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Dentinogenesis imperfecta type 3

ORPHA:166265· ICD-10 K00.5

Definition

Dentinogenesis imperfecta type 3 (DGI-3) is a rare, severe form of dentinogenesis imperfecta (DGI) characterized by opalescent primary and permanent teeth, marked attrition, large pulp chambers, multiple pulp exposure and shell teeth radiographically (i.e. teeth which appear hollow due to dentin hypotrophy).

Prevalence
Unknown
Inheritance
Autosomal dominant
Age of onset
Childhood