Dentin Dysplasia Type II
National Organization for Rare Disorders, Inc.
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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 Many physicians have noted that the Shields classification is out of date.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.
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Last Updated: 1/28/2014
Copyright 2014 National Organization for Rare Disorders, Inc.
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