Dentinogenesis imperfecta

Dentinogenesis imperfecta is a genetic disorder affecting the development of dentin, the hard tissue beneath the enamel of a tooth. This condition is characterized by discolored, translucent teeth that are prone to wear and breakage. It is often associated with other genetic conditions, such as osteogenesis imperfecta.

Classification

Dentinogenesis imperfecta is classified into three types:

• Type I: Occurs in conjunction with osteogenesis imperfecta. Teeth may appear blue-gray or yellow-brown and are often translucent.
• Type II: Also known as hereditary opalescent dentin, this type occurs without any associated systemic disorder. The teeth have a similar appearance to Type I.
• Type III: Known as the Brandywine type, it is rare and primarily found in a racial isolate in Maryland, USA. Teeth may have multiple pulp exposures and shell-like appearance.

Pathophysiology

The condition is caused by mutations in the DSPP gene, which encodes for dentin sialophosphoprotein, a protein crucial for normal dentin formation. These mutations lead to abnormal dentin structure, resulting in the characteristic clinical features of the disorder.

Clinical Features

Patients with dentinogenesis imperfecta typically present with:

• Discolored teeth, often described as opalescent
• Increased susceptibility to dental caries
• Rapid wear and attrition of teeth
• Possible pulpal exposure and abscess formation

Diagnosis

Diagnosis is primarily clinical, supported by radiographic findings. Radiographs may show:

• Obliteration of the pulp chambers
• Short, constricted roots
• "Shell teeth" appearance in Type III

Treatment

Management of dentinogenesis imperfecta focuses on preserving tooth structure and function. Treatment options include:

• Dental crowns to protect teeth from wear
• Endodontic therapy for teeth with pulp exposure
• Orthodontic treatment to address malocclusion

Related Pages

• Dentin
• Osteogenesis imperfecta
• Tooth development

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  Dentinogenesis imperfecta