title: [Orthodontic and oral surgery therapy in cleidocranial dysplasia].
abstract: 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 germination, 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.
authors: Gyermekfogászati és Fogszabályozási Klinika, Budapest.
authors: Gergely
authors: G
authors: Balaton
authors: Ildikó
authors: I
authors: Tarján
authors: Péter
authors: P
authors: Balaton
authors: Zoltán
authors: Z
authors: Barabási
authors: Szabolcs
authors: S
authors: Gyulai Gál
authors: Katalin
authors: K
authors: Nagy
authors: Zoltán
authors: Z
authors: Vajó
mesh_terms: D000293:Adolescent; D002896:Chromosomes, Human, Pair 6; D002973:Cleidocranial Dysplasia / Q000175:diagnosis / Q000235:genetics / Q000534:rehabilitation* / Q000601:surgery*; D050659:Core Binding Factor Alpha 1 Subunit / Q000235:genetics*; D003833:Denture, Partial, Temporary; D005260:Female; D006801:Humans; D020125:Mutation, Missense; D019647:Oral Surgical Procedures / Q000379:methods*; D009971:Orthodontics, Corrective / Q000379:methods*; D014096:Tooth, Supernumerary / Q000601:surgery; D014097:Tooth, Unerupted / Q000601:surgery
chemical_list: D050659:Core Binding Factor Alpha 1 Subunit