Dentinogenesis imperfecta

Dentinogenesis imperfecta (DI, from Latin dentes, tooth ' and Greek γένεσις, genesis, genesis ' and imperfecta latin, imperfect ' ) is an autosomal dominantly inherited malformation / structural disorder of Zahndentitionen that occurs approximately in 1 in 8000 people and a strong abrasion of the has teeth result.

Variants of the DI

Variants based on Shields, et al. (1973).

Although this classification has been commonly used to structure the course forms the boundary of each type is still very confusing and subject to current research articles. Example, it was found that DI type II and III and dentin dysplasia type II, all caused by the mutation of the same allele. Furthermore, there are numerous other syndromes DI exhibit similar phenotypes.

  • Type I ( DI Shields Type I) is expressed as part of the disease osteogenesis imperfecta ( OI)
  • Type II ( DI Shields type II or hereditary opalescent ) and type III ( Brandywine-Typ/Schalenzähne ) also occurs without genetic predispositions, as indicated by the OI on.
  • Type I has been shown in addition to the dental abnormalities and a progressive hearing loss result.
  • Type III was first detected in a population in Brandywine (United States ), where this type seems to limit.

Symptoms

The degree of intensity of symptoms for each type, even within the same family, is highly variable. The general rule is:

  • Discoloration of the teeth (blue- gray, yellow -brown)
  • Transparency of the enamel
  • Teeth tend to quickly wear / abrasion ( abrasion)
  • Dental crowns extremely dense
  • Tooth root extremely closely and dense
  • Defect of the enamel -dentine connection ( dentin enamel junction, DEJ ); extremely soft dentin
  • Obliteration of the pulp chamber and channels

Causes

The cause of the DI type II and III, the DSPP gene ( dentin sialophosphoprotein ) was identified. The DSPP gene for the production of three proteins mainly responsible and essential for the development of tooth. The mutation alters these proteins, so that the arrangement of the dentine, which is responsible for the average dental protective layer is disturbed. The dentin is atypical because the odontoblasts ( dentin forming cells ) are replaced by other cells. This leads to an excessively high water content in the tooth (60 %), with a simultaneous extremely low mineral content in enamel and dentin.

Treatment options

With a lack of treatment may lead to a complete abrasion of the tooth up to the gum line. The treatment aims to minimize the abrasion possible to rehabilitate the teeth which has extreme wear and tear to optimize aesthetics, and on the prevention of secondary damage. Normally, the back teeth with dental crowns are provided, this helps to reduce tooth wear and to stabilize the upper and lower jaw. On the primary posterior tooth parts stainless steel crowns are placed, and the front teeth are treated with tooth-colored filling material. This is required for all permanent teeth.

Tooth eruption
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