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Hereditary Dentin Disorders

The Hereditary Dentine Disorders are a group of  autosomal dominant genetic conditions compound by two disorders called Dentinogenesis imperfecta (DI) and Dentin Dysplasia (DD) that affects the proper development, functional performance, and appearance of teeth.

Hereditary Dentine Disorders are rare. Dentinogenesis imperfecta affects approximately 1 person in 6,000 to 1 person in 8,000 whereas that of Dentin Dysplasia type affects 1 person in 100,000.

How does a Hereditary Dentin Disorder look like?

The Hereditary dentine disorders clinical description involves discolored teeth with issues in its structure such as bulbous crowns and pulp chambers. These disorders also affect the dentine and leave the teeth weakened and exposed to rapid wear.


There are two disorders that form part of the Hereditary Dentin Disorders, these are Dentinogenesis Imperfecta (DI) and Dentin Dysplasia (DD). Additionally, Dentinogenesis imperfecta is formed by three subtypes while Dentin Dysplasia is formed by two subtypes.

  • Dentinogenesis Imperfecta: this genetic disorder that affects the dentine and normal development of primary and permanent teeth causes the discoloration of teeth and also makes them weaker than normal and prone to early loss, wear or breakage. Currently, there are three different types of Dentinogenesis imperfecta that present similar dental abnormalities. DI Type I only affects people who suffer from Osteogenesis Imperfecta (genetic condition that causes brittle bones and prone to break). In another hand, DI type II and type III can occur and affect people that don’t have any other genetic condition.
  • Dentin Dysplasia: DD consists in other genetic disorder caused by mutations in the DSPP gene, which is the one that codes the dentin. These mutations lead to abnormalities in the development of the dentin structure and teeth root. The Dentin Dysplasia is divided into two types, DD type I affects the roots of the teeth and can cause the premature loss of teeth. DD type II affects the color primary dentition and causes pulp chamber shape anomalies and multiple intrapulpal calcifications on the teeth.


Dentinogenesis imperfecta type II and III, as well as Dentin Dysplasia type I and II are produced due to mutations presented in the DSPP gene, which encodes the dentin sialophosphoprotein.

The Dentinogenesis imperfecta type I is inherited with osteogenesis imperfecta (a genetic condition that affects the bones) and also mutations in the genes that encodes collagen type I (COLA1A1 and COL1A2).

Treatment and Management

Hereditary dentin disorders can be treated and controlled by using some procedures that could improve the functional performance and appearance of the teeth. It is primordial to have an early diagnosis and to start a treatment as soon as the disorder is detected to avoid the early loss of teeth. Keeping a good hygiene it’s also primordial to avoid more complications.

The hereditary dentin disorders may not be cured but some procedures like Sealants, veneers, dental implants, crowns, and bridges could be used to improve the appearance of the smile and to save the natural teeth. These procedures must be performed with much care since the Dentinogenesis imperfecta and Dentin dysplasia are genetic disorders that cause weakness in the teeth and make them prone to breakage.


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