Giner-Galvañ Vicente*, Vicente-Navarro David, Sanz-García Javier and Esteban-Giner María Jose
Department of General Internal Medicine, Hospital Mare de Déu dels Lliris, SpainFulltext PDF
Recent obtention of different specific drugs for the management of Gaucher disease and Fabry disease, the two more prevalent lysosomal storage diseases, in the last years, is changing the interest form just those clearly related manifestations to another new emergence tones. Information is very scarce, although it seems that, probably as a consequence of macrophages activation secondary to lysosomal deposit, cardiovascular events are a main determinant of life expectancy in both entities. Cardiovascular events in gaucher disease are related to an increased insulin resistance status, and in Fabry disease there is a direct endothelial deposit with special protagonism for kidney function deterioration. In this increased cardiovascular risk ambient it is highly surprising the absence of basic information about blood pressure. Probably it is now time to take care on blood pressure and some other basic aspects of general health in these complex entities in parallel with the obtention and improvement of new specific drugs and pharmacological strategies.
Vicente G-G, David V-N, Javier S-G, María Jose E-G. Cardiovascular Disease and Hypertension in Lysosomal Diseases. Ann Hypertens. 2018;1(2): 008.