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Volume 2 Issue 3 (July-September, 2014)

Review Articles

AMELOGENESIS IMPERFECTA- A REVIEW
Pankhuri Nigam, Vijay Pal Singh, Krishnadeo Prasad, Jalaj Tak, Anju Sinha, Parveen Grewal


Year: 2014 // Volume: 2/ / Issue: 3/ / Page: 83-90
Department of Oral Pathology, S.B.B.D.C, Ghaizabad, U.P, India
Corresponding Author: Dr. Pankhuri Nigam, Address - Pocket G-5/6, Sector-11, Rohini, Delhi-110085, E-mail: pankhurinigam85@gmail.com
This article may be cited as: Nigam P, Singh VP, Prasad K, Tak J, Sinha A, Grewal P. Amelogenesis Imperfecta- A Review. J Adv Med Dent Scie Res 2014;2(3):83-90.
Dental enamel is the epithelial derived hard tissue covering the crowns of teeth it is most highly mineralized and hardest tissue of the body. Dental enamel is acellular and has no physiological means of repair outside of that provided by saliva. Amelogenesis imperfect (AI) is a disorder which affects the enamel structurally as well as clinically of all or nearly all the teeth in a more or less equal manner. The enamel may be hypoplastic, hypomineralized or both. AI is a group of inherited defects of dental enamel formation that show both clinical and genetic heterogeneity. Enamel findings in AI are highly variable, ranging from deficient enamel formation to defects in the mineral and protein content. AI exists in isolation or associated with other abnormalities in syndromes. It may show autosomal dominant, autosomal recessive, sex-linked and sporadic inheritance patterns. In families with an X-linked form it has been shown that the disorder may result from mutations in the amelogenin gene, AMELX. The enamelin gene, ENAM, is implicated in the pathogenesis of the dominant forms of AI. Autosomal recessive AI has been reported in families with known consanguinity. Diagnosis is based on the family history, pedigree plotting and meticulous clinical observation.

 
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