Dentinogenesis Imperfecta : A Family which was Affected for Over Three Generations

Abstract

Dentinogenesis Imperfecta (DI) or hereditary opalescent dentin is inherited in a simple autosomal dominant mode with high penetrance and low mutation rates. It generally affects both the deciduous and the permanent dentitions. DI corresponds to a localized form of mesodermal dysplasia which is observed in the histo-differentiation. An early diagnosis and treatment are therefore fundamental, which aim at obtaining a favourable prognosis, since at late intervention makes the treatment more complex. We are presenting here a case of DI in which the disease affected the three generations of a family in India.

Keywords

• dentinogenesis imperfecta
• osteogenesis imperfecta
• opalescent dentin
• deciduous dentition
• autosomal dominant