Diagnosis and treatment of portal hypertension caused by rare diseases / 临床肝胆病杂志

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.