Diseases & Conditions


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Dentinogenesis Imperfecta Type III


Synonyms of Dentinogenesis Imperfecta Type III
  • Brandywine Type Dentinogenesis Imperfecta
  • Dentinogenesis Imperfecta, Shields Type

Disorder Subdivisions



    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.

    Organizations related to Dentinogenesis Imperfecta Type III
    • MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
      150 Custer Court
      Green Bay WI 54301-1243
      Phone #: 920-336-5333
      800 #: 877-336-5333
      e-mail: mums@netnet.net
      Home page: http://www.netnet.net/mums/
    • NIH/National Oral Health Information Clearinghouse
      1 NOHIC Way
      Bethesda MD 20892-3500
      Phone #: 301-402-7364
      800 #: --
      e-mail: nohic@nidcr.nih.gov
      Home page: http://www.nohic.nidcr.nih.gov



    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

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