title: Hereditary gingival fibromatosis: a three-generation case and pathogenic mechanism research on progress of the disease.
abstract: BACKGROUND
  Hereditary gingival fibromatosis (HGF) is a rare benign disorder characterized by progressive overgrowth of gingiva. Although the clinical and histopathologic characteristics of HGF are explicit, the pathogenic mechanism remains unclear. The goal of this article is to describe a three-generation HGF case and discuss the diagnosis, treatment, and inheritance of the disease. The known cellular and molecular features of HGF are also emphasized.
  
  
  METHODS
  Family and medical histories of the patients were recorded, and a series of preliminary examinations, including clinical, histologic, radiographic, and gene examination, were performed to make a diagnosis and learn about the genetic characteristics. An all-quadrant flap surgery was performed to remove excess gingiva, and orthodontic treatment was undertaken to help tooth eruption. Recent advances were reviewed for further knowledge of genetic, cellular, and molecular features of HGF.
  
  
  RESULTS
  The patient's manifestations and examinations showed a typical HGF characteristic. There was no recurrence after surgery, and the premolars and molars erupted to bite plane. Genetic studies have found several gene mutations involved in HGF. Only the son-of-sevenless-1 gene is identified. Multiple molecular factors, such as transforming growth factor-β and matrix metalloproteinases, participate in HGF, regulating the extracellular matrix.
  
  
  CONCLUSIONS
  Surgical intervention is the usual treatment of HGF, but patients still have to deal with the risk of recurrence. Once the correlations between gene mutations, molecular changes, histology, and clinical situation are clear, they can be applied to clinical application, providing novel methods for disease prognosis and diagnosis and targets for disease prevention and treatment.
authors.affiliation: Department of Orthodontics, Affiliated Stomatological Hospital, Zhejiang University School of Medicine, Hangzhou, China.
authors.forename: Jiejun
authors.initials: J
authors.lastname: Shi
authors.forename: Weiwei
authors.initials: W
authors.lastname: Lin
authors.forename: Xiaojun
authors.initials: X
authors.lastname: Li
authors.forename: Feng
authors.initials: F
authors.lastname: Zhang
authors.forename: Xutao
authors.initials: X
authors.lastname: Hong
mesh_terms: D000328:Adult; D000368:Aged; D002648:Child; D003094:Collagen; D003238:Connective Tissue / Q000473:pathology; D018450:Disease Progression; D004848:Epithelium / Q000473:pathology; D005091:Exons / Q000235:genetics; D005260:Female; D005351:Fibromatosis, Gingival / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D005808:Genes, Recessive / Q000235:genetics; D005885:Gingival Hyperplasia / Q000473:pathology; D005890:Gingivectomy; D006801:Humans; D008297:Male; D009971:Orthodontics, Corrective; D010375:Pedigree; D020837:SOS1 Protein / Q000235:genetics; D013524:Surgical Flaps
chemical_list: D020837:SOS1 Protein; D003094:Collagen