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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 IPH patients admitted to the 302 Hospital of PLA from January 2012 to December 2016 were retrospectively analyzed. Results Among the 21 patients with IPH, the male to female ratio was 6:15, with an average age of onset of (38.1 ± 12.7) years. Clinical manifestations of portal hypertension were mainly liver function without any significant decrease. The main complications were upper gastrointestinal bleeding and ascites. Twenty-one cases of liver histopathology mainly showed hepatocyte platelet arrangement with no pseudolobule formation, enlarged portal area, fibrosis around portal vein, different degree of cell infiltration around portal vein, vascular disruption, dilation of central vein and interlobular vein, Sinusoidal dilatation, sinus fibrosis. Conclusion IPH patients with portal hypertension and liver dysfunction are not parallel, severe portal hypertension, the diagnosis still need to pathological examination, treatment to control complications based.