Dentinogenesis imperfecta type 1 (Q86040)

A hereditary disorder of tooth development, transmitted as an autosomal dominant trait, and characterised by discoloration of the teeth, ranging from dusky blue to brownish, poorly formed dentin with an abnormally low mineral content, obliteration of the pulp canal, and normal enamel. The teeth usually wear down rapidly, leaving short, brown stumps. Called also d. hypoplastic hereditaria, Capdepont-Hodge syndrome, Capdepont’s syndrome, Fargin-Fayelle syndrome, hereditary dark teeth, hereditary opalescent dentin, hereditary opalescent teeth, odontogenesis imperfect, Stainton-Capdepont syndrome, and Stainton syndrome. Type 1 is related to cementogenesis imperfecta and osteogenesia imperfect.