hereditary opalescent dentin


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Related to hereditary opalescent dentin: dentinogenesis imperfecta, dentin dysplasia, shell teeth

den·tin·o·gen·e·sis im·per·fec·ta

[MIM*125490 & MIM*125500]
an autosomal dominant disorder of the teeth characterized clinically by translucent gray to yellow-brown teeth involving both primary and permanent dentition; the enamel fractures easily, leaving exposed dentin, which undergoes rapid attrition; radiographically, the pulp chambers and canals appear obliterated and the roots are short and blunted; sometimes occurs in association with osteogenesis imperfecta; autosomal dominant inheritance.

hereditary opalescent dentin

An autosomal dominant [MIM 125490] dysodontogenesis which is one of the most common defects in dentin production, affecting 1:8,000 persons, characterised by small, yellow-brown teeth, fracturing of enamel and excessive wearing of teeth
Treatment Multidisciplinary approach focusing on occlusal rehabilitation, and restorative treatment to preserve where possible remaining tooth structure and protect the affected dentin from caries, attrition, abrasion and erosion

dentinogenesis imperfecta

A common (1 in 6–8,000) inherited disorder of tooth development (OMIM:125490), which is characterised by blue-gray or yellow-brown translucencies of the teeth.

Molecular pathology
Dentinogenesis imperfecta is caused by mutations of DSPP, the gene that encodes two key proteins of the dentin extracellular matrix: dentin sialoprotein and dentin phosphoprotein.

o·pal·es·cent den·tin

(ŏ'pă-les'ĕnt den'tin)
Dentin usually associated with dentinogenesis imperfecta that gives an unusual opalescent or translucent appearance to the teeth.
Synonym(s): hereditary opalescent dentin (2) .

den·tin·o·gen·e·sis im·per·fec·ta

(den'ti-nō-jen'ĕ-sis im-pĕr-fek'tă) [MIM*125490 & 125500, MIM*125490, MIM*125500, MIM*125490 &]
Dental disorder characterized clinically by translucent gray to yellow-brown teeth involving both primary and permanent dentition; such enamel fractures easily, leaving exposed dentin, which undergoes rapid attrition.
Synonym(s): hereditary opalescent dentin (1) .

hereditary opalescent dentin (hə-red´iter´ē ō´pəles´ənt den´tin),

n a developmental disturbance in the formation of dentin, better known as dentiogenesis imperfecta. The teeth range from gray to brownish violet and are translucent or opalescent. The crowns fracture easily because of an abnormal dentinoenamel junction.
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