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Gingival Fibromatosis-Progressive Deafness Syndrome

Jones Syndrome


Presentation

  • Our patient presented with the complaint of gingival enlargement associated with progressive deafness, characteristic of Jones syndrome.[ncbi.nlm.nih.gov]
  • The present study describes a case report of father and son aged 42 years and 13 years respectively, presenting features of Zimmermann-Laband syndrome.[jdrntruhs.org]
  • Here, we present an unusual clinical presentation of nonsyndromic idiopathic gingival fibromatosis with unknown etiology in a 20-year-old female.[mdcan-uath.org]
  • Present case report highlights the unusual coexistence of nonsyndromic idiopathic gingival fibromatosis.[ijcdc.com]
  • We present a case of idiopathic gingival fibromatosis.[ijri.org]
Developmental Delay
  • A variant of this, namely sensorineural hearing deficit and premature tooth loss has also been reported as a part of Coffin-Lowry syndrome (mental retardation, developmental delay and characteristic facial changes).[ 5 ] This case report is similar in[ncbi.nlm.nih.gov]
  • delay-lung cysts-overgrowth-Wilms tumor syndrome Global developmental delay-neuro-ophthalmological abnormalities-seizures-intellectual disability syndrome Global developmental delay-osteopenia-ectodermal defect syndrome Global developmental delay-visual[orpha.net]
  • delay Small nail Coarse facial features Mandibular prognathia Oxycephaly Abnormality of cardiovascular system morphology Hamartoma Frontal bossing Downslanted palpebral fissures Nephroblastoma Strabismus Hypertelorism Multiple impacted teeth Plantar[mendelian.co]
  • Developmental delay is usually profound with most patients remaining non-ambulatory and non-verbal throughout their lives. Seizures can develop at any age and tend to become increasingly difficult to control.[atlasgeneticsoncology.org]
Dentist
  • As such, it should be useful to a varied audience, including not only the oral and maxillofacial surgeon but also the oral pathologist, the practitioner of oral medicine, and members of the craniofacial team--the orthodontist, pediatric dentist, speech[books.google.com]
  • This atypical presentation should make dentists, physicians and periodontists aware that isolated cases of gingival fibromatosis with hearing loss do occur without a familial history.[ncbi.nlm.nih.gov]
  • This was her first visit to a dentist. She was the sibling of the case described above. Extra-oral findings were convex profile with incompetent lips.[jicdro.org]
Pain
  • […] carcinoma Hypotrichosis Hypohidrosis Sparse and thin eyebrow Nail dysplasia Agenesis of permanent teeth Edema Sparse hair Erythema Macrocephaly Rare Symptoms - Less than 30% cases Relative macrocephaly Ridged nail Ectodermal dysplasia Hyperhidrosis Pain[mendelian.co]
  • Besides these, no other complaint was present, such as pain. Patient’s past dental, medical and drug history were non-contributory.[ijcdc.com]
Pathologist
  • As such, it should be useful to a varied audience, including not only the oral and maxillofacial surgeon but also the oral pathologist, the practitioner of oral medicine, and members of the craniofacial team--the orthodontist, pediatric dentist, speech[books.google.com]
Poor Oral Hygiene
  • The patients’ maintenance of oral hygiene had to be emphasized as high recurrence rates have been associated with poor oral hygiene.[ 8, 9 ] On follow-up after a year, the patient showed a mild recurrence of the gingival overgrowth.[ncbi.nlm.nih.gov]
Selective Tooth Agenesis
  • tooth agenesis Subcutaneous lipoma Chondrosarcoma Trichilemmal cyst Intestinal polyp Adrenocortical carcinoma Absent gallbladder Papillary thyroid carcinoma Keloids Thoracic kyphoscoliosis Epidermoid cyst Stomach cancer Neoplasm of the lung Skin ulcer[mendelian.co]
Progressive Hearing Loss
  • The case was diagnosed to be idiopathic, generalized gingival fibromatosis with progressive hearing loss. The gingival overgrowth was managed by gingivectomy and periodic review.[ncbi.nlm.nih.gov]
  • Recurrent lobular capillary hemangioma with satellitosis (Warner and Wilson-Jones syndrome). ( 24738100 ) Ballin J.S....Satter E.K. 2014 8 Idiopathic gingival fibromatosis associated with progressive hearing loss: A nonfamilial variant of Jones syndrome[malacards.org]
  • Idiopathic gingival fibromatosis associated with progressive hearing loss: A nonfamilial variant of Jones syndrome. Contemp Clin Dent 2014;5:260-3. 10. Pol DG, Lobo TM, Pol SD.[ijcdc.com]
Eruptions
  • Gingival fibromatosis and significant tooth eruption delay in an 11-year-old male: A 30-month follow-up. Int J Paediatr Dent. 2005; 15 :294–302. [ PubMed ] [ Google Scholar ] 9. Ramer M, Marrone J, Stahl B, Burakoff R.[ncbi.nlm.nih.gov]
  • It has been recommended that whenever possible, this treatment should be performed after the complete eruption of permanent teeth. [2] The goal of treatment for sensorineural hearing loss is to improve hearing.[rarediseases.info.nih.gov]
  • However, severe cases of HGF may require gingivectomy to allow the dentition to erupt when the gingival overgrowth is thought to be responsible for lack of anticipated tooth eruption.[pocketdentistry.com]
  • The eruption of teeth within bone tends to be normal, even when there is partial or complete gingival coverage of the dentition (Fletcher 1966).[documentslide.com]
Hirsutism
  • Syndrome of gingival hyperplasia, hirsutism and convulsions. J Pediatr 1965: 67: 499-502. Winstock D. Hereditary gingivofibromatosis. Br J Oral Surg 1964: 2: 59-64. Zackin SJ, Weisberger D. Hereditary gingival fibromatosis. Re- port of a family.[documentslide.com]
Cryptorchidism
  • Authors Show Affiliations, Hacettepe University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery, Ankara, Turkey. okasabog@hacettepe.edu.tr, Source MeSH Abnormalities, Multiple Adult Cryptorchidism Fibromatosis, Gingival Hearing Loss[wwww.unboundmedicine.com]
  • HEMIHYPERPLASIA, ISOLATED; IH Is also known as hemihyperplasia;hhp, hemihypertrophy, isolated;hemi 3 syndrome; hemicorporal hypertrophy; isolated hemihypertrophy Related symptoms: Autosomal dominant inheritance Scoliosis Cryptorchidism Neoplasm Intellectual[mendelian.co]

Treatment

  • It has been recommended that whenever possible, this treatment should be performed after the complete eruption of permanent teeth. [2] The goal of treatment for sensorineural hearing loss is to improve hearing.[rarediseases.info.nih.gov]
  • This case report describes the evaluation and treatment of a 42-year-old male patient with hereditary gingival fibromatosis, sensorineural hearing loss, undescended testis and maxillary odontogenic cyst (Jones Syndrome).[wwww.unboundmedicine.com]
  • Hereditary gingival fibromatosis: Identification, treatment, control. J Am Dent Assoc. 1996; 127 :493–5. [ PubMed ] [ Google Scholar ] 10. Bittencourt LP, Campos V, Moliterno LF, Ribeiro DP, Sampaio RK.[ncbi.nlm.nih.gov]
  • After treatment, regular recall visits are necessary in order to evaluate oral hygiene, and the stability of the periodontal treatment.[jisponline.com]

Prognosis

  • Oral hygiene and the superimposition of plaque accumulation have a crucial effect on the prognosis of gingival fibromatosis.[ijcdc.com]
  • Prognosis Half of all reported patients were deceased at the time of the initial report. 20% died in the first year, another 17% in the second year and 13% between 2 and 10 years of age.[atlasgeneticsoncology.org]

Etiology

  • The growth is slow and progressive and is drug-induced, idiopathic, or hereditary in etiology. It occurs isolated or frequently as a component of various syndromes.[ncbi.nlm.nih.gov]
  • Many forms of gingival fibromatosis are of unknown etiology and termed as idiopathic gingival fibromatosis (IGF).[mdcan-uath.org]
  • Unilateral fibrotic enlargement of gingiva with unknown etiology: A rare findings. J Oral Res Rev [serial online] 2015 [cited 2019 May 13];7:62-4.[jorr.org]
  • Diagnosis: recurrent pyogenic granuloma or Warner and Wilson-Jones syndrome (1, 2). ( 21103841 ) Benmously-Mlika R....Mokhtar I. 2011 10 Two new patients with Curry-Jones syndrome with trichoblastoma and medulloblastoma suggest an etiologic role of the[malacards.org]
  • The bacterial etiology of destructive periodontal disease: current concepts. J Periodontol 1992; 63: 322-331. [8] Trott JR, Cross HG. An analysis of the principal reasons for tooth extractions in 1813 patients in Manitoba.[article.sapub.org]

Epidemiology

  • Global epidemiology of periodontal diseases: an overview. Periodontol 2000 2002; 29: 7-10. [2] Novak JM. Classification of diseases and conditions affecting the periodontium. In: Newman MG, Takei HH, Klokkevold PR, Carranza FA.[article.sapub.org]
  • Gingival overgrowth in children: epidemiology, pathogenesis, and complications. A literature review. J Periodontol 2005; 76 ( 1): 3–10. 12. Jones G, Wilroy RS Jr, McHaney V.[nature.com]
Sex distribution
Age distribution

Prevention

  • The patient has been periodically followed up for plaque control, which is important in preventing recurrence after treatment [Figure 6] a, b, c. Figure 1: Front view (a) and facial profi le (b) of the father.[jdrntruhs.org]
  • Since it is idiopathic and has a genetic cause for its occurrence, it cannot be prevented. Both cases underwent a surgical intervention to rectify the abnormality and were followed from 6 months to 1 year, during which there was no recurrence.[jicdro.org]
  • The enlargement can be at minimal size by preventing secondary inflammatory involvement 7. Present case report highlights the unusual coexistence of nonsyndromic idiopathic gingival fibromatosis.[ijcdc.com]
  • A thirty-year old male presented with gradual progressive enlargement of both gums since childhood with recent spurt in growth since two years which prevented proper speech articulation, mastication and caused inadequate lip apposition.[ijri.org]
  • Marshfield, WI 54449, USA (Note : for Links provided by Atlas : click ) Other names Schinzel-Giedion syndrome Atlas_Id 10129 Genes implicated in SETBP1 Note The use of the long form of the name, Schinzel-Giedion midface retraction syndrome, is preferred to prevent[atlasgeneticsoncology.org]

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