Dentinogenesis imperfecta type III
is one of five distinct, hereditary disorders of dentin (the bone-like substance that makes up most of the tooth) development affecting the teeth. This condition causes the teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent. Teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (baby) teeth and permanent teeth. Dentinogenesis imperfecta type III is caused by mutations in the DSPP gene which are inherited in an autosomal dominant fashion. Treatment, which may begin as early as infancy, usually continues into adulthood with a number of options including the use of crowns, over-dentures and dental implants depending on the age of the patient and the condition of the dentition. Individuals with dentinogenesis imperfecta type III appear to be limited, in large measure, to a population in the region around Brandywine in southern Maryland. Source: Genetic and Rare Diseases Information Center (GARD), supported by ORDR-NCATS and NHGRI.