Preferred Label : Dentinogenesis imperfecta type 1;
ICD-11 definition : A hereditary disorder of tooth development, transmitted as an autosomal dominant trait,
and characterized 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.;
Origin ID : 1665867595;
Automatic exact mappings (from CISMeF team)
A hereditary disorder of tooth development, transmitted as an autosomal dominant trait,
and characterized 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.
