21例特发性门脉高压临床及病理特点分析

Clinical and pathological features analysis of 21 patients with idiopathic portal hypertension

目的分析特发性门脉高压(idiopathic portal hypertension,IPH)的临床及病理特点。方法回顾性分析2012年1月—2016年12月在解放军第三〇二医院住院治疗(资料完整)的21例IPH患者的临床及病理特点。结果 21例IPH患者中,男女比例6∶15,平均发病年龄(38.1±12.7)岁,临床以门脉高压症表现为主,肝功能无明显减退,主要并发症为上消化道出血及腹水。21例肝组织病理主要表现为肝细胞板排列基本正常,无假小叶形成,汇管区扩大,门静脉周围纤维化,门脉周围有不同程度的细胞浸润,血管紊乱,中央静脉及小叶间静脉扩张,肝窦扩张,窦周纤维化。结论 IPH患者门脉高压和肝功能损害不平行,门脉高压表现较重,确诊仍须病理学检查,治疗以防治并发症为主。

Objective To analyze the clinical and pathological features of idiopathic portal hypertension （IPH）. Methods The clinical and pathological features of 21 patients with IPH in the 302 Military Hospital of China from January 2012 to December 2016 were retrospectively studied. Results There were 6 male and 15 female patients with a mean age of （38.1± 12.7） years old. The main clinical manifestations of IPH were portal hypertension and liver function test had no obvious decline. The main complications were hemorrhage of upper digestive tract and ascites. Liver histopathology of 21 patients showed that the liver plate arrangement was normal, had no pseudolobule formation, portal area expansion, fibrosis surrounding portal vein, different degrees of inflammatory cell infiltration, vascular disturbances, central vein and interlobular vein telangiectasis, liver sinuses dilation, perisinusoidal space fibrosis. Conclusions With sever portal hypertension and mild liver function damage, the diagnosis of IPH patients is depended on liver biopsy. Treatment shouhl be based on prevention and treatment of complications.