特发性门静脉高压症的临床分析  

Clinical analysis of idiopathic portal hypertension

作　　者：程浩[1] 卢成林[1] 潘一明[1] 包善华[1] 谢敏[1] 

机构地区：[1]南京大学医学院附属鼓楼医院普外科,江苏南京210008

出　　处：《现代医学》2015年第10期1287-1290,共4页Modern Medical Journal

摘　　要：目的:探讨特发性门静脉高压症(IPH)的临床特点及诊治方法。方法:回顾性分析11例IPH患者的临床资料。结果:患者的主要临床表现为脾脏肿大(11例),腹水(7例),反复呕血、黑便(6例),合并有免疫系统疾病(5例)。辅助检查提示:所有患者肝功能基本正常,且无肝硬化表现;脾功能亢进9例;食管胃底静脉曲张10例;血管异常8例。4例行肝活检,病理学检查均未见明显特异性改变。5例行手术治疗,3例行内镜下曲张静脉套扎和(或)硬化剂治疗,1例行胃底曲张静脉栓塞术,2例行内科保守治疗。结论:临床上对于肝功能正常、无肝硬化表现,尤其是合并有免疫系统相关性疾病的门脉高压症患者,应高度怀疑IPH的可能。彩色多普勒超声对IPH的诊断有重要意义,但病理学结果仍是IPH必要的诊断依据。外科手术、内镜下治疗、介入方法都可用于IPH患者的治疗。Objective： To analyze the clinical features,diagnosis and treatment of idiopathic portal hypertension（ IPH）. Methods： The clinical data of 11 patients with IPH were retrospectively analyzed. Results： The main clinical manifestations were splenomegaly（ 11 cases）,ascites（ 7 cases）,repeated hematemesis or melena（ 6 cases）,and 5 cases were complicated with the diseases of immune system. The liver function of all the patients were almost normal and no one had the manifestations of liver cirrhosis. Hypersplenism was present in 9 cases and gastroesophageal varices was present in 10 cases. Eight cases had vasculopathy. Four cases were performed with liver biopsy,but pathological findings were normal. Surgery was performed in 5 cases and endoscopic variceal ligation（ EVL） and / or endoscopic sclerotherapy（ EST） were performed in 3 cases,1 case was treated with percutaneous transhepatic obliteration of gastric fundal varices and 2 cases were treated with conservative treatment.Conclusion： IPH should be considered highly when the portal hypertension patients are present with the normal liver function and absense of liver cirrhosis,especially complicated with the diseases of immune system. The color doppler ultrasonography is significant to the diagonosis of IPH,but the pathological findings are necessary. The surgical operation,endoscopic therapy and interventional operation are all effective to the patients with IPH.

关 键 词：特发性门静脉高压症 免疫性疾病 诊断 治疗 

分 类 号：R657.34[医药卫生—外科学]