AMELOGENESE IMPERFEITA PDF

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CiteScore measures average citations received per document published. Read more. SRJ is a prestige metric based on the idea that not all citations are the same.

SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal's impact. SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field. Amelogenesis imperfecta constitutes an abnormality of enamel development, genomic in origin.

The prevalence is and the enamel may be hypoplastic, hypomineralised or hypoplastic - hypomineralised with taurodontism.

This condition may show autosomal dominant, autosomal recessive or sex-linked dominant or recessive inheritance patterns. Several mutations have been identified in amelogenin, enamelin, kallikrein-4 and enamelysin genes, known to be involved in enamel formation. Mutations of the amelogenin gene cause X-linked amelogenesis imperfecta. The enamelin gene is implicated in the pathogenesis of the dominant forms of AI, while the recessive forms may result from mutations in the kallikrein-4 and enamelysin genes.

Amelogenesis imperfecta exists in isolation forms or associated with other abnormalities, in syndromes, and frequently presents problems of function and socialization, but may be managed by early intervention. This article pretends not only developing a deep knowledge about amelogenesis imperfecta, but mostly, with it, alert all the dental health professionals for the gravity of this malformation, associated to serious esthetics and psychiatric problems..

Rev Port Estomatol Cir Maxilofac ;— Indexed in: Scopus See more Follow us:. Discontinued publication For more information click here. Previous article Next article. Issue 4. Pages October - December Download PDF. This item has received. Under a Creative Commons license. Article information. This article pretends not only developing a deep knowledge about amelogenesis imperfecta, but mostly, with it, alert all the dental health professionals for the gravity of this malformation, associated to serious esthetics and psychiatric problems.

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Amelogenesis imperfecta

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. An exceedingly rare form of brachyolmia, characterized by mild platyspondyly, broad ilia, elongated femoral necks with coxa valga, scoliosis, and short trunked short stature associated with amelogenesis imperfecta of both primary and permanent dentition.

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Amelogênese imperfeita

Amelogenesis imperfecta is characterized by enamel structural defects, which may severely affect dental structure in both dentitions. When accompanied by severe decay, it may impair the pulp complex requiring endodontic treatment and in case of incomplete root formation treatment becomes more complex due to pulp volume and anatomic conditions. This study aimed at reporting a clinical case of patient with amelogenesis imperfecta and with multiple incomplete root formation as a consequence. Female patient, 12 years old, leucodermic, with painful symptoms, who has looked for dental assistance. At intraoral clinical evaluation teeth presented with shape and size changes, yellowish color, covered by a thin enamel layer with roughened surface and absent in some areas, with anterior vestibular sulcus fistula and without edema.

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