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特发性门静脉高压(IPH)是指原因不明的脾肿大、脾功能亢进,但从不发展成为肝硬化的门静脉高压.以往有人提出由于免疫损害、肝炎病毒感染、疟疾、暴露于氯乙烯及慢性砷摄入等假说.最近,作者通过经皮经肝门静脉导管检查和肝活检发现有两姐妹患IPH,首先证明了IPH的家族发病性.例1:患者女性,43岁.因腹水和全身不适入院.两年前诊断为缺铁性贫血.体检:有贫血,无肝硬化的皮肤体征.肝未扪及,脾左助下1指.实验室检查示全血细胞减少和肝功能轻度异常.内镜检查食管和胃无静脉曲张.超声波检查示肝脏表面光滑,门静脉和脾静脉
Idiopathic portal hypertension (IPH) refers to the unexplained splenomegaly and hypersplenism, but never develops into cirrhosis of the portal hypertension.People have been proposed as a result of immune damage, hepatitis virus infection, malaria, exposure to vinyl chloride and Chronic arsenic ingestion, etc. Recently, the authors identified two siblings suffering from IPH by percutaneous transhepatic portal venous catheterization and liver biopsy, first demonstrating the familial onset of IPH.Example 1: Female patient, age 43, due to ascites and systemic Not suitable for admission. Two years ago, the diagnosis of iron deficiency anemia. Physical examination: anemia, no signs of cirrhosis of the skin. Liver palpable, spleen left help 1 finger. Laboratory tests showed mild pancytopenia and abnormal liver function Endoscopic examination of the esophagus and stomach without varicosis Ultrasonography showed a smooth surface of the liver, portal vein and splenic vein