Dentin Dysplasia Type II
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.
- coronal dentin dysplasia
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 Many physicians have noted that the Shields classification is out of date.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.
March of Dimes Birth Defects Foundation
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NIH/National Institute of Dental and Craniofacial Research
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Genetic and Rare Diseases (GARD) Information Center
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