Homocysteine and tissue factor pathway inhibitor levels in patients with Fabry's disease

In this study, TFPI levels have been found to be low in allthe patients so suggesting a diffuse endothelial alterationsassociated to the disease per se. Tissue factor pathwayinhibitor plays a primary role in regulating TF-inducedcoagulation and significantly contributes to the anticoagulantproperties of the endothelium [16]. Tissue factor pathwayinhibitor decrease has been reported in conditions in whichblood clotting activatio n occurs [17]. Low levels of plasmaTFPI in Fabry’s disease may be attributed to the endothelialdysfunction and/or consumption of the endothelial cell-associated TFPI owing to the proinflammatory and proco-agulant profile of the Fabry patients. TF may have a role indetermining the procoagulant profile to which other mecha-nisms participate. In conclusion, the present study shows thatHcy may have a role in the occurrence of cerebrovascularevents in Fabry patients and that reduced vitamin availabilityis involved. The possible utility of checking for Hcy andvitamin levels, in particular in the presence of gastrointestinal events, and the opportunity of vitamin administrationdeserves confirmation in a larger group of patients withFabry’s disease.