Dentinogenesis Imperfecta
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What is dentinogenesis imperfecta?
Dentinogenesis imperfecta is a 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.
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. Dentinogenesis imperfecta type II and type III usually occur in people without other inherited disorders. 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.
How common is dentinogenesis imperfecta?
Dentinogenesis imperfecta affects an estimated 1 in 6,000 to 8,000 people.
What genes are related to dentinogenesis imperfecta?
Mutations in the DSPP gene cause dentinogenesis imperfecta.
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.
How do people inherit dentinogenesis imperfecta?
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.
In most cases, an affected person has one parent with the condition.
Source: National Institutes of Health
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Study results from M. Groten and colleagues update understanding of quintessence
2009 JUN 23 - (NewsRx.com) -- According to a study from Tubingen, Germany, "A young patient with dentinogenesis imperfecta and a dentition severely compromised by discolorations, abrasion, and breakage of enamel on several teeth was prosthodontically treated with CAD/CAM-manufactured all-ceramic restorations. To reduce treatment risk, preparations were limited paragingivally." "Maxillary anterior teeth and premolars were restored with adhesively luted ceramic crowns. All other teeth were treated with zirconia-based restorations luted with a self-etching resin-based material. Considering the deficient pretreatment situation, the use of tooth-colored restoration and luting materials yielded a satisfying functional and good esthetic result," wrote M. Groten and colleagues. The researchers concluded: "The patient is highly satisfied, even though 3 minor chippings occurred shortly after the completion of treatment. (Quintessence Int 2009; 40:19-27)'." Groten and colleagues published the results of their research in Quintessence International (Complex all-ceramic rehabilitation of a young patient with a severely compromised dentition: A case report. Quintessence International, 2009;40(1):19-27). For additional information, contact M. Groten, School Dental, Dept. of Prosthodont, Osianderstr 2-8, D-72076 Tubingen, Germany. The publisher of the journal Quintessence International can be contacted at: Quintessence Publishing Co. Inc., 4350 Chandler Drive, Hanover Park, IL 60133, USA. Keywords: Germany, Tubingen, Life Sciences, Prosthodontics, Rehabilitation Research, Quintessence. This article was prepared by Science Letter editors from staff and other reports. Copyright 2009, Science Letter via NewsRx.com.
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