Dentinogenesis Imperfecta Type III

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Dentinogenesis Imperfecta Type III Important
It is possible that the main title of the report Dentinogenesis Imperfecta Type III is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Brandywine Type Dentinogenesis Imperfecta
  • Dentinogenesis Imperfecta, Shields Type

Disorder Subdivisions

  • None

General Discussion


Dentinogenesis imperfecta type III (DGI-III) is one of five distinct, hereditary disorders of dentin development affecting the teeth. Dentin is the hard, bone-like material that makes up most of a tooth and lies under the enamel serving to protect the soft, pulp tissue. These heritable dentin disorders may affect only the teeth or may be associated with the condition known as osteogenesis imperfecta. Whether this association is present is a major criterion in the classification of dentinogenesis imperfecta into three types.

The teeth of people who have inherited one of the DGIs are usually pale-colored and lustrous (opalescent). They are awkwardly formed and situated in the gums; they wear away readily and break easily.

Patients with DGI type I also are affected by osteogenesis imperfecta, and the whites of their eyes (sclera) are blue in color. Patients with DGI type II are NOT affected by osteogenesis imperfecta, but show the other clinical signs. Patients with DGI type III appear to be limited, in large measure, to a population in the region around Brandywine in southern Maryland.

Resources


Osteogenesis Imperfecta Foundation
804 W. Diamond Ave, Suite 210
Gaithersburg, MD 20878
USA
Tel: (301)947-0083
Fax: (301)947-0456
Tel: (800)981-2663
Email: bonelink@oif.org
Internet: http://www.oif.org

NIH/National Institute of Dental and Craniofacial Research
Building 31, Room 2C39
31 Center Drive, MSC 2290
Bethesda, MD 20892
USA
Tel: (301)496-4261
Fax: (301)480-4098
Tel: (866)232-4528
Email: nidcrinfo@mail.nih.gov
Internet: http://www.nidcr.nih.gov/

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Internet: http://rarediseases.info.nih.gov/GARD/

Source...
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