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Type: Artigo de periódico
Title: Ehlers-danlos Syndrome Type Iv And Multiple Aortic Aneurysms--a Case Report.
Author: de Paiva Magalhães, E
Fernandes, S R
Zanardi, V A
Furtado Medeiros, C A
Midori, R Y
Sachetto, Z
Samara, A M
Abstract: Beside atherosclerosis, aortic aneurysms can be part of the clinical spectrum of many systemic diseases, including infectious, inflammatory, genetic and, less often, congenital disorders. A 48-year-old white man presented with multiple large aneurysms of the aorta and its main branches. Medical history was unremarkable except for the presence of a softened abdominal mass since he was 28 years old. On the physical examination, an arterial murmur was heard over the left carotid artery and a palpable mass was noted in the whole right side of the abdomen. No skin or joint abnormalities were noted. Aortography, computed tomography, and magnetic resonance angiography showed multiple large aneurysms of the descending thoracic and abdominal aorta. Aneurysms of the innominate, left subclavian, and carotid arteries were also seen. This case resembles those previously reported, in which multiple aortic aneurysms were associated with abnormalities of the type III procollagen gene (COL3A1). Although the classic stigmas of the Ehlers-Danlos syndrome type IV were lacking, this genetic disease may be the cause of the multiple aneurysms in this patient.
Subject: Aneurysm
Aortic Aneurysm, Abdominal
Aortic Aneurysm, Thoracic
Brachiocephalic Trunk
Carotid Artery Diseases
Diagnosis, Differential
Ehlers-danlos Syndrome
Iliac Aneurysm
Magnetic Resonance Angiography
Middle Aged
Subclavian Artery
Tomography, X-ray Computed
Rights: fechado
Identifier DOI: 
Date Issue: 2001
Appears in Collections:Unicamp - Artigos e Outros Documentos

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