Dentinogenesis imperfecta type 1 (Q86040): Difference between revisions
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Transtorno hereditário de desenvolvimento do dente, transmitido como traço autossômico dominante, e caracterizado por alteração da cor dos dentes, variando de azul moderadamente escuro a amarronzada, dentina mal formada com conteúdo mineral anormalmente baixo, obliteração do canal pulpar e esmalte normal. Os dentes geralmente se desgastam rapidamente, restando tocos curtos e marrons. Também chamado de d. hereditária hipoplásica, síndrome de Capdepont-Hodge, síndrome de Capdepont, síndrome de Fargin-Fayelle, dentes escuros hereditários, dentina opalescente hereditária, dentes opalescentes hereditários, odontogênese imperfeita, síndrome de Stainton-Capdepont e síndrome de Stainton. O tipo 1 está relacionado à cementogênese imperfeita e à osteogênese imperfeita. | |||
| description / en | description / en | ||
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. | |||
Revision as of 11:24, 15 August 2026
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.
| Language | Label | Description | Also known as |
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| default for all languages | ID_1665867595 |
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| English | Dentinogenesis imperfecta type 1 |
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. |
