Amelogenesis Imperfecta


Researchers from University of Ljubljana discuss findings in syndrome



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This article was published in Disease Prevention Week, which you can subscribe to online.

2007 AUG 21 -- According to recent research from Ljubljana, Slovenia, "The prominent dental feature of a boy was severely hypoplastic enamel in both primary and permanent teeth. Case Report. Many permanent teeth were already infected while emerging in the oral cavity."

"Panoramic radiograph showed enlarged and elongated pulp chambers (taurodontism) in the permanent first molars. The clinical and radiological diagnosis was either hypomaturation-hypoplastic amelogenesis imperfecta with taurodontism (AIHHT) or tricho-dento-osseous syndrome (TDO). Histological examination of the upper right permanent first molar revealed thin lamellar or somewhat thicker amorphous enamel on approximal surface only with no rods or incremental lines visible. Histologically, the Witkop type AIG designated 'enamel agenesis' cannot be excluded. The medical and dental history of the family members, as well as the boy's medical examination, was noncontributing. He had thick, blond, curly hair. The bone structure of the jaws and skull was normal," wrote A. Pavlic and colleagues, University of Ljubljana.

The researchers concluded: "For genetic analysis, DLX3 gene was sequenced but no mutation was found. Since the gene defect of TDO has been localized only in the DLX3 gene, the more probable diagnosis was AI."

Pavlic and colleagues published their study in International Journal of Paediatric Dentistry (Severely hypoplastic amelogenesis imperfecta with taurodontism. International Journal of Paediatric Dentistry, 2007;17(4):259-266).

For additional information, contact A. Pavlic, University of Ljubljana, Dept. of Pediatric & Prevention Dental, Faculty Medical, Hrvatski Trg 6, SI-1000 Ljubljana, Slovenia.

Publisher contact information for the International Journal of Paediatric Dentistry is: Blackwell Publishing, 9600 Garsington Rd., Oxford OX4 2DQ, Oxon, England.

Keywords: Slovenia, Ljubljana, Pediatric Dentistry, Syndrome, University of Ljubljana.

This article was prepared by Disease Prevention Week editors from staff and other reports. Copyright 2007, Disease Prevention Week via NewsRx.com.