Marfan's syndrome: early and severe form in siblings
AUTOR(ES)
Leite, Maria de Fátima M. P., Aoun, Nadia Barreto Tenorio, Borges, Monica Scott, Magalhães, Maria Eliane Campos, Christiani, Luiz A.
FONTE
Arquivos Brasileiros de Cardiologia
DATA DE PUBLICAÇÃO
2003-07
RESUMO
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.
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