Kardiovaskularni postizeni u Fabryho nemoci s durazem na endotelialni dysfunkci.

Abstract

The goal of this project was to assess the degree of endothelial dysfunction in the presence of morphological changes and to evaluate the extent of cardiovascular impairment. Besides mapping of cardiovascular symptoms and assessing the degree of organ involvement, we have evaluated several markers reflecting endothelial function. The evaluation of early stages of the disease by establishing endothelial function markers is difficult and our results in most of them did not give significant findings. The best correlation between cardiovascular impairment and the evaluated levels was found in endothelaemia, endotelin-1 level, levels of orosomucoid and fibrinogen. Cardiovascular involvement is most typically characterized by the concentric left ventricle hypertrophy and its remodelling. Even in the presence of the fact that all symptoms and organ involvements have been extensively studied in this project and had possibility to be detected early there is no casual therapy for these patients available and their prognosis stays serious.Summary in English. Text also in English.Available from STL, Prague, CZ / NTK - National Technical LibrarySIGLECZCzech Republi