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]
Figure 5 Abscess presenting as localized gingival enlargement. [wjgnet.com]
Figure 10: Patient’s father presenting with swelling of his gum with areas of surface ulceration and pus collection. Figure 10: Patient’s father presenting with swelling of his gum with areas of surface ulceration and pus collection. [academic.oup.com]
Here, we present an unusual clinical presentation of nonsyndromic idiopathic gingival fibromatosis with unknown etiology in a 20-year-old female. [mdcan-uath.org]
Hematological
- Hemophilia A
Due To Glucose Phosphate Isomerase Deficiency 1 Hemophagocytic Lymphohistiocytosis, Familial, 2 2 Hemophagocytic Lymphohistiocytosis, Familial, 3 2 Hemophagocytic Lymphohistiocytosis, Familial, 4 2 Hemophagocytic lymphohistiocytosis, Familial, 5 2 Hemophilia [preventiongenetics.com]
[…] sickness 【血栓症】*thrombosis 【血栓性静脈炎】*thrombophlebitis 【血栓性動脈内膜炎】*thromboendarteritis 【血栓塞栓症】*thromboembolism 【血痰】*bloody sputum *hemosputum *sputum cruentum[L] 【血鉄症】*hemosiderosis 【血糖症】*glycemia 【血尿】*hematuria *erythruria [赤血球尿症] *urina cruenta[L] 【血友病】*hemophilia [medo.jp]
Entire Body System
- Surgical Procedure
The patients were treated with scaling and root planing along with surgical procedure like gingivectomy. Patients reported 1 month after they had undergone the surgical procedure and were again reviewed 6 months later. [jicdro.org]
procedures being performed using a facial or laryngeal mask. [elsevier.es]
- Pallor
Apart from gingival enlargement other associated features could be oral ulceration, spontaneous gingival bleeding, petechiae, mucosal pallor, herpetic infections and candidiasis. [wjgnet.com]
Gastrointestinal
- Intestinal Disease
Genetic intestinal disease Genetic intestinal disease due to fat malabsorption Genetic intestinal polyposis Genetic intractable diarrhea of infancy Genetic larynx anomaly Genetic lens and zonula anomaly Genetic lethal multiple congenital anomalies/dysmorphic [orpha.net]
Cardiovascular
- Hypertension
Genetic head and neck malformation Genetic hemoglobinopathy Genetic hemophagocytic lymphohistiocytosis Genetic hyperaldosteronism Genetic hyperferritinemia without iron overload Genetic hyperparathyroidism Genetic hyperpigmentation of the skin Genetic hypertension [orpha.net]
[…] serum estradiol Keratoconjunctivitis Corneal scarring Narrow mouth Osteoporosis Polyhydramnios Midface retrusion Congestive heart failure Malar flattening Kyphosis Cardiomyopathy Skeletal muscle atrophy Corneal ulceration Generalized hyperkeratosis Hypertension [mendelian.co]
【高血圧】*hypertension 【高血圧眼底】*fundus hypertonicus[L] 【高血圧性心疾患】*hypertensive heart disease (略 HHD) 【高血圧性脳症】*hypertensive encephalopathy 【高血圧性網膜症】*hypertensive retinopathy 【高血圧発作】*hypertensive episode 【高血糖症】*hyperglycemia =過血糖症 【高コレステロール血症】*cholesterolemia [medo.jp]
Primary, Autosomal Recessive 2 1 Hyperuricemia, Pulmonary Hypertension, Renal Failure, And Alkalosis 2 Hypobetalipoproteinemia 1 Hypocalcemia, autosomal dominant 4 Hypocalcemia, autosomal dominant 2 2 Hypocalciuric Hypercalcemia, Familial, Type III [preventiongenetics.com]
Combination therapy, such as with amlodipine, a calcium channel blocker used in the management of hypertension and angina, and cholesterol-lowering drugs, can result in more severe GO than single-agent therapy [17]. [ojrd.biomedcentral.com]
Jaw & Teeth
- Tooth Loss
Tooth loss is preceded by gingival inflammation and subsequent periodontal bone loss and tooth mobility; Aggregatibacter actinomycetemcomitans (AA) is an identified pathogen in PLS. [pocketdentistry.com]
An analysis of the principal reasons for tooth extractions in 1813 patients in Manitoba. Dent Pract Dent Rec 1966; 17: 20-27. [9] Hirschfeld L, Wasserman B. A long-term survey of tooth loss in 600 treated periodontal patients. [article.sapub.org]
Pleiotropy in Coffin-Lowry syndrome: Sensorineural hearing deficit and premature tooth loss as early manifestations. Am J Med Genet. 1993; 45 :552–7. [ PubMed ] [ Google Scholar ] 6. Jorgenson RJ, Cocker ME. [ncbi.nlm.nih.gov]
- 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]
In addition to being disfiguring, GO may also lead to poor oral hygiene and decreased oral function in individuals already suffering from conditions such as epilepsy, cardiovascular diseases, or immunosuppression [107]. [ojrd.biomedcentral.com]
- 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]
Skin
- 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]
Syndrome of gingival hypertrophy, hirsutism, men-tal retardation and brachymetacarpia in two sisters: specific en-tity or variant of a described condition? Am J Med Genet2000;95:241-6.Bakaeen G, Scully C. [documents.tips]
多発性粘膜神経腫】*multiple mucosal neuroma 【多発性脳梗塞】*multiple cerebral infarction 【多発性脳神経炎】*cranial polyneuritis 【多発性嚢胞】*multiple cysts[pl] 【多発性麻痺】*polyplegia 【多脾症】*polysplenia 【多病】*polypathia 【多病巣性運動神経症】*multifocal motor neuropathy 【多尿症】*polyuria 【多毛症】*pilosis *hirsutism [medo.jp]
- Skin Disease
Psoriasis, a chronic inflammatory skin disease, of multifactorial origin, is characterised by keratinocyte hyperproliferation, dedifferentiation, neoangiogenesis and inflammation. [casereports.bmj.com]
[…] eruption *skin rash 【皮膚炎】*dermatitis 【皮膚関節炎】*dermatoarthritis 【皮膚乾燥症】*dry skin *xeroderma/*xerodermia *xerosis cutis[L] (=乾皮症、乾燥皮膚) 【皮膚癌】*skin cancer 【皮膚筋炎】*dermatomyositis 【皮膚硬化症】*scleroderma (=強皮症) 【皮膚疾患】【皮膚病】*dermopathy *skin disease 【皮膚症】*dermatosis [medo.jp]
- Skin Rash
[…] eruption *skin rash 【皮膚炎】*dermatitis 【皮膚関節炎】*dermatoarthritis 【皮膚乾燥症】*dry skin *xeroderma/*xerodermia *xerosis cutis[L] (=乾皮症、乾燥皮膚) 【皮膚癌】*skin cancer 【皮膚筋炎】*dermatomyositis 【皮膚硬化症】*scleroderma (=強皮症) 【皮膚疾患】【皮膚病】*dermopathy *skin disease 【皮膚症】*dermatosis [medo.jp]
Ears
- Hearing Impairment
impairment Short stature Sensorineural hearing impairment Hyperkeratosis Palmoplantar keratoderma Oral leukoplakia Dystrophic fingernails Aplasia/Hypoplasia of the eyebrow Keratoconjunctivitis sicca Dystrophic toenail Cerebellar hypoplasia Abnormal dermatoglyphics [mendelian.co]
impairment 0000407 Percent of people who have these symptoms is not available through HPO Autosomal dominant inheritance 0000006 Progressive sensorineural hearing impairment 0000408 Showing of 6 | Last updated: 6/1/2019 The exact, underlying genetic [rarediseases.info.nih.gov]
impairment *[=聴力損失] hearing loss *bradyacusia *hypacusis (ニ) 【ニコチン性口内炎】*nicotinic stomatitis 【ニューモシスチス症】*pneumocystosis 【ニワトリ歩行】*steppage gait (=鶏歩) (肉) 【肉眼的膿尿】*macropyuria 【肉芽腫】*granuloma 【肉芽腫症】*granulomatosis 【肉芽腫性口唇炎】*cheilitis granulomatosa 【肉腫】* [medo.jp]
- Progressive Hearing Loss
Periodic review and audiometry once a year were suggested to monitor the progression of the hearing loss. [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]
Neurologic
- Seizure
Seizures of varying types, often including infantile spasms, are reported in most patients. [atlasgeneticsoncology.org]
[…] hotosensitivity B lood Dyscrasias: hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia R enal: proteinuria, casts, hematuria, azotemia A NA+ I mmune abnormalities: LE cells, anti-dsDNA, anti-Smith, VDRL/RPR+, decreased C3 N eurological Disease: seizures [pathologyexpert.com]
Plus, Type 1 5 Generalized Epilepsy With Febrile Seizures Plus, Type 2 3 Generalized Epilepsy With Febrile Seizures Plus, Type 3 5 Generalized Epilepsy With Febrile Seizures Plus, Type 7 5 Generalized Epilepsy with Febrile Seizures Plus, Type 9 3 Genitopatellar [preventiongenetics.com]
[…] dominant type 2 Cortical dysgenesis with pontocerebellar hypoplasia due to TUBB3 mutation Kennedy disease Optic atrophy 2 Isolated growth hormone deficiency type 1A Pseudohypoparathyroidism type 2 Prolidase deficiency Intellectual disability, epileptic seizures [checkrare.com]
There was no history of fever, prolonged medications, anorexia, weight loss, seizures or hearing loss. [ijri.org]
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]
After treatment, regular recall visits are necessary in order to evaluate oral hygiene, and the stability of the periodontal treatment. [jisponline.com]
Treatment of idiopathic gingival enlargement depends on the severity of the enlargement. [ijcdc.com]
The information presented is not intended to replace medical advice or treatment from your own doctor or healthcare provider. [wellnessadvocate.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]
Prognosis is precarious and the risk of recurrence exists. Background Gingival fibromatosis (GF) is a rare condition characterized by pathological, diffuse or local growth of gingiva. [ojrd.biomedcentral.com]
Etiology
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 2015;7:62-4 How to cite this URL: Khan MA, Tripathi AK, Jaishwal RK, Agrawal P. [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 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]
Genetic disorders associated with gingival enlargement: They can be divided into 4 primary categories based on their etiology, clinical features and histology. [wjgnet.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]
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]
Epidemiology GF associated with hereditary factors (non-syndromic) HGF (GINGF, ORPHA 2024, MIM 135300) is a rare disease with unknown prevalence [2–4] (Table 1). [ojrd.biomedcentral.com]
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
And are not intended to diagnose, treat, cure or prevent disease. The information presented is not intended to replace medical advice or treatment from your own doctor or healthcare provider. [wellnessadvocate.com]
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]
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]
Early monitoring of hearing quality is important for young NS-patients in order to prevent speech abnormalities. Chest/back abnormalities are common in NS-patients in a form of pectus carinatum and/or excavatum and scoliosis. [centogene.com]