Dentinogenesis imperfecta
Dentinogenesis Imperfecta
Dentinogenesis imperfecta (pronunciation: den-ti-no-gen-e-sis im-per-fek-ta) is a rare genetic disorder that primarily affects the formation of tooth enamel and dentin, the hard tissue beneath the enamel that forms the bulk of the tooth.
Etymology
The term "Dentinogenesis imperfecta" is derived from the Latin words "dentinum" meaning dentin, "genesis" meaning formation, and "imperfecta" meaning imperfect. Thus, it refers to the imperfect formation of dentin.
Definition
Dentinogenesis imperfecta is characterized by discolored (most often blue-gray or yellow-brown), translucent teeth. This translucency is due to the abnormal formation of the dentin and the increased transparency of the enamel. The teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss.
Types
There are three types of dentinogenesis imperfecta:
- Dentinogenesis Imperfecta Type I: Also known as DI type I, it is associated with Osteogenesis Imperfecta, a condition that causes brittle bones.
- Dentinogenesis Imperfecta Type II: Also known as DI type II, it is not associated with any other abnormalities.
- Dentinogenesis Imperfecta Type III: Also known as DI type III or Brandywine type, it is characterized by multiple abnormalities in the teeth.
Causes
Dentinogenesis imperfecta is caused by mutations in the DSPP gene, which provides instructions for making proteins essential for normal tooth development. These mutations disrupt the normal development of teeth, leading to the characteristic features of dentinogenesis imperfecta.
Treatment
Treatment for dentinogenesis imperfecta primarily involves protecting the teeth, managing symptoms, and improving the appearance of the teeth. This may include the use of crowns, bonding, and other dental procedures.
Related Terms
External links
- Medical encyclopedia article on Dentinogenesis imperfecta
- Wikipedia's article - Dentinogenesis imperfecta
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