Cardiovascular manifestations of Marfan's Syndrome in the young. Am Heart J 1992;3:752-7.Hirata K, Triposkiadis F, Sparks E, et al. The Marfan syndrome: cardiovascular physical findings and diagnostic correlates. Am Heart J 1991;123:743-51....
o259 children (mean age 8 years) with Marfan syndrome (FBN1pathogenic variant and fulfilling 1996 Ghent criteria) were compared to 474 children with suspected Marfan syndrome or relative with Marfan syndrome, but in whom Marfan syndrome was ruled out ochildren with Marfan syndrome significantly tal...
Marfan syndrome is a systemic disorder of connective tissue with a high degree of clinical variability. Cardinal manifestations involve the ocular, skeletal, and cardiovascular systems. FBN1 mutations associate with a broad phenotypic continuum, ranging from isolated features of Marfan syndrome to neonata...
Because the molecular defect in Marfan syndrome involves microfibrils in elastin, the critical manifestations of the disease involve cardiac valves and blood vessels, which are discussed later. Neonatal Phenotype Recognition of the Marfan phenotype in the newborn signifies a particularly severe, usually...
Cardiac manifestations of Marfan syndrome include aortic root dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP in patients with Marfan syndrome undergoing aortic root replacement.We retrospectively analyzed data from 166 MFS patients with MVP who were enrolled in a prospect...
Cardiac imaging With the ever growing appreciation of the complex manifestations of MFS, it has become apparent that patients need assessment of the entire aorta and careful study of valves and biventricular function [81]. As the number and quality of available imaging modalities has increased, it...
This refined understanding of the pathogenesis of vascular disease in Marfan syndrome will facilitate the development of therapeutic strategies. CAS-1 JCR-Q1 SCIE 286 被引用 · 0 笔记 引用 Early Induction of Transforming Growth Factor-β via Angiotensin II Type 1 Receptors Contributes to Cardiac ...
Primary impairment of left ventricu- lar function in Marfan syndrome. Int J Cardiol 2006;112:353–8. 21. Luchner A, Muders F, Dietl O, et al. Differential expression of cardiac ANP and BNP in a rabbit model of progressive left ventricular dysfunction. Cardiovasc Res 2001;51:601–...
The abnormality in the small coronary arteries of patients with Marfan's syndrome as described by James et al2 must make one highly suspicious of Marfan's syndrome in a patient with arachnodactaly and a cardiac conduction defect. Sinclair has mentioned cataracts in Marfan's syndrome, but is ...
We also assume that these molecular alterations, if present in a patient, can also influence the pattern of the extracardiac manifestations of the syndrome. Molecular and extracardiac phenotypic characteristics of the hypothetical malignant group