The Skeletal Dysplasias

Cedars-Sinai Medical Center, West Hollywood CA

Investigators

Linked publications, trials & patents

Abstract

DESCRIPTION (provided by applicant): The purpose of this revised application is to improve the International Skeletal Dysplasia Registry Database. The enhanced capabilities of the database will significantly accelerate the research facilitated by the International Skeletal Dysplasia Registry Database (Core A), and this will facilitate the research goals of all of the Projects supported by the grant, the goals of Core B, and collaborative efforts with investigators outside of the program project team. In recent years, a number of improvements have been made in the search and reporting functions of the Registry's electronic database, however much more needs to be done to maximize its utility, research potential, and ability to accelerate the recruitment of appropriate study subjects. The key element of the anticipated improvements is conversion to a more easily navigable web-enabled interface. Not only would this make the search and reporting functions of the database more efficient, but it would also enable online submission of clinical and radiographic information for research. Streamlined recruitment would drive expansion of the number and variety of cases in the Registry. The improved Registry database will thus constitute a highly interactive design that will allow all of the projects in this program project to benefit by improved access to the database as well as improved recruitment of research subjects. Further, the investigators will also create a publicly accessible website of teaching modules on the skeletal dysplasias. The teaching modules will provide research investigators, clinicians, and students with online access to information on the clinical, radiographic, and morphologic presentation of typical skeletal dysplasia cases derived from the vast archive of cases the investigators have studied over the last 40 years. This revision will utilize two IT technologists, a User Interface Designer, and an Application and Database Designer. They will perform system analysis and design, case matching and comparison, image processing, creation of a teaching atlas, public web portal, clinical system integration, data import/export and migration, navigation and usability improvements, as well as quality assurance. The development of the new web based registry can serve as a model for other disease based registries, thus accelerating the work done by many other groups of investigators. RELEVANCE: The enhanced Skeletal Dysplasia Registry database will greatly improve the case submission and analysis process, thus enhancing the ability of the International Skeletal Dysplasia Registry Database (Core A) to ascertain and recruit research subjects, compare patients on the basis of a wide variety of clinical, radiologic, morphologic, and biochemical/molecular traits, thereby accelerating the work proposed in all of the projects and cores.