Diagnosis and treatment of portal hypertension caused by rare diseases
10.3969/j.issn.1001-5256.2019.01.005
- VernacularTitle:少见、罕见疾病所致门静脉高压症的诊断与治疗
- Author:
Yu WANG
1
;
Min WANG
;
Xiaojuan OU
Author Information
1. Liver Research Center, Beijing Friendship Hospital, Capital Medical University & National Clinical Research Center for Digestive Diseases, Beijing 100050, China
- Publication Type:Research Article
- Keywords:
hypertension, portal;
rare diseases;
diagnosis;
therapy
- From:
Journal of Clinical Hepatology
2019;35(1):29-32
- CountryChina
- Language:Chinese
-
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.
