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Diseases

Genetic and Rare Diseases Information Center (GARD)

Dentinogenesis imperfecta type 3


Other Names for this Disease
  • Dentinogenesis imperfecta type III
  • Brandywine type dentinogenesis imperfecta
  • Dentinogenesis imperfecta Shields type 3
  • Dentinogenesis imperfecta, Shields type 3
Related Diseases
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Overview

Dentinogenesis imperfecta type 3 is a rare and severe form of dentinogenesis imperfecta, a condition that affects tooth development. People affected by this condition generally have discolored (most often a blue-gray or yellow-brown color) and translucent teeth. 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. Dentinogenesis imperfecta type 3 is caused by changes (mutations) in the DSPP gene which are inherited in an autosomal dominant manner.[1] Treatment is usually focused on protecting primary (baby) and then permanent teeth with preformed pediatric crowns and other interventions. The replacement of teeth might be considered in the future with dentures and/or implants.[2]
Last updated: 10/30/2015

References

  1. Dentinogenesis imperfecta type 3. Orphanet. September 2012; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=166265.
  2. Dentinogenesis Imperfecta Type III. National Organization for Rare Disorders (NORD). 2006; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Dentinogenesis%20Imperfecta%20Type%20III. Accessed 2/19/2009.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Dentinogenesis imperfecta type 3. This website is maintained by the National Library of Medicine.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • 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 type 3. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Dentinogenesis imperfecta type III
  • Brandywine type dentinogenesis imperfecta
  • Dentinogenesis imperfecta Shields type 3
  • Dentinogenesis imperfecta, Shields type 3
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.