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Hereditary gingival fibromatosis : ウィキペディア英語版 | Hereditary gingival fibromatosis Hereditary gingival fibromatosis (HGF), also known as idiopathic gingival hyperplasia, is a rare condition of gingival overgrowth.〔Poulami Majumder, Vineet Nair, Malancha Mukherjee, Sujoy Ghosh, and Subrata Kumar Dey, “The Autosomal Recessive Inheritance of Hereditary Gingival Fibromatosis,” Case Reports in Dentistry, vol. 2013, Article ID 432864, 4 pages, 2013. doi:10.1155/2013/432864〕 HGF is characterized as a benign, slowly progressive, nonhemorrhagic, fibrous enlargement of keratinized gingiva. It can cover teeth in various degrees, and can lead to aesthetic disfigurement.〔Thomas C. Hart, Yingze Zhang, Michael C. Gorry, P. Suzanne Hart, Margaret Cooper, Mary L. Marazita, Jared M. Marks, Jose R. Cortelli, Debora Pallos Am J Hum Genet. 2002 April; 70(4): 943–954. Published online 2002 February 26.〕 Fibrous enlargement is most common in areas of maxillary and mandibular tissues of both arches in the mouth.〔 Phenotype and genotype frequency of HGF is 1:175,000 where males and females are equally affected but the cause is not entirely known.〔〔K. B. Butchi, K. Pavankumar, B. R. Anuradha, and N. Arora, “Hereditary gingival fibromatosis—a case report and management using a novel surgical technique,” Revista Sul-Brasileira de Odontologia, vol. 8, no. 4, pp. 453–458, 2011.〕 It mainly exists as an isolated abnormality but can also be associated with a multi-system syndrome.〔 ==Cause== Though much more research needs to be done, researchers have mostly agreed that a mutation in SOS1, son-of-sevenless gene, is responsible for this disease.〔〔S. DeAngelo, J. Murphy, L. Claman, J. Kalmar, and B. Leblebicioglu, “Hereditary gingival fibromatosis—a review,” Compendium of Continuing Education in Dentistry, vol. 28, no. 3, pp. 138–143, 2007. View at Scopus〕〔R. J. Jorgenson and M. E. Cocker, “Variation in the inheritance and expression of gingival fibromatosis,” Journal of Periodontology, vol. 45, no. 7, pp. 472–477, 1974. View at Scopus〕〔A. Poulopoulos, D. Kittas, and A. Sarigelou, “Current concepts on gingival fibromatosis-related syndromes,” Journal of Investigative and Clinical Dentistry, vol. 2, no. 3, pp. 156–161, 2011. View at Publisher • View at Google Scholar〕 SOS1 is a guanine nucleotide-exchange factor that functions in the transduction of signals that control cell growth and differentiation. A mutation in the SOS1 gene results in a single nucleotide insertion.〔 Specific linkage studies have localized the mutation for isolated, nonsyndromic autosomal dominant forms of gingival fibromatosis to chromosomes 2 and 5, more specifically 2p21-p22 and 5q13-q22.〔
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