hereditary opalescent dentin


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. Synonym(s): hereditary opalescent dentin (1)
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) .