The frequency of other anomalies was as follows: molar-incisor hypomineralization 0.25%, turner hypoplasia 0.1%, fluorosis 0.1%, odontoma 0.1%, fusion 0.09%, gemination 0.06%, amelogenesis imperfecta 0.05%, dens invaginatus 0.03%, talon cusp 0.02%, taurodontism 0.02%, macrodontia 0.02%, dentinogenesis imperfecta
0.02%, dilaceration 0.02%, ectopic eruption 0.01% and microdontia 0.01%.
However, many clinicians use a commonly accepted phenotypic classification (Table 4) that includes this dentin-affecting disorder as a type of dentinogenesis imperfecta
. (5,19) In a genotypic sense, dentinogenesis imperfecta
is most associated with various mutations of the DSPP gene.
However, the genes regulating enamel and dentine formation are highly specific for teeth, and their mutations are associated with dental disorders such as amelogenesis imperfecta, dentinogenesis imperfecta
, dentine dysplasia and isolated anomalies of tooth number and size [Bailleul-Forestier et al., 2008].
They describe a 19-year-old Soldier's presentation with a condition known as dentinogenesis imperfecta
, a genetic disorder that affects the majority of the teeth, which, without treatment, ultimately results in serious deterioration and tooth loss.
It is a heritable and systemic disease characterized by bone fragility leading to recurrent bone fractures, as well as by dentinogenesis imperfecta
, blue sclerae, hearing loss, and short stature.
Three patients (2 with obvious dentinogenesis imperfecta
) had normal-colored sclerae (type IV).
Synonyms such as hereditary opalescent dentin have been used to describe opalescent brown teeth as a heritable, isolated defect.
Pulp stones also accompany a number of diseases, among others: end-stage renal failure, dental dysplasia, Ehlers-Danlos syndrome, Ellis-van Creveld syndrome, dentinogenesis imperfecta
, van der Woude syndrome or Marfan syndrome.
(DGI) is defined as an autosomal dominant disorder in dentin mineralization in both the primary and permanent dentitions.