Description Dentinogenesis imperfecta is a disorder of tooth development. 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.
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Description Dentinogenesis imperfecta is a disorder of tooth development. 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. Researchers have described three types of dentinogenesis imperfecta with similar dental abnormalities. Type I occurs in people who have osteogenesis imperfecta , a genetic condition in which bones are brittle and easily broken. Dentinogenesis imperfecta type II and type III usually occur in people without other inherited disorders. A few older individuals with type II have had progressive high-frequency hearing loss in addition to dental abnormalities, but it is not known whether this hearing loss is related to dentinogenesis imperfecta.
Some researchers believe that dentinogenesis imperfecta type II and type III, along with a condition called dentin dysplasia type II, are actually forms of a single disorder. The signs and symptoms of dentin dysplasia type II are very similar to those of dentinogenesis imperfecta.
However, dentin dysplasia type II affects the primary teeth much more than the permanent teeth. Related Information.
Metrics details Abstract The hereditary dentine disorders, dentinogenesis imperfecta DGI and dentine dysplasia DD , comprise a group of autosomal dominant genetic conditions characterised by abnormal dentine structure affecting either the primary or both the primary and secondary dentitions. DGI is reported to have an incidence of 1 in 6, to 1 in 8,, whereas that of DD type 1 is 1 in , Clinically, the teeth are discoloured and show structural defects such as bulbous crowns and small pulp chambers radiographically. The underlying defect of mineralisation often results in shearing of the overlying enamel leaving exposed weakened dentine which is prone to wear. Currently, three sub-types of DGI and two sub-types of DD are recognised but this categorisation may change when other causative mutations are found. Diagnosis is based on family history, pedigree construction and detailed clinical examination, while genetic diagnosis may become useful in the future once sufficient disease-causing mutations have been discovered.
Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional. This website is maintained by the National Library of Medicine. In-Depth Information The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers.