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.     

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。     

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。     

特发性门静脉高压的肝脏病理学分析

耳的观察特发性门静脉高压的肝脏病理改变,明确病理诊断标准,并探讨临床病理联系。方法收集中日友好医院2005年1月-2007年3月病理确诊的特发性门静脉高压病例29例,对其肝组织行HE、网织纤维加Masson三色染色,以及α-平滑肌肌动蛋白、细胞角蛋白7、细胞角蛋白19免疫组织化学染色,并分析病变特点。结果29例中男9例,女20例。临床有门静脉高压、脾大等症状、体征。23例临床误诊为肝硬化。主要病理改变有：明显的汇管区纤维化,伴终末门静脉细小分支闭锁（缺乏）及不完全细纤维隔形成。部分门静脉支扩张并疝入小叶内。肝细胞萎缩及结节状再生相伴。结论特发性门静脉高压的肝脏病变具有一定的形态学特征,汇管区纤维化、门静脉小支闭锁,部分门静脉分支疝入肝实质,肝细胞萎缩及结节状再生相伴,较具诊断价值。     

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…     

肝纤维化的血清学诊断

肝纤维化的血清学诊断高锋，孔宪涛肝纤维化是肝硬化的早期阶段，它是一种病理学诊断，特点为汇管区和肝小叶内有大量纤维组织增生和沉积，但尚未形成小叶内间隔。肝硬化有假小叶生成，中心静脉区和汇管区出现间隔，肝的正常结构遭到破坏，即肝纤维化的进一步发展即为肝硬...     

肝炎后肝硬化门脉高压症的处理

正常门脉压为0.98～2.35kPa(100～240mmH_2O),超过2.45kPa(250mmH_2O)时为门脉高压。肝炎后肝硬化是渐进过程,慢活肝时因汇管区炎症严重,约1/3患者有窦前梗阻而致门脉高压,当组织学改变以肝硬化为主时,临床逐渐表现出门脉高压     

肝或肾移植术后纤维化胆汁淤积性肝炎肝组织中CD4+CD25+调节性T淋巴细胞的表达

目的观察肝或肾移植术后纤维化胆汁淤积性肝炎（FCH）患者肝组织中CD4^＋CD25^＋调节性T淋巴细胞的表达及分布,并对其作用机制进行初步探讨。方法对5例FCH患者进行肝活体组织病理学检查;采用免疫组织化学法检测肝组织中CD4^＋CD25^＋调节性T淋巴细胞的特异性标记物叉状头/翅膀状螺旋回转录因子（FOXP3）;采用末端脱氧核苷酸转移酶介导的脱氧三磷酸尿苷缺口末端标记（TUNEL）检测试剂盒对肝组织内的肝细胞凋亡情况进行观察。结果5例FCH患者中,3例为原位肝移植患者,2例为肾移植患者。光学显微镜下,肝脏汇管区及汇管区周围出现纤维化,肝细胞及胆小管明显胆汁淤积,肝细胞气球样变及毛玻璃样变。免疫组织化学检测显示HBsAg、HBcAg及前S1抗原阳性。FOXP3阳性信号定位于淋巴细胞胞质内,阳性细胞主要聚集在汇管区,小叶肝窦内可见散在的单个淋巴细胞呈阳性表达。汇管区周围可见较多的凋亡细胞。正常肝组织HBsAg、HBcAg及前S1抗原均为阴性,汇管区内有少量的阳性CD4^＋CD25^＋调节性T淋巴细胞,小叶内偶见凋亡细胞。结论FCH具有独特的组织学特征,可能与肝组织中的FOXP3高表达有关。     

Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature

BACKGROUND: Non-cirrhotic portal fibrosis (NCPF), the equivalent of idiopathic portal hypertension in Japan and hepatoportal sclerosis in the United States of America, is a common cause of portal hypertension in India. The clinical features, portographic and histological findings, and management of 151 patients with non-cirrhotic portal fibrosis are presented. METHODS: The disease is diagnosed by the presence of unequivocal evidence of portal hypertension in the definite absence of liver cirrhosis and extrahepatic portal vein obstruction (EHPVO). Retrospective analysis of records of 151 patients with NCPF was analyzed for the clinical presentation, physical findings, laboratory tests, radiological and histological findings, and for the outcome of treatment. RESULTS: The disease is characterized by massive splenomegaly with anemia, preserved liver function and benign prognosis in a majority of patients. Splenoportovenography (SPV) showed massive dilatation of the portal and splenic veins, and the presence of collaterals. Twenty-four (15.9%) patients showed evidence of natural/spontaneous shunts (splenorenal 15, umbilical nine) on SPV; these patients had a lower incidence of variceal bleeding. Liver histology demonstrated maintained lobular architecture, portal fibrosis of variable degree, sclerosis and obliteration of small-sized portal vein radicles, and subcapsular scarring with the collapse of the underlying parenchyma. Piecemeal or hepatocytic necrosis was absent in all histology specimens. Three patients showed nodular transformation along with abnormal liver functions, and may represent late manifestation of NCPF where features are similar to those seen in patients with incomplete septal cirrhosis. In the initial part of the study, surgery (side-to-side lieno-renal shunt) was the preferred modality of treatment, however, endoscopic sclerotherapy or variceal ligation has now become the preferred first line of management of variceal bleeding. CONCLUSIONS: The epidemiological and clinical features of NCPF have more similarity to IPH than has previously been documented. The development of spontaneous shunts tends to protect these patients from variceal bleeding.     

Significance of Enhanced Expression of Nitric Oxide Syntheses in Splenic Sinus Lining Cells in Altered Portal Hemodynamics of Idiopathic Portal Hypertension

Idiopathic portal hypertension (IPH) is characterized by noncirrhotic portal hypertension due mainly to increased intrahepatic, presinusoidal resistance to portal blood flow. Marked splenomegaly is always seen in IPH. To clarify the pathogenetic significance of splenomegaly, immunohistochemical expression of inducible nitric oxide synthese (iNOS), endothelial NOS (eNOS), and endothelin-1 (ET-1) in spleens from patients with IPH was examined. Sinus lining cells of IPH spleens showed diffuse and strong expression of iNOS and eNOS. Sinus lining cells of spleens from patients with liver cirrhosis (LC) also showed positive signals for iNOS and eNOS, but the staining intensity was significantly weak. ET-1 was detectable in only a few mononuclear leukocytes in the red pulp of both IPH and LC spleens. These results suggest that NO liberated in spleen, rather than ET-1, is responsible for the dilatation of splenic sinuses, leading to splenomegaly, and thereby contributes to portal hypertension in IPH.     

Liver pathology of idiopathic portal hypertension. Comparison with non-cirrhotic portal fibrosis of India The Japan idiopathic portal hypertension study*,+

ABSTRACT— Morphological changes of the liver were studied in 24 autopsy cases of noncirrhotic portal hypertension of unknown etiology (idiopathic portal hypertension, IPH), and in 123 surgical biopsies from such patients. For comparison, 15 whole-cut liver slices from autopsy cases of noncirrhotic portal fibrosis (NCPF) from India were also studied. Liver pathology was very similar in IPH and NCPF, characterized by phlebosclerotic changes and perivascular fibrosis of the portal vein system, and parenchymal atrophy perhaps secondary to portal circulatory insufficiency. The distribution of lesions was uneven, and despite marked fibrosis and occasional surface nodularity, there was no diffuse pseudonodule formation in the parenchyma. Surgical specimens showed similar changes except for more frequent portal cellular infiltrates, but the changes seen in one biopsy specimen were limited and not always diagnostic. It seems that IPH of Japan and NCPF of India are the same disease, and perhaps hepatoportal sclerosis elsewhere is also the same disease.     

Acute Deterioration of Idiopathic Portal Hypertension Requiring Living Donor Liver Transplantation: A Case Report

Case reports of severe idiopathic portal hypertension (IPH) requiring liver transplantation are very rare. We report the case of a 65-year-old woman who was diagnosed as having IPH. At the age of 60 years, her initial symptom was hematemesis, due to ruptured esophageal varices. Computed tomography of the abdomen showed splenomegaly and a small amount of ascites, without liver cirrhosis. She was diagnosed as having IPH and followed-up as an outpatient. Five years later, she developed symptoms of a common cold and rapidly progressive abdominal distension. She was found to have severe liver atrophy, liver dysfunction, and massive ascites. Living donor liver transplantation was then performed, and her postoperative course was uneventful. Histopathological findings of the explanted liver showed collapse and stenosis of the peripheral portal vein. The areas of liver parenchyma were narrow, while the portal tracts and central veins were approximate one another, leading to a diagnosis of IPH. There was no liver cirrhosis. The natural history of refractory IPH could be observed in this case. Patients with end-stage liver failure due to severe IPH can be treated by liver transplantation.     

Pathomorphological study of HCV antibody-positive liver cirrhosis

A morphological investigation was carried out to study the pathological features of liver cirrhosis caused by hepatitis C virus (HCV) infection. The materials consisted of liver specimens taken from 47 cases of anti-HCV antibody-positive liver cirrhosis (37 by surgery for hepatocellular carcinoma and 10 by autopsy), and from 21 cases of hepatitis B surface antigen-positive liver cirrhosis as the control. Liver specimens containing more than 10 regenerative nodules were examined. In addition, a histometric study was conducted to determine the degree of fibrosis and the size of regenerative nodule using a computer image-analysis system. The results showed that the histological characteristics of HCV antibody-positive liver cirrhosis are: (i) broadly expanded fibrous septa and small regenerative nodules; (ii) relatively strong inflammatory reaction and prominent lymphoid aggretation in the fibrous septum; and (iii) mild regenerative activity of the liver parenchyma, and infrequent liver cell dysplasia. These findings may facilitate better understanding of the pathology of HCV antibody-positive liver cirrhosis and more accurate pathological diagnosis by needle biopsy.     

Intrahepatic portal venopathy and related disorders of the liver

Intrahepatic portal venopathy leads to various entities that are important causes of portal hypertension. Noncirrhotic portal fibrosis (NCPF) occurs in the Indian subcontinent, whereas idiopathic portal hypertension (IPH) occurs in Japan although the pathogenesis and presentation of both are similar. NCPF presents mainly with upper gastrointestinal bleeding; IPH presents with massive splenomegaly. The liver functions are preserved. Wedged hepatic venous pressure is normal, but portal venous pressure is high indicating a presinusoidal block. Patients are best managed with endoscopic therapy or surgery, with better results than in patients with cirrhosis. Nodular regenerative hyperplasia is a histological diagnosis characterized by development of nodules in the liver due to uneven perfusion of the portal venous blood. These patients may develop portal hypertension and if they bleed would require treatment as in NCPF/IPH. Schistosomiasis produces portal hypertension by the development of fibrous tissue around the portal veins as a response to schistosome eggs. Gratifying results have been reported with praziquantel therapy. Rarely sarcoidosis and chronic biliary obstruction may also produce portal venopathy.     

A case of idiopathic portal hypertension after renal transplantation

A case of idiopathic portal hypertension (IPH) developing after renal transplantation is reported. A 33-year-old Japanese male who had undergone renal transplantation 8 years previously was transferred to our hospital because of hematemesis from ruptured esophageal varices. He had no history of any liver disease before the renal transplantation, but had a history of receiving blood transfusion. Abdominal computed tomography (CT) and ultrasonography revealed marked splenomegaly and collateral channels, but no obliteration which might cause portal hypertension in the hepatic or portal vein. No findings suggestive of hepatitis or liver cirrhosis were found either macroscopically on laparoscopy or by liver biopsy. Light microscopic study of the liver biopsy specimen showed mild periportal fibrosis, inconspicuous portal branches in the most peripheral tracts, but no pseudolobule formation or piecemeal necrosis. However collagen deposition was found in the perisinusoidal space and partly in intercellular space on electron microscopy. We consider that the development of portal hypertension in this case is responsible for the collagen deposition, which may be related to the administration of azathioprine after renal transplantation. There are few reports on IPH after renal transplantation, and it is stressed that a lower amount of azathioprine than previously reported may induce IPH under such conditions.