MALIGNANT HYPERTENSION AND HZELLYA-ERDGEYM'S SYNDROME

Hypertension is one of the main factors that contribute to the degeneration of the middle membrane of the aorta as like a disruption of collagen, smooth myocytes and elastic fibers with the development of the dissection of aorta. Hypertension appears approximately in 84% of patients with aortic dissection. Provoking factors for the dissection of aorta are hypertensive crisis, intense physical activity, pregnancy, chest trauma, iatrogenic factors. In the modern classifications malignant hypertension is not isolated, but the term is used to refer the severe fast-progressive flow of arterial hypertension with the characteristic changes in the ocular fundus. Malignant course can have primary (up to 3% of cases) and secondary (symptomatic) arterial hypertensions (20-25% of cases). As primary hypertension occurs much more often than secondary, it becomes the main source of malignant hypertension. Among the causes of the dissection of aorta by far in patients of the working age (30-50 years) determine Hzellya-Erdheim's syndrome – the dissection of aorta due to its medianecrosis with no signs of atherosclerotic lesion of the vessels wall. In recent years medianecrosis of aorta became the “leader” among nosological forms of the dissection of aorta in young people, having outstripped by the number of cases of Non-specific aortoarteritis and Marfan's syndrome. The article presents the fatal case of Hzellya-Erdheim's syndrome with the total lesion of aorta. During the autopsy the main pathological changes were noted from aorta, heart and kidneys. The whole space of aorta (from the ascending department to the abdominal department inclusively) is with the signs of separation of its wall; stratified space is filled with cherry clots. Histopathologically in the middle membrane of the ascending aorta are presented degenerative changes in elastic fibers with insignificant petrification, in the middle membrane are observed single lymphocytes, the adventitia around vasa vasorum includes slight lymphocytic-macrophage infiltration; on the boarders of external and medium membrane are observed sharply expressed degenerative changes in the walls and the areas of the dissection, in thickness of which are erythrocytes. Slightly thickened intima by mucoid swelling with single lymphocytes. Pathohistologically large and medium-sized branches of the renal artery with sharply narrowed gap due to the thickened intima as a result of the mucoid oedema in large branches and hyperplasia of connective tissue in the middle ones. Arched and interlobular arteries are also with sharply narrowed gap via the connective tissue, muscular- fibrous hyperplasia. Internal elastic membrane with signs of multiplication. In some interlobular arteries is observed circular intimal hyperplasia by the type similar to “proliferative endarteritis”. In the gap of some arterioles are observed thrombotic masses. Glomeruli with small features of decreasing, with thickened glomerular basement membranes (ischemic glomerulopathy). Huge part of glomeruli is globally sclerosed. Tubules are atrophic, interstitial sclerosis.