Amelogenesis imperfecta (AI) is an inherited disorder which results in enamel defects.
The main clinical characteristics are extensive loss of tooth tissue, poor esthetics and tooth sensitivity.
Early recognition followed by appropriate preventive care and oral rehabilitation is essential in the successful management of AI.
This clinical report describes the management of a 3 year-old girl with a hypocalcified type of Al.
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The patient presented early extensive tissue loss of the superior incisors and molars, with general dentinal sensitivity.
The first phase of the treatment was preventive measures to improve dental and periodontal health. On the second phase the anterior teeth were extracted and the molars were endodontic treated and covered with stainless steel crowns.
A functional appliance was made to rehabilitate the anterior extracted teeth on the third phase.
The performed treatment achieved satisfactory esthetic and function, which resulted in an improvement in the child’s quality of life.
Introduction and Review of literature
Amelogenesis imperfecta (AI) is a group of inherited disorders characterized by abnormal enamel formation. These non-syndromic disorders are not associated with defects of other parts of the body or any other health problem.
These enamel defects are a result of gene mutations associated with amelogenin protein and enamelin protein, which are secreted during the amelogenesis process.
However, most forms of AI do not have a defined molecular basis at this time. AI follows an autossomal dominant, autossomal recessive or X-linked pattern of inheritance.
°Natalino Lourenço Neto / Marco A.B. Paschoal / Tatiana Y. Kobayashi / Daniela Rios1, Salete M.B. Silva