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What is Amelogenesis Imperfecta? 1. It is an abnormal formation of the enamel. 2. It is a disorder that affects both primary dentition (baby teeth) and permanent dentition (permanent teeth). 3. Conditions may vary, they can have smaller teeth than normal (microdontia), discoloring, grooved or pitted, and prone to fractures. 4. Amelogenesis Imperfecta is inherited 5. A ratio of 14,000 people to 1 is affected in the United States. Treatment - Since the enamel is easily fractured and damaged, it will affect the appearance of teeth. As hygienist we can advise the patient to seek a dentist and if they don’t have one we’ll provide them with one. The treatment depends on the severity of the problem, the dentist will determine that after diagnose. Full crowns will improve the appearance of the teeth and protect them from damage. Clinical and Hereditary Characteristics of Four Main AI Types (Table 1) [8] Type Clinical Appearance Enamel Thickness Radiographic Appearance Inheritance Hypoplastic (Type I) Crowns size varies from small to normal, small teeth may lack proxmial contacts, color varies from normal to opaque white – yellow brown Varies from thin and smooth to normal thickness with grooves, furrows and/or pits Enamel has normal to slightly reduced contrast/ thin Autosomal dominant, recessive, or Xlinked Hypomaturation (Type II) Varies from creamy opaque to marked yellow/brown, surface of teeth soft and rough, dental sensitivity and open bite common Normal thickness with enamel that often chips and abrades easily Enamel has contrast similar to or > than dentin, unerupted crowns have normal morphology Autosomal dominant, recessive, or Xlinked Hypocalcified (Type III) Opaque white to yellow-brown, soft rough enamel surface, dental sensitivity and open bite common, heavy calculus formation common Normal thickness with enamel that often chips and abrades easily Enamel has contrast similar to or < dentin, unerupted crowns have normal morphology Autosomal dominant, recessive Hypomaturation/ Hypoplasia/ Taurodontism (Type IV) White/Yellow- Brown mottled, teeth can appear small and lack proximal contact Reduced, hypomineralized areas and pits Enamel contrast normal to slightly > dentin, large pulp chambers Autosomal dominant - http://www.dentistry.unc.edu/research/defects/pages/ai.htm Crowns are sometimes being used to compensate for the soft enamel. Usually stainless steel crowns are used in children who may be replaced by porcelain once they reach adulthood. In the worst case scenario, the teeth may have to be extracted and implants or dentures are required. People afflicted with Amelogenesis Imperfecta have teeth with abnormal color: yellow, brown or grey. The teeth have a higher risk for dental cavities and are hypersensitive to temperature changes. This disorder can afflict any number of teeth. Mutations in the AMELX, ENAM, MMP20, and KLK-4 genes have been found to cause Amelogenesis Imperfecta (non-syndromic form). The AMELX, ENAM, KLK-4 and MMP20 genes provide instructions for making proteins that are essential for normal tooth development. These proteins are involved in the formation of enamel, which is a hard, calcium-rich material that forms the protective outer layer of each tooth. Mutations in any of these genes alter the structure of these proteins or prevent the genes from making any protein at all.