Amelogenesis imperfecta type IE

Common Name(s)

Amelogenesis imperfecta type IE, Amelogenesis imperfecta hypopolastic/hypomaturation X-linked 1

Amelogenesis imperfecta type 1E is a specific type of Amelogenesis Imperfecta (AI) in which the tooth enamel does not develop to full thickness. Tooth enamel is normally the visible part of the human tooth and is the hardest substance in the human body. The color of tooth enamel normally ranges from light yellow to grayish or bluish white.This causes the teeth to be unusually small. As with all forms of AI, the enamel of the teeth is discolored, pitted or grooved, and brittle. In this form, the teeth are often referred to as "snow-capped" meaning they have white patches on the suface. This is sometimes mistaken for fluorosis, a condition that occurs when there is over-exposure of fluoride in childhood. There are restorative therapies for this condition including bonding porcelain or composite resin to existing tooth enamel. Orthodontic therapy, braces, and crowns, can also help fix teeth spacing and misaligned jaw bites. This type of AI is x-linked which means that the changed gene is present on the X chromosome (one of the sex chromosomes). In most cases, males affected with X-linked AI have more severe dental abnormalities than females who are affected with this condition. Females can show a wide variation in presentation, from very mild to more severe. Vertical ridges in the enamel has been noted in some females. Talk to your doctor or orthodontist to see if you or your child has been diagnosed with this condition to find the right treatment options for you.

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Scientific Literature

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Symptoms, Diagnosis, and Treatment

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