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Amelogenesis imperfecta

Amelogenesis imperfecta Definition

Amelogenesis imperfecta is a disorder of a person's tooth development, which causes teeth to become discolored, grooved, pitted, prone to breakage, become unusually small and develop other dental abnormalities. This condition produces varying effects and can affect both baby teeth and permanent teeth. Fourteen types of amelogenesis imperfecta exist. Each type is distinguished by specific dental abnormalities present. All types of amelogenesis imperfecta can affect any number of teeth. People with amelogenesis imperfecta develop teeth with abnormal color, such as grey, brown or yellow. Their teeth have greater risk of developing cavities and become hypersensitive to changes in the temperature. It is estimated that 1 in 14,000 people in the U.S. and 1 in 700 people in Sweden suffer from amelogenesis imperfecta. However, the exact incidence of this condition remains uncertain.

Amelogenesis imperfecta Genetics

Mutations in the KLK4, ENAM, AMELX and MMP20 genes are discovered to cause amelogenesis imperfecta. These genes provide instructions to make proteins needed to form enamel and for normal tooth development. When mutations occur in any of these genes, the protein structures are changed and stops the genes from making protein, which in turn, results in abnormally soft and thin enamel with brown or yellow color. When this happens, teeth become weak and easily damaged.

Amelogenesis imperfecta Treatment

To compensate for soft enamel, crowns are usually used. Children with this condition usually use stainless steel crowns, which are replaced by porcelain crowns once they become adults. For severe cases of amelogenesis imperfecta, teeth could be extracted and replaced by dentures or implants.

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