Division of Liver Diseases, Department of Medicine, The Mount Sinai Medical Center, New York, NY, USA.
Virchows Arch. 2011 Feb;458(2):231-5. doi: 10.1007/s00428-010-1004-7. Epub 2010 Nov 6.
Hepatoportal sclerosis (HPS) is one of several entities known to cause noncirrhotic portal hypertension. To date, its etiology is unknown. There have been increasing reports of HPS occurring in patients with human immunodeficiency virus (HIV), and the US Food and Drug Administration (FDA) recently issued an advisory regarding the development of noncirrhotic portal hypertension in association with didanosine (ddI) use. We report on a patient with HIV who had taken ddI for 4 years and who developed portal hypertension. Histopathological review of paired liver biopsies showed an initial drug hepatotoxicity, microvascular liver injury, and the presence of HPS. Despite cessation of ddI, the latter biopsy showed resolution of the drug-induced injury, but it also showed progression of the HPS. The patient's portal hypertension also progressed suggestive of an unremitting vascular injury. This case demonstrates the development of HPS resulting from a drug-induced microvascular injury. The paired biopsies demonstrate that the initial vascular injury may disappear but that the portal hypertension and HPS progress.
肝门静脉硬化症（HPS）是引起非肝硬化性门静脉高压症的几种病症之一。迄今为止，其病因尚不清楚。越来越多的报道称，人类免疫缺陷病毒（HIV）患者会出现 HPS，美国食品和药物管理局（FDA）最近发布了一项关于与使用叠氮胸苷（ddI）相关的非肝硬化性门静脉高压症发展的咨询。我们报告了一例 HIV 患者，该患者服用 ddI 已有 4 年，且出现了门静脉高压症。对配对肝活检的组织病理学检查显示，最初存在药物肝毒性、微血管肝损伤和 HPS。尽管停止了 ddI 的使用，但第二次活检显示药物引起的损伤已经消退，但也显示出 HPS 的进展。患者的门静脉高压也有所进展，提示存在持续的血管损伤。该病例表明，HPS 是由药物引起的微血管损伤所致。配对活检显示，最初的血管损伤可能消失，但门静脉高压和 HPS 仍在进展。
