OSTEOGENESIS IMPERFECTA

Course overview

OSTEOGENESIS IMPERFECTA (OI) IS A GENETIC DISORDER OF CONNECTIVE TISSUES CAUSED BY AN ABNORMALITY IN THE SYNTHESIS OR PROCESSING OF TYPE I COLLAGEN. IT IS ALSO CALLED BRITTLE BONE DISEASE. IT IS CHARACTERIZED BY AN INCREASED SUSCEPTIBILITY TO BONE FRACTURES AND DECREASED BONE DENSITY. OTHER MANIFESTATIONS INCLUDE BLUE SCLERAE, DENTINOGENESIS IMPERFECTA, SHORT STATURE, AS WELL AS DEAFNESS IN ADULTHOOD. THIS ACTIVITY REVIEWS THE PATHOPHYSIOLOGY OF OSTEOGENESIS IMPERFECTA AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ITS MANAGEMENT.