International Meeting on Pseudoxanthoma Elasticum

Uitto, Jouni

Abstract

(from the application) This grant application requests partial support for a Symposium, entitled """"""""International Centennial Meeting on Pseudoxanthoma Elasticum"""""""". The meeting will be held on November 6 and 7, 1997, at Bethesda Marriott, Bethesda, MD. The meeting is sponsored by PXE International, a patient advocacy organization dedicated to advancement of understanding of the biology, pathology, genetics and treatment of PXE. This symposium is organized on the premise that significant progress has been made in basic understanding of the structural components affected in PXE, but the critical link between the basic science and the pathogenesis of this group of diseases is missing. The purpose of this meeting is to review the progress in this area of cellular and molecular biology, and identify potential mechanisms which would lead to the PXE phenotype, in the context of clinical, morphologic, ultrastructural and genetic observations. A concluding panel discussion will formulate recommendations for targeted areas critical to basic research, as well as clinical areas of emphasis. The organizing committee consists, in addition to the President of PXE International (Ms. Sharon Terry), of four eminent scientists active in PXE related research. Three of these, Drs. Charles Boyd, Joel Rosenbloom and Jouni Uitto are funded in this area of research, and the fourth, Dr. Mark Lebwohl, has published extensively on PXE. The invited speakers represent a cross section of individuals touched by PXE, varying from lay representatives of PXE International, physician-scientists working at the basic science-clinical interface of PXE research, and basic scientists with expertise in the extracellular matrix and molecular biology of PXE. It is expected that this cadre of individuals, with complementary expertise and working in the interdisciplinary fields relating to PXE, will allow a fruitful exchange of ideas towards better understanding of this disease. The last similar meeting, organized in the Jefferson Medical College in 1992, was highly successful but an abundance of new information related to PXE has resulted from research between 1992 and today. The previous meeting was summarized in a report format in The Journal of Investigative Dermatology, and these recommendations have served as a blueprint of active development of basic research and clinical applications in relation to PXE. It is expected that the Symposium to be held in November of 1997 will be equally successful in identifying future critical research and clinical areas of PXE.