Oral Health Center

Dentin Dysplasia, Coronal

Important
It is possible that the main title of the report Dentin Dysplasia, Coronal 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

Anomalous Dysplasia of Dentin
Coronal Dentin Dysplasia
Dentin Dysplasia, Type II
Pulp Stones
Pulpal Dysplasia

Disorder Subdivisions

None

General Discussion

Coronal dentin dysplasia is a rare inherited dental defect that is also known as dentin dysplasia, type II. It is characterized by abnormal development (dysplasia) of the hard tissue (i.e., dentin) that is beneath the enamel, surrounds the pulp, and forms the major part of the teeth. In those with coronal dentin dysplasia, the baby teeth (primary or deciduous teeth) are brownish blue with a translucent "opalescence." (Opalescence refers to a milky, opal-like display of colors in reflected light [iridescence].) However, the permanent teeth appear normal in color.

As seen on dental x-ray imaging, the pulp chambers of the primary teeth--or the natural cavities that contain living pulp in the exposed portion of the teeth (crowns)--are obliterated by abnormal dentin. In addition, the pulp-containing canals within the roots of the teeth (root canals) are smaller than normal. The permanent teeth also have distinctive abnormalities of the pulp chambers. Coronal dentin dysplasia is transmitted as an autosomal dominant trait.
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Resources

NIH/National Oral Health Information Clearinghouse
1 NOHIC Way
Bethesda, MD 20892-3500
USA
Tel: 3014027364
Fax: 3019078830
TDD: 3016567581
Email: nohic@nidcr.nih.gov
Internet: http://www.nohic.nidcr.nih.gov

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)519-3194
Fax: (240)632-9164
Tel: (888)205-2311
TDD: (888)205-3223
Email: gardinfo@nih.gov
Internet: http://www.genome.gov/100000409 or http://rarediseases.info.nih.gov/html/resources/info_cntr.html

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

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated: 3/22/2001
Copyright 1988, 1989, 2001 National Organization for Rare Disorders, Inc.