Dentinogenesis imperfecta
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| Dentinogenesis imperfecta Classification and external resources | |
| ICD-10 | K00.5 |
|---|---|
| ICD-9 | 520.5 |
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Dentinogenesis imperfecta (hereditary Opalescent Dentin) is a genetic disorder of tooth development. This condition causes 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. This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. Dentinogenesis imperfecta affects an estimated 1 in 6,000 to 8,000 people.
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.It is usually an autosomal dominant trait with variable expressivity but can be recessive if the associated osteogenesis imperfecta is of recessive type.
Type II : Occurs in people without other inherited disorders ie Osteogenesis imperfecta.It is an autosomal dominant trait and is in fact on of the most common autosomal dominant disorders in human beings. A few families with type II have progressive hearing loss in addition to dental abnormalities.
Type III dentinogenesis imperfecta was first identified in a population from Brandywine, Maryland. Researchers now believe that type II and type III may be the same disorder.
Mutations in the DSPP gene have been identified in people with type II and type III dentinogenesis imperfecta. Type I occurs as part of osteogenesis imperfecta, which is caused by mutations in one of several other genes. The DSPP gene provides instructions for making three proteins that are essential for normal tooth development. These proteins are involved in the formation of dentin, which is a bone-like substance that makes up the protective middle layer of each tooth. Mutations in the DSPP gene may affect the proteins made by the gene, leading to the production of abnormally soft dentin. Teeth with defective dentin are discolored, weak, and more likely to decay and break. It remains unclear how DSPP mutations lead to hearing loss in some families with dentinogenesis type II.
Clinical Features
Clinical appearance is variable.However, the teeth usually involved and more severely affected are deciduous teeth in type 1; whereas in type 2 both the dentitions are equally affected.
The teeth may be gray to yellowish brown. They exhibit transluscent or opalescent hue.Enamel is usually lost early due to loss of scalloping at DEJ. The teeth however are not more susceptible to dental caries than normal ones.
Radiographic Features'
Type 1 and 2 show total obliteration of dentin and pulp chamber.
Type 3 shows thin dentin and extremely enormous pulp chamber.These teeth are usually known as Shell Teeth.
Histology
Dentinal tubules are irregular and are bigger in diameter. Areasof uncalcified matrix are seen.Sometimes odontoblasts are seen in dentin.
This article incorporates public domain text from The U.S. National Library of Medicine
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| Articles on Dentinogenesis imperfecta | Most recent articles on Dentinogenesis imperfecta • Most cited articles on Dentinogenesis imperfecta • Review articles on Dentinogenesis imperfecta • Articles on Dentinogenesis imperfecta in N Eng J Med, Lancet, BMJ |
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| Evidence Based Medicine Regarding Dentinogenesis imperfecta | Cochrane Collaboration on Dentinogenesis imperfecta • Bandolier on Dentinogenesis imperfecta • TRIP on Dentinogenesis imperfecta |
| Cost Effectiveness of Dentinogenesis imperfecta | Cost Effectiveness of Dentinogenesis imperfecta |
| Clinical Trials Involving Dentinogenesis imperfecta | Ongoing Trials on Dentinogenesis imperfecta at Clinical Trials.gov • Trial results on Dentinogenesis imperfecta • Clinical Trials on Dentinogenesis imperfecta at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Dentinogenesis imperfecta | US National Guidelines Clearinghouse on Dentinogenesis imperfecta • NICE Guidance on Dentinogenesis imperfecta • NHS PRODIGY Guidance • FDA on Dentinogenesis imperfecta • CDC on Dentinogenesis imperfecta |
| Textbook Information on Dentinogenesis imperfecta | Books and Textbook Information on Dentinogenesis imperfecta |
| Pharmacology Resources on Dentinogenesis imperfecta | Dosing of Dentinogenesis imperfecta • Drug interactions with Dentinogenesis imperfecta • Side effects of Dentinogenesis imperfecta • Allergic reactions to Dentinogenesis imperfecta • Overdose information on Dentinogenesis imperfecta • Carcinogenicity information on Dentinogenesis imperfecta • Dentinogenesis imperfecta in pregnancy • Pharmacokinetics of Dentinogenesis imperfecta • |
| Genetics, Pharmacogenomics, and Proteinomics of Dentinogenesis imperfecta | Genetics of Dentinogenesis imperfecta • Pharmacogenomics of Dentinogenesis imperfecta • Proteomics of Dentinogenesis imperfecta |
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| Patient Resources on Dentinogenesis imperfecta | Patient resources on Dentinogenesis imperfecta • Discussion groups on Dentinogenesis imperfecta • Patient Handouts on Dentinogenesis imperfecta • Directions to Hospitals Treating Dentinogenesis imperfecta • Risk calculators and risk factors for Dentinogenesis imperfecta |
| Healthcare Provider Resources on Dentinogenesis imperfecta | Symptoms of Dentinogenesis imperfecta • Causes & Risk Factors for Dentinogenesis imperfecta • Diagnostic studies for Dentinogenesis imperfecta • Treatment of Dentinogenesis imperfecta |
| Continuing Medical Education (CME) Programs on Dentinogenesis imperfecta | CME Programs on Dentinogenesis imperfecta |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
