Get Aortic Dissection and Related Syndromes (Developments in PDF

By Ragavendra R. Baliga (Editor), Christoph A. Nienaber (Editor), Eric M. Isselbacher (Editor), Kim A.

ISBN-10: 038736000X

ISBN-13: 9780387360003

ISBN-10: 0387360018

ISBN-13: 9780387360010

This state-of the-art e-book is end result of the the mixed efforts of contributors from the foreign Registry of Aortic Dissection (IRAD). it's the such a lot entire reference on aortic dissection The e-book has been divided into sections. every one bankruptcy presents a succinct assessment of the present scientific literature and contains illustrations for additional rationalization.

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Circ. Res. 88, 37–43. 31. Segura AM, Luna RE, Horiba K, et al. (1998). Immunohistochemistry of matrix metalloproteinases and their inhibitors in thoracic aortic aneurysms and aortic valves of patients with Marfan’s syndrome. Circulation 98, II331–II337; discussion II337–II338. 32. Sakomura Y, Nagashima H, Aoka Y, et al. (2002). Expression of peroxisome proliferatoractivated receptor-γ in vascular smooth muscle cells is upregulated in cystic medial degeneration of annuloaortic ectasia in Marfan syndrome.

26. Aoyama T, Francke U, Dietz H, Furthmyer H (1994). Quantitative differences in biosynthesis and extracellular deposition of fibrillin in cultured fibroblasts distinguish five groups of Marfan syndrome patients and suggest distinct pathogenetic mechanisms. J. Clin. Invest. 94, 130–137. 27. De Paepe A, Devereux R, Dietz H, Hennekam R, Pyeritz R (1996). Revised diagnostic criteria for the Marfan syndrome. Am. J. Med. Genet. 62, 417–426. 28. Boileau D, Jondeau G, Babron MC, et al. (1993). Autosomal dominant Marfan-like connective-tissue disorder with aortic dilatation and skeletal anomalies not linked to the fibrillin genes.

The diagnosis of the Marfan syndrome is currently based on revised clinical criteria of the Gent nosology9 . The Gent criteria pay particular attention to genetic information like Marfan’s syndrome in kindreds of an unequivocally affected individual. Moreover, both skeletal and cardiovascular features are major (diagnostic) criteria, and Marfan’s is said to be present when four of eight typical manifestations are present. Considering, however, borderline manifestations (such as the MASS phenotype) or subtle phenotypic features (forme fruste), the molecular analysis of suspected Marfan’s syndrome and the delineation of criteria for differentiating other inherited conditions (genotypes) from a Marfan phenotype are of keen interest22–28 .

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Aortic Dissection and Related Syndromes (Developments in Cardiovascular Medicine) by Ragavendra R. Baliga (Editor), Christoph A. Nienaber (Editor), Eric M. Isselbacher (Editor), Kim A.


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