Dentinogenesis Imperfecta – “A Hereditary Developmental Disturbance of Dentin”

Dentinogenesis Imperfecta (DGI) is a genetic disorder of tooth development inherited in an autosomal, dominant way, characterized by the presence of translucent or opalescent dentin, resulting in discoloration (amber-like, bluish grey color) of the teeth. It is a mesodermal defect wherein the dental papilla of either or both the primary and secondary dentition is abnormal. This paper describes the dental findings in a family where the father (33 year old) and the son (3 year old) both showed the characteristic dental features of DGI I.