Fogorvosi szemle, 2007 (100. évfolyam, 1-6. szám)
2007-02-01 / 1. szám
FOGORVOSI SZEMLE 100. évf. 1. sz. 2007. 21 3. Elischer Z, Botyik M: A dysostosis cleidocranialis kombinált ortodonciai, sebészi és protetikai kezelése. Esetismertetés. Fogorv Szle 1980; 73: 161-164. 4. Fukuta Y, Totsuka M, Fukuta Y, Takeda Y, Yoshida Y, Niitsu J. és Mtsai: Histological and analytical studies of a tooth in patient with cleidocranial dysostosis. J Oral Sei 2001 ; 43: 85-89. 5. Gáspár E, Kovács K, Orosz M, Kenéz J, Csiba Á: Dysostosis cleidocranialis. Orvosképzés 1982; 57: 269-304. 6. Golan I, Baumert U, Wagener H, Preising M, Lorenz B, Niederellmann H. és mtsai: Evidence of intrafamilial variability of CBFA1/RUNX2 expression in cleidocranial dysplasia- a famili study. J Orofac Orthop 2002; 63: 190-198. 7. Golan I, Baumert U, Wagener H, Dauwerse J, Preising M, Lorenz B. és mtsai: Atypical expression of cleidocranial dysplasia: clinical and molecular-genetic analysis. Orthod Craniofac Res 2002; 5: 243- 249. 8. Golan I, Baumert U, Hrala Bp, Mussig D: Dentomaxillofacial variability of cleidocranial dysplasia: clinicoradiological presentation of systemic review. Dentomaxillofac radio12003; 32: 347-354. 9. Golan I, Baumert U, Hrala Bp, Schaumburger J, Wiech O, Grifka J. és mtsai: Symptômes and signs in cleidocranial dysplasia (CCD). Z Orthop Ihre Grenzgeb 2003; 141: 336-340. 10. Golan I, Baumert U, Hrala Bp, Mussig D: Early craniofacial signs of cleidocranial dysplasia, tnt J Peadatr Dent 2004; 14: 49-53. 11. Jensen Bl, Kreiborg S: Development of the dentition in cleidocranial dysplasia. J Oral Pathol Med 1990; 19: 89-93. 12. Marie P, Sainton P: Sur la dysostose cléido-cranienne héréditaire. Rev Neurol 1898; 6: 835-838. 13. Mitchell L, Mitchell D: Oxford handbook of clinical dentistry. Oxford University Press, 1992. 709. 14. Rimoin Dl: International nomenclature of constitutional diseases of bone. J Pediatr 1978; 93: 614-618. 15. Sakai N, Hasegawa H, Yamazaki Y, Ui K, Tokunaga K, Hírőse R. és mtsai: A case of a Japanase patient with cleidocranial dysplasia possesing a mutation of CBFA1 gene. J Craniofac Surg 2002; 13: 31-34. 16. Scheuthauer G: Kombination rudimentärer Schlüsselbeine mit Anomalien des Schädels beim erwachsenen Menschen. Alig Wien Med Ztg 1871 ; 16: 293-295. 17. Shaikh R, Shusterman S: Delayed dental maturation in cleidocranial dysplasia. J Dent Child 1998; 65: 325-329. 18. Soule, Ab: Mutational Dysostosis. Bone joint Surg 1946; 28: SI- 102. 19. Suba Zs: A szájüreg klinikai pathológiája. Medicina, Budapest, 1999. 26. 20. Zhou G, Chen Y, Zhou L, Thirunavukkarasu K, Hecht J, Chitayat D és mtsai: CBFA1 mutation analysis and functional correlation with phenotypic variability in cleidocranial dysplasia. Hum Mol Genet 1999; 8: 2311-2316. Dr. Balaton G, Dr. Tarján I, Dr. Balaton P, Dr. Barabási Z, Dr. GYulai Gál Sz, dr. Nagy K, Dr.Vajó Z Orthodontic and oral surgery therapy in cleidocranial dysplasia A cleidocranial dysplasia is an autosomal dominant inherited condition consisting of generalized skeletal disorder. Associated dental signs are present in 93,5%; failure of tooth eruption with multiple supernumerary teeth, dilaceration of roots, crown gemination, microdontia, high arched palate, midface hypoplasia, high gonion angle. The molecular- genetic analysis revealed a missense mutation in the CBFA1 gene located on chromosome 6p21, which is considered to be etiological factor for CCD. Orthodontic and oral surgery therapy of a 13 year-old child with CCD was performed due to aesthetic and functional problems. The supernumerary germs were removed and the teeth were aligned with orthodontic appliances. Temporary functional rehabilitation was solved with partial denture. The presented case and the literature data support the importance of early diagnosis of CCD. The good collaboration of the orthodontic and maxillo-facial surgery specialists help achieve the correct rehabilitation of the patient. Key words: Cleidocranial dysplasia, oral surgery and orthodontic treatment