title: Dental management of severe dentinogenesis imperfecta in a mild form of osteogenesis imperfecta.
abstract: Dentinogenesis Imperfecta (DI), in which the teeth are discolored, translucent and brittle, can occur in isolation as a familial trait and as a component of the skeletal dysplasia Osteogenesis Imperfecta (OI). In a Cape Town family, 20 persons in 3 generations had mild OI, with the additional manifestation of severe DI. The family was assessed at the Dental Genetic Unit of the University of the Western Cape and appropriate dental treatment was provided. In this setting, a detailed treatment plan was devised for a severely affected woman. This plan proved to be efficient and cost effective, and the final outcome was pleasing to the patient. Dentinogenesis Imperfecta is not uncommon and may well be encountered in conventional dental practice. The necessary clinical expertise is within the scope of the skills of the general dentist.
authors.affiliation: Faculty of Dentistry, University of the Western Cape, UWC Dental Special Dental Clinic for Genetic Disorders, Red Cross Children's Hospital, Rondebosch, Cape Town, South Africa. LStephen@uwc.ac.za
authors.forename: L X G
authors.initials: LX
authors.lastname: Stephen
authors.forename: P
authors.initials: P
authors.lastname: Beighton
mesh_terms: D000293:Adolescent; D003442:Crowns; D003811:Dentinogenesis Imperfecta / Q000150:complications / Q000209:etiology / Q000628:therapy*; D003828:Denture, Overlay / Q000191:economics; D005260:Female; D006801:Humans; D008310:Malocclusion / Q000209:etiology / Q000628:therapy*; D016876:Metal Ceramic Alloys; D010013:Osteogenesis Imperfecta / Q000150:complications*; D019217:Tooth Attrition / Q000209:etiology / Q000534:rehabilitation*; D014716:Vertical Dimension
chemical_list: D016876:Metal Ceramic Alloys