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Diseases

Genetic and Rare Diseases Information Center (GARD)

Dentinogenesis imperfecta


Other Names for this Disease
  • Dentinogenesis imperfecta without osteogenesis imperfecta
  • Capdepont teeth
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Overview

Dentinogenesis imperfecta is a condition that results in issues with tooth development, causing the teeth to be translucent and discolored (most often a blue-gray or yellow-brown in color). Individuals with this disorder tend to have teeth that are weaker than normal which leads to increased wear, breakage, and loss of the teeth. This can affect both primary (baby) and permanent teeth. Dentinogenesis imperfecta is caused by mutations in the DSPP gene and is inherited in an autosomal dominant manner.[1]

There are three types of dentinogenesis imperfecta.
Type I: occurs in people who have osteogenesis imperfecta, a genetic condition in which bones are brittle, causing them to break easily.
Type II and type III: usually occur in people without another inherited disorder. Some families with type II also have progressive hearing loss. Type III was first identified in a population in Brandywine, Maryland. Some researchers believe that dentinogenesis imperfecta type II and type III, along with a similar condition called dentin dysplasia type II, are actually just different forms of a single disorder.[1]

Last updated: 12/3/2015

References

  1. Dentinogenesis imperfecta. Genetics Home Reference (GHR). November 2009; http://ghr.nlm.nih.gov/condition=dentinogenesisimperfecta. Accessed 9/29/2015.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Dentinogenesis imperfecta . This website is maintained by the National Library of Medicine.

In Depth Information

  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Dentinogenesis imperfecta . Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Dentinogenesis imperfecta without osteogenesis imperfecta
  • Capdepont teeth
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.