Abstract
A comparative clinic-morphological analysis of 7 dead patients aged from 2.5 months to 35 years was performed in order to investigate patho- and morphogenesis of aortic aneurysms in case of Marfan syndrome. It was established that life-time diagnostics of Marfan syndrome is not always possible owing to rapid development of clinical picture, quite often combined with acute cardio-vascular insufficiency. We noted features of morphological changes in aortic aneurysms in young and grown-up Marfan patients. Cystic medianecrosis, cumulation of smooth myocytes with secretory activity appeared on the areas of aortic aneurysm ruptures in pediatric patients. Outside aortic ruptures contractile smooth myocyte proliferation and connective tissue formation with multiple small vessels was observed in pediatric patients. Aortic cystic medianecrosis alongside with aortic walls hypoplasia, hupoelastosis and minimal signs of atherosclerosis was observed in adult Marfan patients. The acquired data allows to specify aortic aneurysm pathogenesis in Marfan syndrome and treatment strategy.
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