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Hereditary Gingival Fibromatosis

Autosomal Dominant Gingival Fibromatosis


Presentation

  • HGF can present as an isolated feature or as part of a syndrome. Recent findings report a defect in the Son of sevenless-1 gene on chromosome 2p21-p22 (HGF1) as a possible cause of this clinical presentation.[ncbi.nlm.nih.gov]
  • It presents as a localized or generalized, smooth or nodular overgrowth of the gingival tissues of varying severity. It can be isolated, with autosomal dominant inheritance, or as part of a syndrome.[orpha.net]
Family History of Mental Retardation
  • There also may or may not be any signs of medical and/or family history of mental retardation, hypertrichosis, nor clinical symptoms that can be associated with gingival enlargement.[en.wikipedia.org]
Chest Deformity
  • A case of a 9-year-old child with hereditary gingival fibromatosis, supernumerary tooth, chest deformities, auricular cartilage deformation, joint laxity and undescended testes is described.[ncbi.nlm.nih.gov]
Social Isolation
  • All complained similarly of weight loss, difficulty with articulation, and social isolation. Physical examination revealed massive overgrowth of both maxillary and mandibular gingiva, as well as multiple ectopic teeth.[ncbi.nlm.nih.gov]
Psychomotor Retardation
  • Gingival fibromatosis combined with cherubism and psychomotor retardation: A rare syndrome. J Periodontol 1999;70:201-4. 17. Anderson J, Cunliffe WJ, Roberts DF, Close H. Hereditary gingival fibromatosis. Br Med J 1969;3:218-9. [ PUBMED ] 18.[njms.in]
Speech Disorders
  • disorders Other dental and oral problems In some cases, Hereditary Gingival Fibromatosis may cause bleeding from the gums, or gum ulcerations.[en.wikipedia.org]
  • disorders [1] Other dental and oral problems [4] Mechanism Genetic linkage studies are among the most popular methods of study to look at the mechanism of this HGF.[ipfs.io]

Workup

Delayed Bone Age
  • In addition to HGF, both patients had short stature, subnormal growth velocity, and delayed bone age but no abnormalities on magnetic resonance imaging of the brain. Serum prolactin levels and results of thyroid function tests were normal.[ncbi.nlm.nih.gov]
Lymphocytic Infiltrate
  • Underlying connective tissue showed dense fibro-collagenous tissue and mild lymphocytic infiltration. Hence, the case was diagnosed as gingival hyperplasia [Figure 4].[jicdro.org]

Treatment

Prognosis

  • (Outcomes/Resolutions) The prognosis of Hereditary Gingival Fibromatosis is very good with suitable treatment A complete excision usually results in low tumor recurrence However, the prognosis of this condition also depends on the severity of any underlying[dovemed.com]
  • 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[ncbi.nlm.nih.gov]
  • Significantly revised and updated content throughout this edition brings you the latest information on the etiology, clinical features, histopathology, treatment, and prognosis of each disease entity, as well as cutting-edge topics such as bisphosphonate[books.google.com]
  • If the patient's disease is treated by means of surgery, it is recommended that the patient undergoes post-surgical therapies for maintenance and periodic monitoring of gums for the sake of the possibility of re-occurrence of HGF. [8] Treatment and prognosis[ipfs.io]

Etiology

  • Abstract Generalized gingival enlargement can be caused by a variety of etiological factors.[ncbi.nlm.nih.gov]
  • AU - Livada, Rania AU - Shiloah, Jacob PY - 2012/1/1 Y1 - 2012/1/1 N2 - Gingival enlargement is common among patients and can be caused by a variety of etiological factors.[uthsc.pure.elsevier.com]

Epidemiology

  • Chapters on epidemiology, embryology, non-syndromic hearing loss, and syndromic forms of hearing loss have all been updated with particular attention to the vast amount of new information on molecular mechanisms, and chapters on clinical and molecular[books.google.com]
  • Chronic periodontitis Epidemiology of periodontal diseases Gingivitis Gum graft Periodontist Tooth loss Gingival recession American Academy of Periodontology Home Page Periodontal Disease as a Specific, albeit Chronic, Infection: Diagnosis and Treatment[en.wikipedia.org]
  • […] syndrome (ear, nose, bone, and nail defects with hepatosplenomegaly), Rutherford syndrome, microphthalmia, mental retardation, athetosis, and hypopigmentation), Murray-Puretic Drescher syndrome, and Ramon syndrome. [1] See also Chronic periodontitis Epidemiology[ipfs.io]
  • Gingival overgrowth in children: Epidemiology, pathogenesis and complications. A literature review. J Periodontol. 2005; 76 :3–10. [ PubMed ] [ Google Scholar ] 5. Lobao DS, Silva LC, Soares RV, Cruz RA.[ncbi.nlm.nih.gov]
  • Gingival overgrowth in children: Epidemiology, pathogenesis, and complications. A literature review. J Periodontol 2005;76:3-10. 31. Farrer-Brown G, Lucas RB, Winstock D. Familial gingival fibromatosis: An unusual pathology.[njms.in]
Sex distribution
Age distribution

Pathophysiology

  • Pathogenesis and Pathophysiology The pathologic manifestation of GF is excessive accumulation of ECM proteins, including collagen type I [ 110 – 114 ].[ojrd.biomedcentral.com]

Prevention

  • Another class of medication associated with gingival enlargement is immunosuppressive agents given to organ-transplant patients to prevent rejection of the new element such as cyclosporine.[ncbi.nlm.nih.gov]
  • Since this condition is generally agreed upon to be hereditary, nothing can be done to prevent HGF.[en.wikipedia.org]

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