Dentinogenesis Imperfecta Type III
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Dentinogenesis Imperfecta Type III is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Dentinogenesis imperfecta type III (DGI-III) is one of five distinct, hereditary disorders of dentin development affecting the teeth. Dentin is the hard, bone-like material that makes up most of a tooth and lies under the enamel serving to protect the soft, pulp tissue. These heritable dentin disorders may affect only the teeth or may be associated with the condition known as osteogenesis imperfecta. Whether this association is present is a major criterion in the classification of dentinogenesis imperfecta into three types.
The teeth of people who have inherited one of the DGIs are usually pale-colored and lustrous (opalescent). They are awkwardly formed and situated in the gums; they wear away readily and break easily.
Patients with DGI type I also are affected by osteogenesis imperfecta, and the whites of their eyes (sclera) are blue in color. Patients with DGI type II are NOT affected by osteogenesis imperfecta, but show the other clinical signs. Patients with DGI type III appear to be limited, in large measure, to a population in the region around Brandywine in southern Maryland.
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