Developmental defects of dentine

Developmental Defects of the Dentine: Inherited Conditions and Characteristics

• Dentinogenesis imperfecta and dentin dysplasia are autosomal dominant genetic conditions that affect dentin structure.
• These conditions can affect primary or both dentitions and are characterized by brown/blue or opalescent brown discoloration.
• The prevalence of Dentinogenesis imperfecta is 1:6,000 or 1:8,000, while Dentin dysplasia type I is 1:100,000.
• The organic matrix of dentine is formed by odontoblasts, and dentin-phospho-protein acts as a nucleator of hydroxyapatite crystals during mineralization.
• Most dentin defects are caused by mutations in genes encoding the major protein constituents of dentin.
• Dentinogenesis imperfecta Type I appears in conjunction with osteogenesis imperfecta, affects both dentitions, and has variable expressivity.
• Osteogenesis imperfecta is a group of autosomal dominant conditions with thinning of cortical bone and decrease in trabecular density as the main anomaly.
• Dentinogenesis imperfecta II is similar to DGI-type I, but without osteogenesis imperfecta, and affects mainly primary teeth.
• Dentinal dysplasia type 1 affects root dentine, causes shortening of roots, and affects mainly permanent teeth.
• Dentinal dysplasia type 2 mainly affects crowns of deciduous teeth, is autosomal dominant, and resembles DGI-II.
• There are some syndromes and systemic conditions associated with Dentinogenesis imperfecta, such as Ehler Danlos syndrome and Schimke immune-osseous dysplasia.
• Treatment for these conditions varies and requires a multidisciplinary team, including preventive measures, periapical pathology treatment, good oral hygiene, and unsatisfactory root treatment for obliterated root canals.