少见、罕见疾病所致门静脉高压症的诊断与治疗

王宇; 王民; 欧晓娟; 贾继东

doi:10.3969/j.issn.1001-5256.2019.01.005

少见、罕见疾病所致门静脉高压症的诊断与治疗

DOI: 10.3969/j.issn.1001-5256.2019.01.005

首都医科大学附属北京友谊医院肝病中心国家消化系统疾病临床医学研究中心

基金项目:

首都临床特色应用研究与成果推广(Z171100001017037)；北京市卫生系统高层次卫生技术人才学科骨干资助项目(2015-3-003)；

详细信息

中图分类号: R575.2
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出版历程
- 出版日期: 2019-01-20

Diagnosis and treatment of portal hypertension caused by rare diseases

Liver Research Center, Beijing Friendship Hospital, Capital Medical University & National Clinical Research Center for Digestive Diseases, Beijing 100050, China

Research funding:

摘要

摘要: 少见、罕见疾病所致门静脉高压症多属于窦前性(肝前)或肝后性门静脉高压症。因多数为非肝硬化门静脉高压症,故肝静脉压力梯度不能准确反映其门静脉压力,临床诊治存在较大困难。此类疾病种类较多,重点介绍了6种少见、罕见疾病所致门静脉高压症的机理和治疗进展,并就目前的治疗现状进行了总结,希望对提高临床医生对此类疾病的认识有所帮助。
- 高血压,门静脉 /
- 少见病 /
- 诊断 /
- 治疗
Abstract: Portal hypertension caused by rare diseases is mostly presinusoidal (prehepatic) or posthepatic.It is non-cirrhotic portal hypertension in most cases, so hepatic venous pressure gradient cannot accurately reflect the portal venous pressure of such patients, which causes difficulties in clinical diagnosis and treatment.There are many diseases in this category.This article introduces the advances in the pathogenesis and treatment of portal hypertension caused by six rare diseases and summarizes the current status of treatment, in order to help improve the awareness of these diseases among clinicians.
- hypertension, portal /
- rare diseases /
- diagnosis /
- therapy

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参考文献(39)

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