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.
Hunter ML, Collard MM, Razavi T, Hunter B. Increased primary tooth size in a 47,XXY male: A first case report. International journal of paediatric dentistry/the British Paedodontic Society [and] the International Association of Dentistry for Children 2003 Jul;13(4):271-3.
Fruhmesser A, Kotzot D. Chromosomal Variants in Klinefelter Syndrome. Sex Dev Apr 29.
Lahdesmaki R, Alvesalo L. Root lengths in the permanent teeth of Klinefelter (47,XXY) men. Archives of oral biology 2007 Sep;52(9):822-7.
Gorlin RJ, Redman RS, Shapiro BL. Effect of X-Chromosome Aneuploidy on Jaw Growth. Journal of dental research 1965 Jan- Feb;44:SUPPL:269-82.
Celebic A, Brkic H, Kaic Z, Vojvodic D, Poje Z, Singer Z. Occlusal plane orientation in Klinefelter syndrome (47,XXY males). Journal of oral rehabilitation 1997 Dec;24(12):942-6.
Yilmaz YF, Titiz A, Yurur-Kutlay N, Ozcan M, Unal A. Congenital bilateral parotid gland agenesis in Klinefelter syndrome. J Craniomaxillofac Surg Jun;38(4):248-50.
Hata S, Maruyama Y, Fujita Y, Mayanagi manifestations of XXXXY syndrome: A case report. International journal of paediatric dentistry/the British Paedodontic Society [and] the International Association of Dentistry for Children 2001 Mar;11(2):138-42.
Alvesalo L, Tammisalo E, Townsend G. Upper central incisor and canine tooth crown size in 47,XXY males. Journal of dental research 1991 Jul;70(7):1057-60.
Yeh SC, Hsu TY. Endodontic treatment in taurodontism with Klinefelter’s syndrome: A report. Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics 1999 Nov;88(5):612-5.
Varrela J, Alvesalo L. Taurodontism in 47,XXY males: An effect of the extra X chromosome on root development. Journal of dental research 1988 Feb;67(2):501-2.
Marques-da-Silva B, Baratto-Filho F, Abuabara A, Moura P, Losso EM, Moro A. Multiple taurodontism: The challenge of endodontic treatment. Journal of oral science;52(4):653-8.
Official Publication of Cumhuriyet University Faculty of Dentistry
Year 2014,
Volume: 1 Issue: 1, 21 - 25, 05.12.2014
Hunter ML, Collard MM, Razavi T, Hunter B. Increased primary tooth size in a 47,XXY male: A first case report. International journal of paediatric dentistry/the British Paedodontic Society [and] the International Association of Dentistry for Children 2003 Jul;13(4):271-3.
Fruhmesser A, Kotzot D. Chromosomal Variants in Klinefelter Syndrome. Sex Dev Apr 29.
Lahdesmaki R, Alvesalo L. Root lengths in the permanent teeth of Klinefelter (47,XXY) men. Archives of oral biology 2007 Sep;52(9):822-7.
Gorlin RJ, Redman RS, Shapiro BL. Effect of X-Chromosome Aneuploidy on Jaw Growth. Journal of dental research 1965 Jan- Feb;44:SUPPL:269-82.
Celebic A, Brkic H, Kaic Z, Vojvodic D, Poje Z, Singer Z. Occlusal plane orientation in Klinefelter syndrome (47,XXY males). Journal of oral rehabilitation 1997 Dec;24(12):942-6.
Yilmaz YF, Titiz A, Yurur-Kutlay N, Ozcan M, Unal A. Congenital bilateral parotid gland agenesis in Klinefelter syndrome. J Craniomaxillofac Surg Jun;38(4):248-50.
Hata S, Maruyama Y, Fujita Y, Mayanagi manifestations of XXXXY syndrome: A case report. International journal of paediatric dentistry/the British Paedodontic Society [and] the International Association of Dentistry for Children 2001 Mar;11(2):138-42.
Alvesalo L, Tammisalo E, Townsend G. Upper central incisor and canine tooth crown size in 47,XXY males. Journal of dental research 1991 Jul;70(7):1057-60.
Yeh SC, Hsu TY. Endodontic treatment in taurodontism with Klinefelter’s syndrome: A report. Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics 1999 Nov;88(5):612-5.
Varrela J, Alvesalo L. Taurodontism in 47,XXY males: An effect of the extra X chromosome on root development. Journal of dental research 1988 Feb;67(2):501-2.
Marques-da-Silva B, Baratto-Filho F, Abuabara A, Moura P, Losso EM, Moro A. Multiple taurodontism: The challenge of endodontic treatment. Journal of oral science;52(4):653-8.
Cumhuriyet Dental Journal (Cumhuriyet Dent J, CDJ) is the official publication of Cumhuriyet University Faculty of Dentistry. CDJ is an international journal dedicated to the latest advancement of dentistry. The aim of this journal is to provide a platform for scientists and academicians all over the world to promote, share, and discuss various new issues and developments in different areas of dentistry. First issue of the Journal of Cumhuriyet University Faculty of Dentistry was published in 1998. In 2010, journal's name was changed as Cumhuriyet Dental Journal. Journal’s publication language is English.
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