Non-cirrhotic portal hypertension versus idiopathic portal hypertension

Portal hypertension occurs in a number of disorders other than cirrhosis and they are collectively called non-cirrhotic portal hypertension (NCPH). The common causes of NCPH include idiopathic portal hypertension (IPH), non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous thrombosis (EHPVT). Other causes include schistosomiasis, hepatic venous outflow tract obstruction, veno-occlusive disease and congenital hepatic fibrosis. Patients with IPH and EHPVT present with upper gastrointestinal bleeding, splenomegaly, ascites after gastrointestinal bleeding, features of hypersplenism, growth retardation and jaundice due to portal biliopathy. The diagnosis is usually made by abdominal ultrasound, upper gastrointestinal endoscopy, normal liver function tests and normal liver histology. Variceal bleeding in NCPH has lower mortality as compared with cirrhosis because of better liver functions in NCPH. Treatment for NCPH includes primary prophylaxis for variceal bleeding and prevention of repeat bleeding using drugs like beta-blockers, endoscopic sclerotherapy and endoscopic band ligation of varices. In patients with uncontrolled variceal bleeding or symptomatic hypersplenism, porto-systemic shunt surgery or splenectomy are required.     

Non-cirrhotic portal hypertension

A chronic increase of the portal venous pressure is not only a sequel of liver cirrhosis. There is a large group of different diseases leading to "non-cirrhotic portal hypertension". Clinical presentation, diagnosis and treatment are discussed. The discrimination between cirrhotic and non-cirrhotic portal hypertension is important for the understanding of differences in clinical signs and course of the diseases, however.     

Noncirrhotic portal hypertension

Portal hypertension is characterized by an increase in portal pressure (>10 mm Hg) and could be a result of cirrhosis of the liver or noncirrhotic diseases. Noncirrhotic portal hypertension (NCPH), as it generally is termed, is a heterogeneous group of diseases that is due to intrahepatic or extrahepatic etiologies. In general, the lesions in NCPH are vascular in nature and can be classified based on the site of resistance to blood flow. Noncirrhotic portal fibrosis and extrahepatic portal vein obstruction are two diseases that are common in developing countries; they most often present only with features of portal hypertension and not of parenchymal dysfunction. These are described in detail.     

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。     

Noncirrhotic portal hypertension

This article reviews the different conditions leading to noncirrhotic intrahepatic portal hypertension, describes the related vascular lesions, and provides a review of the clinical characteristics, diagnosis, and treatment options available. Diseases associated with noncirrhotic portal hypertension are also specifically discussed.     

Noncirrhotic portal hypertension

Noncirrhotic portal hypertension represents a heterogeneous group of conditions that have distinct clinical and hemodynamic features that often help distinguish them from cirrhosis. [figure: see text] The sites of portal flow resistance may not be precisely localized to one area of the hepatic lobule and may extend beyond the site where the pathogenetic process began. Even in patients with portal hypertension caused by an increased flow, there may be subsequent development of increased resistance. The prognosis is variable; outcomes are better in patients with presinusoidal portal hypertension. A good understanding of the presentation of the various noncirrhotic conditions that cause portal hypertension will help determine the cause, the site of resistance, and the therapeutic plan. Ascites is not a feature of presinusoidal portal hypertension, whereas it may be the predominant feature in postsinusoidal portal hypertension.     

胰源性区域性门脉高压症

区域性门脉高压症 (ＲｅｇｉｏｎａｌＰｏｒｔａｌＨｙｐｅｒｔｅｎｓｉｏｎ ,ＲＰＨ) ,亦有称为“左侧门脉高压”、“局限性门脉高压”等 ,占肝外型门脉高压症的 5% ,但却是唯一可治愈的门脉高压症[1] 。据病因ＲＰＨ可分为胰源性、脾源性和腹膜后源性三类 ,其中以胰源性最为常见[2 ] 。Ｓｕｔｔｏｎ等复习了 190 0～ 196 7年、Ｍａｄｓｏｎ等复习了 196 8～ 1984年间英国文献 ,胰源性ＲＰＨ分别为 52 %及 73% [1] ;施宝民等[2 ] 复习 1998年前 10年国内文献 ,加上自己的 7例共 6 0例 ,胰源性占 4 7例 (86 7% )。据本文检索1998～ 1999两…     

Pulmonary hypertension complicating portal hypertension.

We report 9 patients with pulmonary hypertension complicating portal hypertension. The cause of portal hypertension was cirrhosis in 7 patients, nodular regenerative hyperplasia of the liver in 1, and portal vein obstruction in 1. Six patients had been treated by portal-systemic shunting before the clinical onset of pulmonary hypertension. The interval between the first manifestation of portal hypertension and the recognition of pulmonary hypertension ranged from 2 to 15 years. Histologic examination in 1 of these patients revealed medial hypertrophy, concentric intimal proliferation, and plexiform lesions affecting the small pulmonary arteries. Pulmonary hypertension might result from the effect of a vasoconstrictive agent on the small pulmonary arteries or of a substance toxic to the walls of these vessels that is produced in the splanchnic territory, destroyed by the liver in normal subjects, and reaches the pulmonary arteries through portal-systemic shunts in these patients.     

Somatostatin in portal hypertension

The effect of somatostatin on portal pressure is mediated by splanchic arterial vasoconstriction which induces a reduction in portal blood flow and pressure. One of the most important characteristics of somatostatin is that its splanchic effect is not accompanied by major systemic hemodynamic effects. Somatostatin has been used in several controlled trials to test its potential in controlling acute variceal bleeding. The results remain controversial. Different findings in existing clinical trials may derive in part from distinct protocols for somatostatin administration. Published trials suggest that somatostatin may be as effective as vasopressin in the acute management of variceal bleeding. However, since the efficacy of vasopressin has been questioned, a comparison of two potentially ineffective drugs cannot establish definitively the efficacy of somatostatin in controlling variceal bleeding. The most significant finding of the two published studies has been the lower incidence of minor and major complications with somatostatin when compared to vasopressin. Newer trials in progress may shed new light into the potential use of somatostatin for the treatment of variceal bleeding.     

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。     

Ultrasonography in portal hypertension

High resolution real time ultrasound is a non-invasive method of evaluating patients with suspected portal hypertension. The portosystemic collateral most frequently identified is the dilated coronary vein and its associated gastro-oesophageal varices. Other collaterals that can be seen include: gastrorenal, splenorenal, paraduodenal, periportal, pelvic and retroperitoneal varices along with a recanalized umbilical vein and ductus venosus. Using duplex doppler ultrasound, the rate and direction of portal blood flow can be ascertained. Sonography is better than barium studies in assessing whether gastro-oesophageal varices are present, however, it is not as sensitive as endoscopy, laparoscopy or portography.