Cystic medial necrosis of aorta
Other Names: Erdheim cystic medial necrosis of aorta; Erdheim disease
Familial aortic dissection is the term used to describe rupture of the aortic wall at the level of the media, resulting in the formation of a false channel and deviation of part of the aortic flux. Familial predisposition to thoracic aortic aneurysms and type A dissections (concerning the ascending aorta and/or the aortic arch) has been demonstrated in around 19% of patients presenting with thoracic aortic dissections and several loci have been identified so far (16p12.2-p13.13, 3p24-25). This predisposition is transmitted in an autosomal dominant manner.
NIH genetic and rare disease info[edit source]
Cystic medial necrosis of aorta is a rare disease.
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