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Cystic medial necrosis of aorta

A thoracic aortic aneurysm characterized by degeneration and fragmentation of ELASTIC FIBERS, loss of smooth muscle cells, and an accumulation of BASOPHILS in non-fibrillar extracellular matrix (ground substance). OMIM: 607086
Also Known As:
Erdheim Cystic medial necrosis of aorta
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Disease Context: Research Results