DENTINOGENESIS IMPERFECTA: A CASE REPORT WITH FAMILY HISTORY

Dr. Juhi Gupta*, Dr. Anshul Aggarwal, Dr. Kauser Jahan Khwaja,

ABSTRACT

Dentinogenesis imperfecta (DI) or hereditary opalescent dentin was first described in the late 19th century. It is a localized mesodermal dysplasia affecting both the primary and permanent dentition. The disease is inherited in a simple autosomal dominant mode with high penetrance and a low mutation rate. The reported incidence in the USA is 1:8000 births. Shields et al proposed three types of dentinogenesis imperfecta: DI type 1 is associated with osteogenesis imperfecta. DI type 2 has essentially the same clinical radiographic and histological features as DI type 1 but without osteogenesis imperfecta; DI type 3 is rare and is only found in the tri racial Brandywine population of Maryland. It has been suggested that DI type 2 and DI type 3 are different expressions of the same gene.

Keywords: Opalescent dentin, abnormal dentino-enamel junction, dentin hypoplasia, amber coloured teeth, Bulbous crown.