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Genetic and Rare Diseases Information Center (GARD)

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Dentinogenesis imperfecta Shields type 3


Other Names for this Disease

  • Brandywine type dentinogenesis imperfecta
  • Dentinogenesis imperfecta type III
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Overview

Dentinogenesis imperfecta type III is one of five distinct, hereditary disorders of dentin (the bone-like substance that makes up most of the tooth) development affecting the teeth.[1] This condition causes the 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. Dentinogenesis imperfecta type III is caused by mutations in the DSPP gene which are inherited in an autosomal dominant fashion.[2] Treatment, which may begin as early as infancy, usually continues into adulthood with a number of options including the use of crowns, over-dentures and dental implants depending on the age of the patient and the condition of the dentition.[3] Individuals with dentinogenesis imperfecta type III appear to be limited, in large measure, to a population in the region around Brandywine in southern Maryland.[1]
Last updated: 2/19/2009

References

  1. 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.
  2. Dentinogenesis imperfecta. Genetics Home Reference (GHR). November 2009; http://ghr.nlm.nih.gov/condition=dentinogenesisimperfecta. Accessed 4/29/2011.
  3. Barron MJ, McDonnell ST, MacKie I, Dixon MJ. Dentinogenesis imperfecta. Orphanet. November 2008; http://www.orpha.net//consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=49042. Accessed 2/19/2009.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Dentinogenesis imperfecta Shields type 3. This website is maintained by the National Library of Medicine.
  • The National Institute of Dental and Craniofacial Research (NIDCR), purposes to improve oral, dental and craniofacial health through research, research training, and the dissemination of health information. Click on the link to view information on this topic. 
  • 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

  • The 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 Shields type 3. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Brandywine type dentinogenesis imperfecta
  • Dentinogenesis imperfecta type III
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.