[Gaucher's disease type I: an infrequent cause of portal hypertension].
Portal hypertension associated to Gaucher's disease type I with non cirrhotic liver involvement is a very in-equent finding. We report a case of Gaucher's disease type I without cirrhosis in which portal hypertension is the main manifestation. The mechanism of the portal hypertension is discussed in our report and can be related to an elevation in hepatic fluid resistance due to the Gaucher's cells compression on sinusoids.
Adult↗