TAURODONTISM AND KLINEFELTER SYNDROME: CASE REPORT AND REVIEW OF THE LITERATURE

This paper presents aseven-year old patient with Klinefelter Syndrome who needs dental treatmentbecause of pain in his teeth. The patient had mildmental retardation, learning and speaking difficulties, taurodonticteeth, missing premolars and incisor teeth. This paper emphasizes theimportance of regular dental care and panoramicradiographs to lead early recognisation of systemic disorder and improvequality of patient’s life.Klinefelter’s syndrome (KS) is a disorder first described by Dr. Klinefelter et al. in 1942 that is characterized by an extra X chromosome in human cells.1-3 The patient with KS have two X chromosomes with Y.2,4-8 Approximately one in 1000 boys is born with an additional X chromosome and the most common type of this syndrome is 47,XXY, which represents 82% of these cases and the remaining cases show a variety of aneuploidies like 48,XXXY; 48,XXYY; 49XXXXY and mosaic patterns like 46,XY/47,XXY, 47,XXY/48XXXY.