'''Dentinogênese imperfeita''' (Dentina opalescente hereditária) é uma doença genética do desenvolvimento dentário. Esta condição provoca descoloração de dentes (a maior parte das vezes em cores azul-cinza ou marrom-amarelo) e translúcido. Os dentes são também mais fraco do que o normal, tornando-as mais expostas ao desgaste rápido, quebra, e de perda. Estes problemas podem afetar dentes primários quanto dentes permanentes. É hereditária autossômica dominante em um padrão, o que significa uma cópia do gene modificado em cada célula é suficiente para causar a doença. Dentinogênese imperfeita afeta um número estimado de 1 entre 6000 e 8000 pessoas.

==Variedades==
Tem-se descrito três tipos de dentinogêneses imperfeitas semelhantes.

;Tipo I: Ocorre em pessoas que têm osteogênese imperfeita, uma condição genética na qual ossos frágeis quebram com facilidade. Isso normalmente é traço autossômico dominante com expressividade variável, mas podem ser recessivos se associado a osteogênese imperfeita do tipo recessiva.

;Tipo II: Sem uma hereditária, ou seja, Osteogênese imperfecta. É um traço autossômico dominante e é, de fato, a mais comum das doenças autossômicas dominantes nos seres humanos. Algumas famílias com tipo II têm progressiva perda auditiva, além de anormalidades dentárias.

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'''Dentinogenesis imperfecta''' (hereditary Opalescent Dentin)  is a genetic disorder of tooth development. This condition causes teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent. Teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (baby) teeth and permanent teeth. This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. Dentinogenesis imperfecta affects an estimated 1 in 6,000 to 8,000 people.

Researchers have described three types of dentinogenesis imperfecta with similar dental abnormalities. 

Type I: Occurs in people who have osteogenesis imperfecta, a genetic condition in which bones are brittle and easily broken.It is usually an autosomal dominant trait with variable expressivity but can be recessive if the associated osteogenesis imperfecta is of recessive type.

Type II : Occurs in people without other inherited disorders ie Osteogenesis imperfecta.It is an autosomal dominant trait and is in fact on of the most common autosomal dominant disorders in human beings.  A few families with type II have progressive hearing loss in addition to dental abnormalities. 

Type III dentinogenesis imperfecta was first identified in a population from Brandywine, Maryland. Researchers now believe that type II and type III may be the same disorder.

Mutations in the ''DSPP'' gene have been identified in people with type II and type III dentinogenesis imperfecta. Type I occurs as part of osteogenesis imperfecta, which is caused by mutations in one of several other genes. The DSPP gene provides instructions for making three proteins that are essential for normal tooth development. These proteins are involved in the formation of dentin, which is a bone-like substance that makes up the protective middle layer of each tooth. Mutations in the ''DSPP'' gene may affect the proteins made by the gene, leading to the production of abnormally soft dentin. Teeth with defective dentin are discolored, weak, and more likely to decay and break. It remains unclear how DSPP mutations lead to hearing loss in some families with dentinogenesis type II.

'''Clinical Features'''

Clinical appearance is variable.However, the teeth usually involved and more severely affected are deciduous teeth in type 1; whereas in type 2 both the dentitions are equally affected.