Orozco H, Takahashi T, García-Tsao G, Mercado M A, Diliz H, Hernández-Ortiz J
División de Cirugía, Clinica de Hipertensión Portal, Delegación Tlalpan, México, D.F.
Rev Invest Clin. 1991 Jan-Mar;43(1):80-6.
Patients with portal hypertension without a demonstrable cause have been reported in the literature under several different terms, such as tropical splenomegaly, phlebosclerosis, obliterative portal venopathy of the liver, hepatoportal sclerosis, noncirrhotic portal fibrosis and idiopathic portal hypertension (IPH). Such patients have been described worldwide, with a greater frequency in India and Japan. The etiology of IPH is still unknown, although some of the theories that have been proposed are: exposure to toxic substances or drugs, relationship with the hepatitis-B virus, immunologic abnormalities, systemic or intra-abdominal infections and clotting abnormalities. The main histopathologic findings are periportal fibrosis, obliteration of small portal veins and sclerosis of the interhepatic portal system. Although these abnormalities could be secondary to portal hypertension, it has been proposed that the vascular changes are the primary event that leads to portal hypertension. The site of increased resistance in IPH is found at the presinusoidal level with some component at the sinusoidal and postsinusoidal level. The main symptoms and signs in IPH are upper gastrointestinal tract bleeding secondary to esophago-gastric varices, symptoms related to anemia, and splenomegaly. The long-term prognosis for patients with IPH is better than for cirrhotic patients, with a 77% survival at ten years. Variceal bleeding is the main cause of death, and some treatment to prevent bleeding or its recurrence is warranted. Although no comparative trial has been performed in IPH patients, the surgical management could be the first choice for elective treatment in these patient without liver failure, because of the high re-bleeding rates with chronic sclerotherapy. Pharmacologic management could be considered for prophylactic treatment of these patients.
文献中报道了一些门静脉高压但病因不明的患者，他们被冠以多种不同的术语，如热带脾肿大、静脉硬化、肝门脉闭塞性病变、肝门静脉硬化、非肝硬化性门静脉纤维化和特发性门静脉高压（IPH）。世界各地均有此类患者的描述，在印度和日本更为常见。尽管已提出的一些理论认为，IPH的病因可能是接触有毒物质或药物、与乙肝病毒的关系、免疫异常、全身或腹腔内感染以及凝血异常，但其病因仍不明确。主要的组织病理学发现为门周纤维化、小门静脉闭塞以及肝内门静脉系统硬化。虽然这些异常可能是门静脉高压的继发性表现，但也有人提出血管变化是导致门静脉高压的原发性事件。IPH阻力增加的部位在窦前水平，同时在窦内和窦后水平也有一定影响。IPH的主要症状和体征包括食管胃静脉曲张继发的上消化道出血、与贫血相关的症状以及脾肿大。IPH患者的长期预后优于肝硬化患者，十年生存率为77%。静脉曲张出血是主要死因，因此有必要采取一些预防出血或其复发的治疗措施。尽管尚未在IPH患者中进行比较试验，但由于慢性硬化疗法的再出血率较高，手术治疗可能是这些无肝功能衰竭患者选择性治疗的首选。可考虑对这些患者进行药物预防治疗。
