| Literature DB >> 12908076 |
Maria de Fátima M P Leite1, Nadia Barreto Tenorio Aoun, Monica Scott Borges, Maria Eliane Campos Magalhães, Luiz A Christiani.
Abstract
Marfan's syndrome is an inherited disorder of the connective tissue. Cardiologic manifestations, especially aortic dilation, are important causes of morbidity and mortality in the clinical course of the disease in adults and teenagers. In children, the presence of aortic aneurysm and its dissection or rupture is rare, occurring in patients with genetic mutation of the fibrillin gene but not in those who have the familial form of the disease. We describe here 2 patients, from the same family (siblings), diagnosed with gigantic aortic aneurysm early in infancy, one of them successfully undergoing surgery.Entities:
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Year: 2003 PMID: 12908076 DOI: 10.1590/s0066-782x2003000900008
Source DB: PubMed Journal: Arq Bras Cardiol ISSN: 0066-782X Impact factor: 2.000