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

• Main Authors: Poornima Surendra, Rohan Shah, Roshan N.M., V.V. Subba Reddy
• Format: Article
• Language: English
• Published: JCDR Research and Publications Private Limited 2013-08-01
• Series: Journal of Clinical and Diagnostic Research
• Subjects: dentinogenesis imperfecta; osteogenesis imperfecta; opalescent dentin; deciduous dentition; autosomal dominant
• Online Access: https://jcdr.net/articles/PDF/3286/91-%205723_PF1(M)_E(C)_F(T)_F1(V)_F1(T)_PF1(VRP)_PFA(PR)_PF3_(PUH)_B.pdf

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.