Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection

The article presents the pathologic changes in the aortic wall in patients with MFS (three men at the age of 25, 27, 35 years) operated on the aneurysm with dissection of the ascending aorta. Morphological changes in the middle of the shell of the aorta in patients with MFS were characterized by sev...

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Main Authors: R. K. Zhuraev, P. V. Kuzyk
Format: Article
Language:English
Published: Zaporizhzhia State Medical and Pharmaceutical University 2012-08-01
Series:Patologìâ
Subjects:
Online Access:http://pat.zsmu.edu.ua/article/view/16745/14266
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author R. K. Zhuraev
P. V. Kuzyk
author_facet R. K. Zhuraev
P. V. Kuzyk
author_sort R. K. Zhuraev
collection DOAJ
description The article presents the pathologic changes in the aortic wall in patients with MFS (three men at the age of 25, 27, 35 years) operated on the aneurysm with dissection of the ascending aorta. Morphological changes in the middle of the shell of the aorta in patients with MFS were characterized by severe restructuring with profound irreversible alterative changes in all of its components. The pathological process captured media of the aorta throughout its entire length, not just in the areas of rupture and separation. The main pathomorphological signs of MFS were the focal accumulation of mucoid substances, ribbon-like nuclear-free zones, degenerative changes in smooth muscle cells, the formation of cystic media degeneration cavities, changes in elastic fibers - fragmentation, hyperelastosis, multiplication, thinning and straightening, areas of significant elastolysis. Multiple CMD was detected in all patients with MFS, the changes revealed the same type, but at different stages of the process.
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institution Kabale University
issn 2306-8027
2310-1237
language English
publishDate 2012-08-01
publisher Zaporizhzhia State Medical and Pharmaceutical University
record_format Article
series Patologìâ
spelling doaj-art-6a766e9fa2644c7e9245ca0129629d6f2025-08-20T03:33:42ZengZaporizhzhia State Medical and Pharmaceutical UniversityPatologìâ2306-80272310-12372012-08-012464910.14739/2310-1237.2012.2.16745Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissectionR. K. ZhuraevP. V. KuzykThe article presents the pathologic changes in the aortic wall in patients with MFS (three men at the age of 25, 27, 35 years) operated on the aneurysm with dissection of the ascending aorta. Morphological changes in the middle of the shell of the aorta in patients with MFS were characterized by severe restructuring with profound irreversible alterative changes in all of its components. The pathological process captured media of the aorta throughout its entire length, not just in the areas of rupture and separation. The main pathomorphological signs of MFS were the focal accumulation of mucoid substances, ribbon-like nuclear-free zones, degenerative changes in smooth muscle cells, the formation of cystic media degeneration cavities, changes in elastic fibers - fragmentation, hyperelastosis, multiplication, thinning and straightening, areas of significant elastolysis. Multiple CMD was detected in all patients with MFS, the changes revealed the same type, but at different stages of the process.http://pat.zsmu.edu.ua/article/view/16745/14266Marfan syndromeaortic dissectionpathomorphologyprognosisdiagnosis
spellingShingle R. K. Zhuraev
P. V. Kuzyk
Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection
Patologìâ
Marfan syndrome
aortic dissection
pathomorphology
prognosis
diagnosis
title Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection
title_full Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection
title_fullStr Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection
title_full_unstemmed Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection
title_short Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection
title_sort marfan syndrome pathologic features of aneurysm with dissection and aortic dissection
topic Marfan syndrome
aortic dissection
pathomorphology
prognosis
diagnosis
url http://pat.zsmu.edu.ua/article/view/16745/14266
work_keys_str_mv AT rkzhuraev marfansyndromepathologicfeaturesofaneurysmwithdissectionandaorticdissection
AT pvkuzyk marfansyndromepathologicfeaturesofaneurysmwithdissectionandaorticdissection