Clinical Medicine and Hepatology Unit, University Campus Bio-Medico of Rome, 00128 Rome, Italy.
World J Gastroenterol. 2011 May 28;17(20):2580-4. doi: 10.3748/wjg.v17.i20.2580.
Non-cirrhotic portal hypertension is a poorly understood condition characterized by portal hypertension in the absence of conventional hepatic cirrhosis and described in association with blood coagulation disorders, myeloproliferative and immunological diseases and with exposure to toxic drugs. Very recently, precise classification criteria have been proposed in order to define four distinct subcategories. The present case highlights how the clinical presentation, the confounding results from imaging studies, and the difficulties in the histological evaluation often render cases of non-cirrhotic portal hypertension a real diagnostic challenge. It also underscores the classification problems which can be faced once this diagnosis is performed. Indeed, the different subcategories proposed result from the prevalent subtypes in a spectrum of hepatic regenerative responses to a variety of injuries determining microcirculatory disturbances. More flexibility in classification should derive from this etiopathogenic background.
非肝硬化性门静脉高压是一种发病机制尚不清楚的疾病，其特征为存在门静脉高压而无常规的肝硬化，并与凝血功能障碍、骨髓增生性和免疫性疾病以及接触毒性药物有关。最近，人们提出了精确的分类标准，以定义四个不同的亚类。本病例突出了非肝硬化性门静脉高压的临床表现、影像学研究结果的干扰以及组织学评估的困难如何常常使这类病例成为真正的诊断挑战。它还强调了一旦做出这种诊断，就可能面临的分类问题。事实上，所提出的不同亚类源于肝再生反应谱中各种损伤导致微循环障碍的主要亚型。这种病因发病机制应使分类更具灵活性。
