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      Multispeciality Approach in the Management of Patient with Hereditary Gingival Fibromatosis: 1-Year Followup: A Case Report

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      International Journal of Dentistry
      Hindawi Publishing Corporation

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          Abstract

          Background. Hereditary gingival fibromatosis is a fibrotic enlargement of the gingiva. It may exist as an isolated abnormality or as part of multisystem syndrome. This paper reports a case of 16-year-old male with generalized severe gingival overgrowth, involving the maxillary and mandibular arches and covering almost all teeth. Methods. Periodontal management of gingival enlargement included gingivectomy in both arches except in the lower right molar region where flap surgery was done under general anesthesia. After a 2-month followup period, orthodontic treatment was started with fixed appliances. Monthly periodontal checkups and maintainance (scaling and polishing) were scheduled to control the gingival inflammation. Results. Reevaluation of the patient of surgical treatment after two months did not show any recurrence of condition; however, minimal overgrowth was noted 1 month after the beginning of orthodontic treatment which was treated nonsurgically. Conclusions. Although the risk of recurrence is high with this condition, surgical treatment with correction of malocclusion and regular followup can provide excellent outcome as seen in this case.

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          Most cited references21

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          A mutation in the SOS1 gene causes hereditary gingival fibromatosis type 1.

          Hereditary gingival fibromatosis (HGF) is a rare, autosomal dominant form of gingival overgrowth. Affected individuals have a benign, slowly progressive, nonhemorrhagic, fibrous enlargement of the oral masticatory mucosa. Genetic loci for autosomal dominant forms of HGF have been localized to chromosome 2p21-p22 (HGF1) and chromosome 5q13-q22 (HGF2). To identify the gene responsible for HGF1, we extended genetic linkage studies to refine the chromosome 2p21-p22 candidate interval to approximately 2.3 Mb. Development of an integrated physical and genetic map of the interval identified 16 genes. Sequencing of these genes, in affected and unaffected HGF1 family members, identified a mutation in the Son of sevenless-1 (SOS1) gene in affected individuals. In this report, we describe the genomic structure of the SOS1 gene and present evidence that insertion of a cytosine between nucleotides 126,142 and 126,143 in codon 1083 of the SOS1 gene is responsible for HGF1. This insertion mutation, which segregates in a dominant manner over four generations, introduces a frameshift and creates a premature stop codon, abolishing four functionally important proline-rich SH3 binding domains normally present in the carboxyl-terminal region of the SOS1 protein. The resultant protein chimera contains the wild-type SOS1 protein for the N-terminal amino acids 1-1083 fused to a novel 22-amino acid carboxyl terminus. Similar SOS1 deletion constructs are functional in animal models, and a transgenic mouse construct with a comparable SOS1 chimera produces a phenotype with skin hypertrophy. Clarification of the functional role of this SOS1 mutant has implications for understanding other forms of gingival fibromatosis and corrective gingival-tissue management.
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            Hereditary gingival fibromatosis: a systematic review.

            Generalized gingival enlargement can be caused by a variety of etiological factors. It can be inherited (hereditary gingival fibromatosis [HGF]); associated with other diseases characterizing a syndrome; or induced as a side effect of systemic drugs, such as phenytoin, cyclosporin, or nifedipine. HGF, previously known as elephantiasis gingivae, hereditary gingival hyperplasia, and hypertrophic gingiva, is a genetic disorder characterized by a progressive enlargement of the gingiva. This review will focus on diagnosis, treatment, and control of HGF. The pattern of inheritance, the histopathologic characteristics, and the known biologic and genetic features associated with HGF are also emphasized.
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              Hereditary gingival fibromatosis: identification, treatment, control.

              Hereditary gingival fibromatosis, or HGF, is characterized by varying degrees of attached gingival hyperplasia. The authors describe a case of generalized severe hereditary gingival fibromatosis involving the maxillary and mandibular arches. Removal of excess gingival tissue by conventional gingivectomy dramatically improved the patient's appearance.
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                Author and article information

                Journal
                Int J Dent
                IJD
                International Journal of Dentistry
                Hindawi Publishing Corporation
                1687-8728
                1687-8736
                2010
                23 December 2010
                : 2010
                : 575979
                Affiliations
                Department of Periodontology and Implantology, Meenakshi Ammal Dental College and Hospital, Maduravoyal, Chennai 95, India
                Author notes

                Academic Editor: Dimitris N. Tatakis

                Article
                10.1155/2010/575979
                3014701
                21234408
                993d2ed9-a4e2-4c6d-9d78-ff7fedf54f2a
                Copyright © 2010 T. Ramakrishnan and M. Kaur.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 15 August 2010
                : 25 September 2010
                : 24 November 2010
                Categories
                Case Report

                Dentistry
                Dentistry

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