Dentinogenesis imperfecta, shields type 3
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Dentinogenesis imperfecta type III is one of five distinct, hereditary disorders of dentin (the bone-like substance that makes up most of the tooth) development affecting the teeth.[2] This condition causes the teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent. Teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (baby) teeth and permanent teeth. Dentinogenesis imperfecta type III is caused by mutations in the DSPP gene which are inherited in an autosomal dominant fashion.[1] Treatment, which may begin as early as infancy, usually continues into adulthood with a number of options including the use of crowns, over-dentures and dental implants depending on the age of the patient and the condition of the dentition.[3] Individuals with dentinogenesis imperfecta type III appear to be limited, in large measure, to a population in the region around Brandywine in southern Maryland.[2]
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Genetics Home Reference (GHR) contains a condition summary on Dentinogenesis imperfecta, shields type 3. Click on the link to go to GHR and review this summary.
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The National Institute of Dental and Craniofacial Research Institue (NIDCR), purposes to improve oral, dental and craniofacial health through research, research training, and the dissemination of health information. Click on the link to view information on this topic.
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The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
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The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Dentinogenesis imperfecta, shields type 3. Click on the link to go to OMIM and review these resources.
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Orphanet is a database dedicated to information on rare diseases and orphan drugs. Access to this database is free of charge. Click on the link to read information on this topic.
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PubMed is a searchable database of medical literature and lists journal articles that discuss Dentinogenesis imperfecta, shields type 3. Click on the link to view a sample search on this topic.
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