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Dentin Dysplasia Type II

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Dentin Dysplasia Type II 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

  • coronal dentin dysplasia
  • DD-II

Disorder Subdivisions

  • None

General Discussion

Dentin dysplasia type II, also known as coronal dentin dysplasia, is a rare genetic disorder that affects the teeth. It is characterized by abnormal development (dysplasia) of dentin. Dentin is the hard tissue found beneath the enamel that surrounds and protects the pulp and forms the major part of teeth. Affected children may exhibit brownish-blue discoloration of baby teeth (primary or deciduous teeth) and obliteration of the pulp chambers. Permanent teeth are usually unaffected or only mildly affected. Dentin dysplasia type II only affects the teeth. The disorder is caused by mutations of the DSPP gene.



Dentin dysplasia type II belongs to a group of disorders known as the hereditary dentin disorders. In 1973, a physician and his colleagues defined five disorders characterized by inherited dentin defects (Shields classification). Many physicians have noted that the Shields classification is out of date. As new research reveals genetic mutations and better defines these disorders, a new classification system will be warranted. Unfortunately, the current understanding of these disorders is insufficient to allow the creation of this updated classification.

Resources

March of Dimes Birth Defects Foundation

1275 Mamaroneck Avenue

White Plains, NY 10605

Tel: (914)997-4488

Fax: (914)997-4763

Tel: (888)663-4637

Email: Askus@marchofdimes.com

Internet: http://www.marchofdimes.com



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/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

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:  1/14/2011

Copyright  1988, 1989, 2001, 2011 National Organization for Rare Disorders, Inc.

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