Purpose of the study. Estimation of the parameters of matrix metalloproteinases system (MMP-1, MMP-9, TIMP-1) in patients with connective tissue dysplasia (DST). Material and methods. 82 patients (64 males and 18 females, average age 21.96 ± 4.47 years) with DST were examined. The criteria for inclusion in the study were: DST in patients of both sexes, 18-35 years old who have signed informed consent of participation in the study. MMP-1, MMP -9 and TIMP-1parameters in blood serum of connective tissue dysplasia patients were studied. Dysplastic syndromes and phenotypes were represented by primary myxomatous (n = 14) and familial mitral valve prolapse (n = 14), sporadic mitral valve prolapse syndrome (n = 49), unclassifiable phenotype (n = 1), increased dysplastic stigmatization (n = 4). To assess the balance between MMP and their inhibitor, the MMP-1 / TIMP-1 and MMP-9/TIMP-1 coefficients were studied. Results. It was fixed an increase in MMP-1, MMP-9, MMP-1 / TIMP-1 and MMP-9 / TIMP-1 coefficients in case of DST. More expressed deviations in MMP system are recorded in groups with bone-joint dysplastic manifestations, as well as with myxomatous degeneration of the prolabsing mitral valve and hemodynamically significant mitral regurgitation. Conclusion. Serum indexes of MMP-1 and -9, as well as their imbalance with TIMP-1, can be used as laboratory tests to evaluate remodeling of connective tissue structures in DST.

Keywords: connective tissue dysplasia, matrix metalloproteinase-1, matrix metalloproteinase-9, tissue inhibitor of matrix metalloproteinase-1