Hereditary gingival fibromatosis (HGF), also known as idiopathic gingival hyperplasia, is a rare condition of gingival overgrowth. HGF is characterized as a . Hereditary, drug-induced, and idiopathic gingival overgrowth have been reported . Hereditary gingival fibromatosis can occur as an isolated. Mutation in SOS1, son-of-sevenless gene, is thought to be responsible for hereditary gingival fibromatosis. This report shows a case of.

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Hereditary gingival fibromatosis HGFalso known as idiopathic gingival hyperplasiais a rare condition of fibromatisis overgrowth. It can cover teeth fibronatosis various degrees, and can lead to aesthetic disfigurement. There may or may not be any evidence of history of HGF in the family nor any usage of taking long-term medicines for any particular disease when it comes to diagnosing HGF.

Although, enlargement of gingiva, interdental papillahindered speech, and secondary inflammatory changes taking place in the mouth commonly at the marginal gingiva are all very indicative of this condition.

Commonly the patient will have mandiblular and maxilliary inflammation and overgrowth as opposed to the traditional pink, firm, and fleshy consistency of healthy gingiva. The patient’s jaw may fibromqtosis appear distorted because of the gingiva englargements.

Overgrowth of the gingiva can range from slightly covering the surface of teeth or it can even completely cover the surrounding teeth. The patient can also experience damage ginfival loss of teeth. Though much more research needs herwditary be done, researchers have mostly agreed that a mutation in SOS1son-of-sevenless gene, is responsible for this disease. A mutation in the SOS1 gene results in a single nucleotide insertion.

HGF may also be caused by unwanted side effects of pharmacological agents like phenytoinciclosporinand some calcium-channel blockers, meaning HGF is a disease that can be drug-induced.

Genetic linkage studies are among the most popular methods of study to look at the mechanism of this HGF. Genetic linkage studies have found to localize genetic loci for autosomal dominant forms of HGF to chromosome 2pp22 indicative of HGF1 and chromosome 5qq22 indicative of HGF2.

Here, a mutation is found in sequencing these 16 genes. There is an insertion of a cytosine between nucleotidesandin codon of the SOS1 gene, meaning there is a mutation in SOS1. This causes a problem because SOS1 introduces a frameshift mutation and creates a premature stop codon.

Also, it can segregate over generations, most commonly four. Once it causes a premature stop codon, the chromosome loses four important proline -rich SH-3 binding domains in the hereditxry region of the SOS1 protein. As a result, the N-terminal amino acids for SOS1 is fused into a 22—amino acid carboxyl terminus. There are very few ways to test a patient for Dibromatosis. Currently, the most common way to diagnose a patient is by means of a physical evaluation. The physician can make a physical evaluation of the patient and send them to a dentist or better yet a specialist like a periodontist to evaluate signs of gingival overgrowth, quality of gingiva, inflammation, mechanical difficulties of the mouth, tooth conditions, and any sort of discomfort.

Aside from obvious physical symptoms seen in a physical evaluation, molecular tests can be run to check if there is a mutation in the SOS1 gene gingoval confirm the diagnosis.

If there is indeed a mutation in this gene coupled with the typical physical symptoms, then it is quite probable that a patient suffers from this disease. Also, looking at family history is also becoming more prominent in aiding to diagnose the patient. Otherwise, researchers are working to find new and better ways to test for the presence of HGF. Since this condition is generally agreed upon to be hereditarynothing can be done to firomatosis HGF.

However, in some cases where it can develop as a result of rare multi-system syndromes, such as: 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.

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This disease has not been shown to be life-threatening or the cause of death in patients. However, treatment is necessary to maintain a healthy lifestyle. Most recent methods of treatment take the form of surgeries such as oral prophylaxisfollowed by post-surgical therapies to monitor, provide proper oral hygiene, and correct the deformity.

Although, the nature of recurrence post-treatment is virtually unknown, let alone what type of treatment is most effective for HGF. SOURCE 2 In some cases, there is re-growth after surgical removal of the excess gingival tissues, in others there is minimal.

No cases yet have shown any particular treatment or form of medicine to permanently remove HGF. One type of procedure that can be executed is as follows: Removal of excess tissue under anesthesia through an internal bevel gingivectomy or undisplaced flap followed by gingivoplasty and continuous sling suture placements and periodontal dressing; after about a week of recovery after the surgery, remove sutures and periodically do observational evaluations to look for any signs of re-occurrence.

Some researchers suggest that HGF is transmitted as a Mendelian trait since both autosomal dominant and autosomal recessive transmission has been reported since the early s. SOURCE 1 In more recent scientific literature, there is evidence in which pedigree analyses confirm autosomal dominant, autosomal recessive or even as X-linked inherited cases of the HGF trait.

Inresearchers described the SOS1 gene and proved for the first time that a single-nucleotide—insertion mutation of the SOS1 gene on codon is the preliminary cause of HGF1 in humans.

Source 1 Later on inthere was a case study done on a year-old male with severe gingival overgrowth, almost covering all teeth. Researchers approached this issue with periodontics – a partial gingivectomy and flap surgery.

This case study concluded that surgery followed by regular follow-ups is a good way to treat HGF despite the fact that the risks of re-occurrence of the condition remain high. Even more recently, a study was done in on a family that showed history of autosomal recessive inheritance of HGF.

The study did not dismiss the return of HGF after treatment but did claim that general surgical intervention after scaling and root planning of teeth supplemented with good oral hygiene is good enough to prevent the re-occurrence of HGF. This case study also acknowledged how HGF can be part of a multi-system syndrome associated with disorders such as Zimmermann Laband syndrome ear, nose, bone, and nail defects with hepatosplenomegalyRutherford syndrome microphthalmia, mental retardation, athetosis, and hypopigmentationMurray-Puretic Drescher syndrome and Ramon syndrome.

From Wikipedia, the free encyclopedia. Hereditary gingival fibromatosis Synonyms Autosomal dominant gingival hyperplasia Classification and external resources Specialty oral and maxillofacial surgery ICD – 10 K Hart, Yingze Zhang, Michael C.

Published online February J Clin Periodontol Am J Hum Genet 1: Dentistry involving supporting structures of teeth Periodontology. Chronic periodontitis Localized aggressive periodontitis Generalized aggressive periodontitis Periodontitis as a manifestation of systemic disease Periodontosis Necrotizing periodontal diseases Abscesses of the periodontium Combined periodontic-endodontic lesions.

Debridement Scaling and root planing Full mouth disinfection Full mouth ultrasonic debridement. Apically positioned flap Bone graft Coronally positioned flap Crown lengthening Open flap debridement Gingival graft Gingivectomy Guided bone regeneration Guided tissue regeneration Enamel matrix derivative Implant placement Lateral pedicle graft Pocket reduction surgery Socket preservation Sinus lift Subepithelial connective tissue graft Tools Curette Membrane Probe Scaler.

Oral and maxillofacial pathology K00—K06, K11—K14—, — Bednar’s aphthae Cleft palate High-arched palate Palatal cysts of the newborn Inflammatory papillary hyperplasia Stomatitis nicotina Torus palatinus. Oral mucosa — Lining of mouth. Squamous cell papilloma Keratoacanthoma Malignant: Adenosquamous carcinoma Basaloid squamous carcinoma Mucosal melanoma Spindle cell carcinoma Squamous cell carcinoma Verrucous carcinoma Oral florid papillomatosis Oral melanosis Smoker’s melanosis Pemphigoid Benign mucous membrane Pemphigus Plasmoacanthoma Stomatitis Aphthous Denture-related Herpetic Smokeless tobacco keratosis Submucous fibrosis Ulceration Riga—Fede disease Verruca vulgaris Verruciform xanthoma White sponge nevus.

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Hereditary gingival fibromatosis: Characteristics and treatment approach

Teeth pulpdentinenamel. Periodontium gingivaperiodontal ligamentcementumalveolus — Gums and tooth-supporting structures. Cementicle Cementoblastoma Gigantiform Cementoma Eruption cyst Epulis Pyogenic granuloma Congenital epulis Gingival enlargement Gingival cyst of the adult Gingival cyst of the newborn Gingivitis Desquamative Granulomatous Plasma cell Hereditary gingival fibromatosis Hypercementosis Hypocementosis Linear gingival erythema Necrotizing periodontal diseases Acute necrotizing ulcerative gingivitis Pericoronitis Peri-implantitis Periodontal abscess Periodontal trauma Periodontitis Aggressive As a manifestation of systemic disease Chronic Perio-endo lesion Teething.

Periapical, mandibular and maxillary hard tissues — Bones of jaws. Nasopalatine duct Median mandibular Median palatal Traumatic bone Osteoma Osteomyelitis Osteonecrosis Bisphosphonate-associated Neuralgia-inducing cavitational osteonecrosis Osteoradionecrosis Osteoporotic bone marrow defect Paget’s disease of bone Periapical abscess Phoenix abscess Periapical periodontitis Stafne defect Torus mandibularis. Temporomandibular jointsmuscles of mastication and malocclusions — Jaw joints, chewing muscles and bite abnormalities.

Benign lymphoepithelial lesion Ectopic salivary gland tissue Frey’s syndrome HIV salivary gland disease Necrotizing sialometaplasia Mucocele Ranula Pneumoparotitis Salivary duct stricture Salivary gland aplasia Salivary gland atresia Salivary gland diverticulum Salivary gland fistula Salivary gland hyperplasia Salivary gland hypoplasia Salivary gland neoplasms Benign: Basal cell adenoma Canalicular adenoma Ductal papilloma Monomorphic adenoma Myoepithelioma Oncocytoma Papillary cystadenoma lymphomatosum Pleomorphic adenoma Sebaceous adenoma Malignant: Orofacial soft tissues — Soft tissues around the mouth.

Eagle syndrome Hemifacial hypertrophy Facial hemiatrophy Oral manifestations of systemic disease. Lysosome granules biogenic amines Histamine Serotonin. Macrophage Epithelioid cell Giant cell Granuloma. Rubor Calor Tumor Dolor Functio laesa. Hepatitis Ascending cholangitis Cholecystitis Pancreatitis Peritonitis. Dermatitis Folliculitis Cellulitis Hidradenitis.

Insulitis Hypophysitis Thyroiditis Parathyroiditis Adrenalitis. Retrieved from ” https: Infobox medical condition Articles contradicting other articles. Views Read Edit View history. This page was last edited on 20 Decemberat By using this site, you agree to the Terms of Use and Privacy Policy.

Hereditary gingival fibromatosis: Characteristics and treatment approach

Diagnoses Chronic periodontitis Localized aggressive periodontitis Generalized aggressive periodontitis Periodontitis as a manifestation of systemic disease Periodontosis Necrotizing periodontal diseases Abscesses of the periodontium Combined periodontic-endodontic lesions. Conventional therapy Debridement Scaling and root planing Full mouth disinfection Full mouth ultrasonic debridement.

Palate Bednar’s aphthae Cleft palate High-arched palate Palatal cysts of the newborn Inflammatory papillary hyperplasia Stomatitis nicotina Torus palatinus. Periodontium gingivaperiodontal ligamentcementumalveolus — Gums and tooth-supporting structures Cementicle Cementoblastoma Gigantiform Cementoma Eruption cyst Epulis Pyogenic granuloma Congenital epulis Gingival enlargement Gingival cyst of the adult Gingival cyst of the newborn Gingivitis Desquamative Granulomatous Plasma cell Hereditary gingival fibromatosis Hypercementosis Hypocementosis Linear gingival erythema Necrotizing periodontal diseases Acute necrotizing ulcerative gingivitis Pericoronitis Peri-implantitis Periodontal abscess Periodontal trauma Periodontitis Aggressive As a manifestation of systemic disease Chronic Perio-endo lesion Teething.

Periapical, mandibular and maxillary hard tissues — Bones of jaws Agnathia Alveolar osteitis Buccal exostosis Cherubism Idiopathic osteosclerosis Mandibular fracture Microgenia Micrognathia Intraosseous cysts Odontogenic: Temporomandibular jointsmuscles of mastication and malocclusions — Jaw joints, chewing muscles and bite abnormalities Bruxism Condylar resorption Mandibular dislocation Malocclusion Crossbite Open bite Overbite Overeruption Overjet Prognathia Retrognathia Scissor bite Maxillary hypoplasia Temporomandibular joint dysfunction.

Salivary glands Benign lymphoepithelial lesion Ectopic salivary gland tissue Frey’s syndrome HIV salivary gland disease Necrotizing sialometaplasia Mucocele Ranula Pneumoparotitis Salivary duct stricture Salivary gland aplasia Salivary gland atresia Salivary gland diverticulum Salivary gland fistula Salivary gland hyperplasia Salivary gland hypoplasia Salivary gland neoplasms Benign: Orofacial soft tissues — Soft tissues around the mouth Actinomycosis Angioedema Basal cell carcinoma Cutaneous sinus of dental origin Cystic hygroma Gnathophyma Ludwig’s angina Macrostomia Melkersson—Rosenthal syndrome Microstomia Noma Oral Crohn’s disease Orofacial granulomatosis Perioral dermatitis Pyostomatitis vegetans.

Other Eagle syndrome Hemifacial hypertrophy Facial hemiatrophy Oral manifestations of systemic disease.