amelogenesis imperfecta


Also found in: Dictionary, Thesaurus, Legal, Financial, Encyclopedia, Wikipedia.
Related to amelogenesis imperfecta: dentinogenesis imperfecta

amelogenesis

 [ah-mel″o-jen´ĕ-sis]
formation of dental enamel.
amelogenesis imperfec´ta a hereditary disease in which there is imperfect formation of enamel, resulting in brownish coloration and friability of the teeth.

am·e·lo·gen·e·sis im·per·fec·ta

a group of hereditary ectodermal disorders in which the enamel is defective in structure or deficient in quantity. Three major groups are recognized: hypoplastic types, with defective enamel matrix deposition but normal mineralization; hypomineralization types, with normal matrix but defective mineralization; and hypomaturation type, in which the enamel crystallites remain immature. The several types may be inherited as autosomal dominant [MIM*104500, 104510, 104530], recessive [MIM*204650, 204690, 204700] or X-linked [MIM*301100, 301200, 301201].

amelogenesis imperfecta

(ĭm′pər-fĕk′tə)
n.
A hereditary condition in which the dental enamel does not develop properly, often because of insufficient calcification.

amelogenesis imperfecta

a condition characterized by brown or white chalky discoloration of the teeth and resulting from either severe enamel hypocalcification or enamel hypoplasia. The condition, which is inherited as an autosomal-dominant trait, is classified according to severity: in agenesis, there is a complete lack of enamel; in enamel hypoplasia, defective matrix formation causes the enamel to be normal in hardness but deficient in quantity; and in enamel hypocalcification, defective maturation of ameloblasts results in enamel that is normal in quantity but soft and undercalcified. Also called hereditary brown enamel,hereditary enamel hypoplasia.Compare dentinogenesis imperfecta.

am·e·lo·gen·e·sis im·per·fec·ta

(amĕ-lō-jenĕ-sis im-pĕr-fektă)
Hereditary ectodermal disorders in which dental enamel is defective in structure or deficient in quantity.

amelogenesis imperfecta

A hereditary defect of the mineralization of tooth enamel which is unusually soft and is rapidly worn away.

am·e·lo·gen·e·sis im·per·fec·ta

(amĕ-lō-jenĕ-sis im-pĕr-fektă)
Group of hereditary ectodermal disorders in which tooth enamel is defective in structure or deficient in quantity. Three major groups are recognized.
Synonym(s): enamel dysplasia.

amelogenesis imperfecta (am´əlōjen´əsis),

n a broad category of developmental disturbances in the structural formation of enamel. The disease is divided into four main types (type 1, Hypoplastic; type 2, Hypomaturation; type 3, Hypocalcified; type 4, mixed) and 15 subtypes, which range from mild to severe.
Enlarge picture
Amelogenesis imperfecta.

amelogenesis

formation of dental enamel.

amelogenesis imperfecta
imperfect formation of enamel, resulting in brownish coloration and friability of the teeth.
References in periodicals archive ?
Characterization of molecular defects in X-linked amelogenesis imperfecta (AIH1).
Clinical features of a family with X-linked amelogenesis imperfecta mapping to a new locus (AIH3) on the long arm of the x chromosome.
Some oro-dental findings, such as congenitally missing teeth, amelogenesis imperfecta or cleft of the lip and palate, may be isolated defects.
Mutation in kallikrein 4 causes autosomal recessive hypomaturation amelogenesis imperfecta.
white spot lesions, white cuspal ridges or a patchy appearance of amelogenesis imperfecta or fluorosis.
Mutations in genes involved in enamel (AMELX, ENAM, MMP20, and KLK4) identified, as possible causes of amelogenesis imperfecta, have not been tested.
Certainly, some of the findings are similar to known developmental defects, such as lack of enamel, crown resorption, and delayed eruption that may be associated with severe amelogenesis imperfecta (AI).