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Abstract Behçet disease (BD) is a multisystemic inflammatory disorder, which can affect all types of blood vessels. Complications of the disease can be serious and even life threatening especially Thrombotic vasculopathy. Genetic background has been reported to play a decisive role in BD susceptibility and development of its associated hypercoagulable state.This requires further research to understand the coagulable state in these patients. protein Z (PZ) and its genetic polymorphisms have been studied in a variety of thrombotic diseases but these but in an insufficient way in BD. In this study, we evaluated the prevalence of intronic protein Z G79A (rs3024735) and promoter protein Z G-103A (rs3024719) polymorphisms in Egyptian patients with BD by using Real-time PCR TaqMan probes for allelic discrimination of these two PZ SNPs and presence of association between these protein Z polymorphisms and the increased risk of vascular affections and other clinical findings. Our preliminary data revealed that, compared to the GG genotype, the AA and GA PZ G79A genotypes were significantly associated with BD susceptibility and also, they highly associated with occurrence of deep vein thrombosis (DVT) and retinal vascular occlusion. On the other hand, there was no significant association between PZ G-103A genotypes and BD susceptibility |