Spontaneous perforation of small intestine followed by rupture of the cystic artery: the natural history of Vascular Ehlers-Danlos Syndrome

Spontaneous perforation of small intestine followed by rupture of the cystic artery: the natural history of Vascular Ehlers-Danlos Syndrome

Vascular Ehlers-Danlos Syndrome (VEDS) is a rare autosomal dominant disorder caused by mutations in the COL3A1 or COL1A1 genes. Its mortality is secondary to sudden and spontaneous rupture of arteries or hollow organs. The genotype influences the distribution of arterial pathology with aneurysms of...

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Bibliographic Details
Main Authors: Christopher Antônio Febres-Aldana, Amilcar Antonio Castellano-Sanchez, John Alexis
Format: Article
Language:English
Published: University of São Paulo 2019-08-01
Series:Autopsy and Case Reports
Subjects:
Aneurysm
False
Ehlers-Danlos Syndrome
Intestinal Perforation
Dissecting
Online Access:http://www.revistas.usp.br/autopsy/article/view/161006

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