Amelogenesis Imperfecta Hypomaturation


Type of disease: Rare conditions

Amelogenesis Imperfecta Hypomaturation (AI hypomaturation) is a rare genetic disorder that causes the abnormal formation of tooth enamel. 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. Amelogensis imperfecta in general causes teeth to be abnormally small, discolored, pitted or grooved, and brittle. The hypomaturation type means there are defects in the later stage of enamal formation. The main symptoms for this type of AI include having opaque and porous tooth enamel. They may also have yellow, brown, or grey teeth and are at higher risk for dental cavities as well as the teeth being hypersensitive to temperature changes. This type of AI can be inherited or passed through famillies in an autosomal dominant, recessive or x-Linked manner. In most cases, males affected with x-linked AI have more severe dental abnormalities than females who are affected with this condition. A genetic counselor can provide a better understanding of the underlying causes and recurrence risks. If you or a family member may have these symptoms, please consult your doctor and/or dentist.

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