Literature DB >> 12908076

Marfan's syndrome: early and severe form in siblings.

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.

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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


  1 in total

1.  Cardiovascular Manifestation of a Family with Marfan's Syndrome.

Authors:  Mohammad Hossein Davari; Toba Kazemi; Hossein Alimirzaei; Mohamad Reza Rezvani
Journal:  J Tehran Heart Cent       Date:  2011-02-28
  1 in total

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