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      Supernumerary Teeth: Review of the Literature with Recent Updates

      1 , 2
      Conference Papers in Science
      Hindawi Limited

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          Abstract

          A supernumerary tooth (ST) is defined as any tooth or odontogenic structure that is formed from tooth germ in excess of usual number for any given region of the dental arch. They may be single or multiple and unilateral or bilateral in distribution and can occur in any region of the dental arch. These may occur in primary and permanent dentition. Supernumerary teeth are more frequent in males. They are classified based on form, morphology, location, and occurrence. Several hypotheses have been proposed to explain the occurrence of ST. However, combination of environmental and genetic factors has been proposed. Supernumerary teeth cause a range of complications like crowding, displacement, dilacerations, cyst formation, and so forth. Early identification and appropriate treatment plan should minimize the potential complications caused by ST.

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          Supernumerary teeth: review of the literature and a survey of 152 cases.

          A review of the literature relating to supernumerary teeth is presented along with a survey of 152 cases. The study population consisted of 152 children who visited the department of Paediatric Dentistry at the Jordan University Hospital. Patients ranged in age from 5 to 15 years. Supernumeraries were detected by clinical examination and radiographs. Males were affected more than females with a sex ratio of 2.2 : 1. Seventy-seven percent of the patients had one supernumerary tooth, 18.4% had double teeth, and 4.6% had three or more supernumeraries. Ninety percent of the supernumerary teeth occurred in the premaxilla, of which 92.8% were in the central incisor region and of these latter 25% were located in the midline. The other 10.4% of the supernumeraries were located in the premolar, canine, molar, and lower central incisor regions. Two cases were of non-syndrome supernumerary teeth. Seventy-five percent of the supernumeraries were conical, 83.1% were in the normal vertical position and 26.5% were erupted. Conical-shaped supernumerary teeth had a significantly higher rate of eruption compared to the tuberculate type.
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            Missense mutations abolishing DNA binding of the osteoblast-specific transcription factor OSF2/CBFA1 in cleidocranial dysplasia.

            Cleidocranial dysplasia (CCD) is an autosomal dominant disorder characterized by hypoplastic or absent clavicles, large fontanelles, dental anomalies and delayed skeletal development. The phenotype is suggestive of a generalized defect in ossification and is one of the most common skeletal dysplasias not associated with disproportionate stature. To date, no genetic determinants of ossification have been identified. CCD has been mapped to chromosome 6p21, where CBFA1, a gene encoding OSF2/CBFA1, a transcriptional activator of osteoblast differentiation, has been localized. Here, we describe two de novo missense mutations, Met175Arg and Ser191Asn, in the OSF2/CBFA1 gene in two patients with CCD. These two mutations result in substitution of highly conserved amino acids in the DNA-binding domain. DNA-binding studies with the mutant polypeptides show that these amino acid substitutions abolish the DNA-binding ability of OSF2/CBFA1 to its known target sequence. Concurrent studies show that heterozygous nonsense mutations in OSF2/CBFA1 also result in CCD, while mice homozygous for the osf2/cbfa1 mull allele exhibit a more severe lethal phenotype. Thus, these results together suggest that CCD is produced by haploinsufficiency of OSF2/CBFA1 and provide direct genetic evidence that the phenotype is secondary to an alteration of osteoblast differentiation.
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              Mutations in a new gene, encoding a zinc-finger protein, cause tricho-rhino-phalangeal syndrome type I.

              Tricho-rhino-phalangeal syndrome type I (TRPS I, MIM 190350) is a malformation syndrome characterized by craniofacial and skeletal abnormalities and is inherited in an autosomal dominant manner. TRPS I patients have sparse scalp hair, a bulbous tip of the nose, a long flat philtrum, a thin upper vermilion border and protruding ears. Skeletal abnormalities include cone-shaped epiphyses at the phalanges, hip malformations and short stature. We assigned TRPS1 to human chromosome 8q24. It maps proximal of EXT1, which is affected in a subgroup of patients with multiple cartilaginous exostoses and deleted in all patients with TRPS type II (TRPS II, or Langer-Giedion syndrome, MIM 150230; ref.2-5). We have positionally cloned a gene that spans the chromosomal breakpoint of two patients with TRPS I and is deleted in five patients with TRPS I and an interstitial deletion. Northern-blot analyses revealed transcripts of 7 and 10.5 kb. TRPS1has seven exons and an ORF of 3,843 bp. The predicted protein sequence has two potential nuclear localization signals and an unusual combination of different zinc-finger motifs, including IKAROS-like and GATA-binding sequences. We identified six different nonsense mutations in ten unrelated patients. Our findings suggest that haploinsufficiency for this putative transcription factor causes TRPS I.
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                Author and article information

                Journal
                Conference Papers in Science
                Conference Papers in Science
                Hindawi Limited
                2356-6108
                2356-6094
                2014
                2014
                : 2014
                : 1-6
                Affiliations
                [1 ]Department of Pedodontics and Preventive Dentistry, SDDCH, Parbhani, India
                [2 ]Department of Dentistry, Abhiram Institute of Medical Sciences, Atmakur, Nellore 524322, India
                Article
                10.1155/2014/764050
                5d3fd27e-54d0-4c0e-9433-da074c58bf8a
                © 2014

                http://creativecommons.org/licenses/by/3.0/

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