Dentinogenesis imperfecta type 3

Dentinogenesis imperfecta type 3 (DGI-III), also known as Brandywine type dentinogenesis imperfecta or Shields type III, is a rare hereditary disorder affecting dentin formation in the teeth. It was originally described in a triracial isolate population from Brandywine, Maryland. DGI-III is caused by mutations in the DSPP (dentin sialophosphoprotein) gene located on chromosome 4q22.1. The condition affects both the primary (deciduous) and permanent dentitions, though primary teeth are often more severely affected. Clinically, DGI-III shares features with other forms of dentinogenesis imperfecta, including opalescent, amber-to-blue-gray discolored teeth that may appear translucent. However, DGI-III is distinguished by the occurrence of shell teeth — teeth with extremely thin dentin and enlarged pulp chambers, which can sometimes lead to multiple pulp exposures. The enamel may chip away from the abnormal underlying dentin, leading to rapid attrition and wear of the exposed dentin surfaces. Teeth may become significantly worn down, affecting chewing function and facial aesthetics. There is no cure for DGI-III, and management focuses on preserving tooth structure and maintaining dental function. Treatment strategies include the use of full-coverage crowns (such as stainless steel crowns in children) to protect teeth from wear, composite or porcelain restorations, and in severe cases, overdentures or dental implants in adulthood. Early dental intervention is recommended to prevent progressive tooth loss and to maintain occlusion. Regular dental monitoring throughout life is essential, and genetic counseling is advised for affected families.

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