Dentin dysplasia

Dentin Dysplasia is a rare genetic disorder that affects the formation of teeth, specifically the dentin. The dentin is the hard tissue located beneath the enamel that forms the bulk of a tooth. Dentin dysplasia is characterized by abnormal dentin formation that can affect both primary (baby) and permanent teeth.

Types

There are two types of dentin dysplasia, Type I and Type II.

• Type I, also known as radicular dentin dysplasia, is characterized by short roots, obliterated pulp chambers, and periapical abscesses without any obvious cause.

• Type II, also known as coronal dentin dysplasia, is characterized by primary teeth that appear amber and translucent. The pulp chambers of these teeth may be completely or partially filled with dentin.

Symptoms

The symptoms of dentin dysplasia vary depending on the type. Common symptoms include:

• Short roots
• Abscesses without any obvious cause
• Amber-colored primary teeth
• Translucent teeth

Causes

Dentin dysplasia is caused by mutations in the DSPP gene. This gene provides instructions for making two proteins, dentin sialophosphoprotein and dentin phosphoprotein, that are essential for the formation of dentin.

Diagnosis

Diagnosis of dentin dysplasia is based on a clinical examination of the teeth, dental X-rays, and genetic testing.

Treatment

Treatment for dentin dysplasia is focused on maintaining the affected teeth for as long as possible. This may involve regular dental check-ups, good oral hygiene, and in some cases, dental procedures such as root canals or dental implants.

See Also

• Genetic Disorders
• Tooth Abnormalities
• DSPP Gene

References

Dentin dysplasia Resources
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