What is the Amelogenesis ?
The crown of a tooth is composed of enamel and dentin. The enamel is the outer layer of a whitish color mostly composed of hard anorganic substance - over 96% and organic matter and water - less than 4%. This layer is much tougher and more resistant than the inside dentin that has a yellowish color and represents only 45% hard anorganic substance, the rest being 33% organic matter and 22% water.
Amelogenesis is the formation of dental enamel and begins when the crown forms within the maxillary bones, after the dentinogenesis (formation of the first dentin layer) and before tooth root formation.
What is Amelogenesis Imperfecta ?
Amelogenesis Imperfecta is a rare congenital disorder in which the teeth exhibit an abnormal development and mineralization of enamel unrelated to other systemic or general diseases. It is due to the malfunction of the enamel proteins (ameloblastin, enamelin, tuftelin and amelogenin) and results in a lower concentration of the anorganic substance in the enamel and low resistance to chemical and physical factors in the oral cavity.
Patients with this condition have an abnormal color of the teeth (yellow, brown or gray), and may have signs of the disease on all teeth or only a part of them. The disease is manifested in a variety of ways, depending on the type of amelogenesis, with defects in the shape or volume of the teeth. Teeth are hypersensitive to temperature variations, more susceptible to cavities, rapid wear (abrasion, bruising), excessive tartar and gingival hyperplasia.
Signs may arise from the teeth eruption period, but in the case of permanent teeth they become more apparent between 20 and 30 years, and the progression of the disease even results in the total loss of dental crowns after the age of 30.