Sarin Shiv K, Khanna Rajeev
Department of Hepatology, Institute of Liver and Biliary Sciences, D-1 Vasant Kunj, New Delhi 110070, India.
Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, D-1 Vasant Kunj, New Delhi 110070, India.
Clin Liver Dis. 2014 May;18(2):451-76. doi: 10.1016/j.cld.2014.01.009.
Non-cirrhotic portal hypertension (NCPH) encompasses a wide range of disorders, primarily vascular in origin, presenting with portal hypertension (PHT), but with preserved liver synthetic functions and near normal hepatic venous pressure gradient (HVPG). Non-cirrhotic portal fibrosis/Idiopathic PHT (NCPF/IPH) and extrahepatic portal venous obstruction (EHPVO) are two prototype disorders in the category. Etiopathogenesis in both of them centers on infections and prothrombotic states. Presentation and management strategies focus on repeated well tolerated episodes of variceal bleed and moderate to massive splenomegaly and other features of PHT. While the long-term prognosis is generally good in NCPF, portal biliopathy and parenchymal extinction after prolonged PHT makes outcome somewhat less favorable in EHPVO. While hepatic schistosomiasis, congenital hepatic fibrosis and nodular regenerative hyperplasia have their distinctive features, they often present with NCPH.
非肝硬化性门静脉高压症（NCPH）涵盖多种疾病，主要起源于血管性疾病，表现为门静脉高压（PHT），但肝脏合成功能保留且肝静脉压力梯度（HVPG）接近正常。非肝硬化性门静脉纤维化/特发性门静脉高压症（NCPF/IPH）和肝外门静脉阻塞（EHPVO）是该类别中的两种典型疾病。它们的病因发病机制均以感染和血栓前状态为中心。临床表现和管理策略集中于静脉曲张出血的反复发作且耐受性良好、中度至重度脾肿大以及门静脉高压的其他特征。虽然NCPF的长期预后通常良好，但门静脉胆管病以及长期门静脉高压后的实质萎缩使得EHPVO的预后略差。虽然肝血吸虫病、先天性肝纤维化和结节性再生性增生有其独特特征，但它们常表现为非肝硬化性门静脉高压症。
