特发性门静脉高压症10例临床分析

Clinical analysis of 10 cases of idiopathic portal hypertension

目的 探讨特发性门静脉高压症（IPH）的临床特点,以提高临床医生对此病的认识,减少误诊、漏诊.方法 对2008年12月至2014年12月我院收住院、经病理诊断为IPH患者共10例,其中男、女患者各5例,平均年龄（38.6±16.1）岁,对其临床及病理资料进行回顾性分析.结果 脾大10例,食管胃底静脉曲张10例,上消化道出血3例,血小板减少9例,贫血6例;肝功能正常7例,轻度异常3例;6例超声和/或CT检查提示肝硬化（10例行超声、7例CT检查）,全部病例的肝组织病理学检查（4例手术治疗、6例为明确病因行穿刺检查）结果为肝小叶结构存在,无假小叶形成,均有不同程度汇管区和门静脉纤维化;8例因门脉高压的并发症及影像学检查提示肝硬化曾被误诊为肝硬化,误诊时间1个月-15年,为明确肝硬化病因及治疗经病理学检查而最终确诊.结论 IPH患者与肝硬化所致的门脉高压症表现相似,而肝组织病理学检查可除外肝硬化,并有汇管区及其周围纤维化和肝内终末门脉分支闭塞,有助于诊断.

Objective To review the clinical characteristics of idiopathic portal hypertension （IPH）.Methods The clinical and pathological data of 10 patients with idiopathic portal hypertension admitted from December 2008 to December 2014 were retrospectively analyzed.Results Among 10 patients 5 were males and 5 females with averaged age of （38.6 ± 16.1） years.There were splenomegaly and esophageal varices in all 10 cases,upper gastrointestinal bleeding in 3 cases,thrombocytopenia in 9 cases and anemia in 6 cases.Liver function was normal in 7 cases,mild abnormality in 3 cases.Ten cases underwent ultrasound examination and 7 cases had CT scan,cirrhosis was suggested with ultrasound/CT scan in 6 cases.Liver histology showed lobular architecture in existence,no false flocculus to form,variable degree of portal fibrosis appeared.Eight cases were misdiagnosed as liver cirrhosis,the duration of misdiagnosis varied from 1 month to 15 years.Conclusions The clinical manifestation of IPH is similar as cirrhosis caused portal hypertension.Liver histopathological examination can exclude liver cirrhosis,and portal fibrosis and liver terminal portal branch occlusion in histopathology is helpful to the diagnosis of IPH.