Dentinogenesis imperfecta is a genetic disease that causes brittle and discolored teeth due to abnormal development of dentin. It can be inherited and is recognizable in childhood. Treatment options include cosmetic dental procedures and artificial implants.
Dentinogenesis imperfecta is a genetic disease that makes teeth particularly brittle and discolored. The name refers to the underdeveloped or abnormal development of the internal material in the teeth, called dentin. Dentinogenesis imperfecta tends to affect both baby and permanent teeth, and many patients who acquire the condition also suffer from bone fragility and deformity. There is currently no cure for the disorder, but cosmetic dental procedures are available to improve the appearance and function of the teeth.
Researchers have identified several very specific genes that contribute to the growth of hard tissues in the body, including the dentin and enamel that make up teeth. When a particular gene is mutated or inactivated, it is unable to produce the proteins needed to form strong, hard dentin. Most cases of dentinogenesis imperfecta are inherited in an autosomal dominant fashion, which refers to the fact that only one of a child’s parents needs to carry a mutated copy of the gene for it to be passed on.
Most cases of dentinogenesis imperfecta are recognizable in early childhood once the primary teeth begin to grow. They are typically smaller than the average baby tooth and have a jagged appearance. Teeth often have a gray, brown, or yellow tint and may appear partially translucent because the outer enamel layer is very thin. Children with dentinogenesis imperfecta tend to lose their teeth at an earlier age than their peers.
As permanent teeth grow in, they share the same unusual characteristics. Teens and adults with this condition are prone to breaking their teeth easily. Misaligned teeth usually cannot be corrected with traditional techniques such as braces because they are too fragile. Permanent teeth can start falling out as early as the third or fourth decade of life.
A dentist or pediatrician can diagnose dentinogenesis imperfecta based on the physical appearance of the teeth. Genetic blood tests may be done to confirm the presence of mutated genes. Dentinogenesis imperfecta can sometimes be a symptom of a larger underlying disorder called osteogenesis imperfecta, which impairs the body’s ability to build and maintain strong bones. Whole-body radiographs are often done to confirm or rule out bone involvement in patients initially seen for dental problems.
Treatment for dentinogenesis imperfecta depends on the severity of the condition and the age of the patient. In many cases, doctors prefer to wait for permanent teeth to grow in before making treatment decisions. Options include covering damaged teeth with a synthetic form of enamel or placing crowns on particularly delicate or broken teeth to protect them. In some cases, teeth are removed completely and replaced with permanent artificial implants.
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