Dentinogenesis imperfecta type I: A case report with literature review on nomenclature system

Abstract

Dentinogenesis imperfecta (DI) is an inherited disorder affecting dentin. Defective dentin formation results in discolored teeth that are prone to attrition and fracture. Mutation in dentin sialophosphoprotein (DSPP) has been found to cause the dentin disorders DI - I and II (shields II and III). Early diagnosis and treatment of DI is recommended as it may prevent or intercept deterioration of the teeth and occlusion and improve esthetics. Here, we report a case with characteristic clinical, radiological and histological features of DI-I. The etiology and classification followed in literature is confusing since dentinoenamel junction (DEJ) in DI seems to be structurally and functionally normal and DI is clearly a disorder distinct from osteogenesis imperfecta (OI), but we still relate etiology of DI to DEJ and follow Shields classification. Therefore, we have briefly reviewed etiology and nomenclature system of DI.

Dentinogenesis Imperfecta: A Case Report of Five Patients in the Same Family Group

“…Majorana et al (2010) added that the temporary dentition is affected more severely than the permanent, the damage being usually less severe in permanent teeth, and in some cases, almost clinically undetectable. The literature (Barron et al, 2008;Rabassa et al;Devaraju et al) describe some radiographic features of teeth affected by DI: crowns with bullous appearance, which have a very strong constriction at the cervical level, shorter and thin roots, and the reduction or obliteration of the pulp chamber. In addition, the same authors pointed out that these features occur in both temporary and permanent teeth, and that the other tissues show a normal appearance.…”

“…It is originated in the histodifferentiation during odontogenesis process, being a localized form of mesodermal dysplasia characterized by an alteration of the dental proteins (Surendra et al, 2013;Devaraju et al, 2014). DI types II and III are caused by a defect in dentin sialophosphoprotein gene (DSPP) (Kim & Simmer, 2007).…”

Tip II dentinogenezis imperfekta’nın protetik tedavisi: olgu bildirimi

“…DI types I, II, and III are caused by the dentin sialophosphoprotein (DSPP) mutation. 13 As a result of defective dentin formation, the teeth are prone to break. The main oral problems seen in DI are residual roots, a reduced vertical dimension, hypodontia, and extensive decay.…”

“…[8][9][10][11][12] A wide range of treatments are available for the permanent teeth in patients with DI: carbamide peroxide bleaching, veneers, dental implants, overdenture prostheses, faceting or stainless steel crowns on the anterior teeth, a simple removable appliance, metalceramic restorations, crowns jackets, and adhesive castings. [8][9][10][11][12][13][14][15][16][17] In the presented case, we used metalceramic fixed dentures in the lower jaw and an overdenture prosthesis in the upper jaw due to multiple tooth loss, with the aim of restoring the patient's vertical dimension.…”

“…The need for invasive procedures can be avoided by preventing dental caries, periapical lesions, and premature tooth loss by proper oral hygiene, diet, and restoration. [13][14][15][16] The patient's age, degree of tooth destruction, patient cooperation, and dentition period are the most important criteria in the selection of therapy for treating the oral problems in DI. Dentinogenesis imperfecta patients can develop severe esthetic and functional problems.…”