscopic variceal ligation or sclerotherapy are equally effective in about 90-95% of the patients. Gastric varices are seen in about 25% patients and a bleed from them can be managed with cyanoacrylate glue injection or surgery. Other indications for surgery include failure of endoscopic therapy to control acute bleed and symptomatic hypersplenism. The prognosis of patients with NCPF is good and 5-years survival rates in patients in whom variceal bleeding can be controlled is about > 95%.     

Portal ductopathy: clinical importance and nomenclature

Non-cirrhotic portal hypertension(PHT)accounts for about 20%of all PHT cases,portal vein thrombosis(PVT) resulting in cavernous transformation being the most common cause.All known complications of PHT may be encountered in patients with chronic PVT.However,the effect of this entity on the biliary tree and pancreatic duct has not yet been fully established.Additionally,a dispute remains regarding the nomenclature of common bile duct abnormalities which occur as a result of chronic PVT.Although many clinical...     

肝硬化门脉高压患者血红素氧合酶——一氧化碳系统与血浆内皮素关系的初步研究

目的探讨肝硬化门静脉高压时HO—CO系统及ET变化，以及这两种血管活性物质之间是否存在相关性。方法试验对象分为3组：健康对照组20人，中、重度慢性肝炎患者组20人及肝硬化门脉高压组26人。运用双波长紫外分光比度法测定全血COHb百分比浓度以间接反映HO—CO系统水平，同时用放射免疫学方法检测血浆ET-1水平，了解二者与肝硬化门脉高脉的关系及二者之间的关系。结果肝硬化门脉高压组的HO—CO水平显著高于对照组及慢性肝炎组（P〈0．05），而慢性肝炎组与对照组之间的HO—CO水平无娃著差异（P〉0．05）。肝硬化门静脉高压组的血浆ET-1水平明显高于对照组及慢性肝炎组（P〈0．05），且慢性肝炎组的血浆ET—1水平较对照组亦显著升高（P〈0．05）。肝硬化门静脉高压组的HO—CO系统水平和血浆ET水平呈正相关（P〈0.05）。慢性肝炎组的HO—CO系统水平和血浆ET水平无明显相关性（P〉0．05）。结论HO-CO系统在肝硬化门静脉高压中发挥重要作用，但与慢性肝纤维化的程度无明显关系。血浆ET在肝硬化门静脉高压中发挥重要的生物学效应，并且与肝脏纤维化程度有关系。肝硬化门静脉高压患者的HO-CO系统水平与血浆ET水平呈正相关关系，两者共同参与维持肝硬化门静脉高压的高动力循环状态。     

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。