The Sixth International Marfan Syndrome Symposium will be held in Seattle, WA from August 11 -14, 2001. This meeting will bring together investigators who represent the most advanced research directed toward determining the underlying basis of Marfan Syndrome, the fundamental biology of the fibrillin genes and their interacting partners, the identification of other matrix components that are important for the construction of the elastic fiber network, the mechanisms by which the mutations are translated into the phenotypic findings, the issues of locus heterogeneity and the relationship to other disorders that result from mutations in related genes, the natural history of the disorder, the change in natural history by medical or surgical intervention, and the manner in which analysis of these genes assist in clinical decision making. The theme of the Symposium will be """"""""Current Controversies in Research in Pathogenesis and Treatment of Marfan Syndrome and Related Disorders"""""""". These issues will be examined in five scientific sessions of approximately 3 to 3.5 hours each. The first session will be devoted to the relationship of genotype to phenotype, the role of molecular diagnostics, and the effect of mutations on the fibrillin proteins. The second will examine the function of fibrillitts, the cell biology of the proteins, their processing and fibrillogenesis, and interaction with other components of the matrix, and how defects in these processes lead to Marfan and other phenotypes. The third will examine the organization of tissues, the tole of fibrillins in non-Marfan phenotypes, and of other disorders that may mimic some of the Marfan phenotypes. The fourth will be devoted to issues in medical therapy, including the use of P-blockers, calcium channel blockers, and inhibitors of matrix degradation to slow the rate of aortic enlargement, the major risk factor for premature death. The fifth will examine issues surrounding cardiac surgery and surgery for lens dislocation. These issues include the use of """"""""valve-sparing"""""""" surgery in aortic foot replacement, the timing of aortic surgery, and the role of lens removal for treatment of visual disturbance. This will be the first major gathering of investigators in three years and will be held in conjunction with the National Marfan Foundation annual meeting. The expected 125 participants should galvanize research efforts for the next years.
