a disorder of tooth developmentpresents with abnormal formation of the enamel or externallayer of teeth.due to the malfunction of the proteins in the enamel:ameloblastin, enamelin, tuftelin and amelogenin. condition causes teeth to be unusuallysmall, discolored, pitted or grooved, and prone to rapid wearand breakage. these defects, which vary among affected individuals, canaffect both primary (baby) teeth and permanent teeth. How common is amelogenesis imperfecta?The exact incidence of amelogenesis imperfecta is uncertain.Estimates vary widely, from 1 in 700 people in northern Swedento 1 in 14,000 people in the United States.
People afflicted with amelogenesisimperfecta have teeth with abnormalcolor: yellow, brown or grey. The teethhave a higher risk for dental cavitiesand are hypersensitive to temperaturechanges. This disorder can afflict anynumber of teeth.
Mutations in the AMELX, ENAM, and MMP20 genes cause amelogenesis imperfecta.
The enamel of the tooth is soft and thin. The teeth appear yellow and are easily damaged. Both baby teeth and permanent teeth are affected.
X-ray showing lack of enamel opacity and a pathological loss of enamel in patient with amelogenesis imperfecta Fullcrowns are sometimes being used to compensate for the soft enamel.Usually stainless steel crowns are used in children which 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.