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Gingival Fibromatosis-Hypertrichosis Syndrome

CGHT


Presentation

  • On intraoral examination, generalized gingival hyperplasia was present covering the anatomical crowns of the deciduous teeth as well as the permanent teeth.[ijdr.in]
  • Thus, the present report expands the phenotypic spectrum of this uncommon syndrome. Still can't find the full text of the article? We can help you send a request to the authors directly.[pubfacts.com]
  • Hypertrichosis may be present at birth but often appears at puberty. The entity was later on defined as generalized hypertrichosis terminalis with or without gingival hyperplasia ( OMIM 135400 ).[lessonsingenetics.blogspot.com]
  • A downy growth of hair was present on the cheeks, chin, upper lip, and chin [Figure 1], and a profuse growth of dark brown hair was present on both the upper and lower limbs the mid back [Figure 2] a and b.[jiaomr.in]
  • Figure 4: (G) CT scan there were no abnormalities (H) orthopantography: all teeth were present and in good position.[latunisiemedicale.com]
Epilepsy
  • Another well-known syndrome with gingival fibromatosis associates generalized hypertrichosis and inconstant mental retardation and epilepsy.[ncbi.nlm.nih.gov]
  • Various syndromes have been associated with these features such as epilepsy, mental retardation, cardiomegaly, or osteochondrodysplasia.[ijdr.in]
  • Gingival overgrowth, congenital generalized hypertrichosis, mental retardation and epilepsy: case report and overview. Clin Dysmorphol 2009;18(4):205–8. 17. Stankiewicz P, Pursley AN, Cheung SW.[degruyter.com]
  • We have to wait up to the 1971 for a second report on the syndrome, when Witkop described 52 cases of gingival fibromatosis with hypertrichosis, noting an association with epilepsy and mental retardation.[lessonsingenetics.blogspot.com]
Feeding Difficulties
  • difficulties Cleft palate Nystagmus Cornea verticillata Abnormal glomerular filtration rate Abnormality of the common coagulation pathway Increased glomerular filtration rate Increased carotid artery intimal medial thickness Abnormal ST segment Abnormal[mendelian.co]
Dysmorphic Face
  • Keywords: Ambras syndrome, dysmorphic face, gingival fibromatosis, hypertrichosis How to cite this article: Ishita A, Sujatha G P, Pramod G V, Ashok L. Ambras syndrome: A rare case report.[jisppd.com]
Gingival Swelling
  • Patient gave history of trauma to the left side of his face three months ago after which the gingival swelling first appeared and gradually increased to its present size. However, no correlation was found between trauma and gingival enlargement.[hindawi.com]
Cardiomegaly
  • Various syndromes have been associated with these features such as epilepsy, mental retardation, cardiomegaly, or osteochondrodysplasia.[ijdr.in]
  • It is often associated with additional anomalies including gingival hyperplasia, deafness, cardiomegaly and bone abnormalities. The association of gingival fibromatosis and a coarse facies could further worsen the esthetics.[jiaomr.in]
Freckles
  • The most characteristic features in this case were posterior laryngeal cleft and the presence of numerous freckles and lentigines in childhood. Growth restriction, microcephaly and moderate mental retardation were also observed.[lessonsingenetics.blogspot.com]
  • […] to malignancy Peutz-Jeghers Syndrome inheritance mutation autosomal dominant 1 in 1000,000 to 200,00 births 35% of patients represents new mutation mutation of STK11 gene on chromosome 19p13.3 Peutz-Jeghers Syndrome signs and symptoms mucocutaneous freckle-like[quizlet.com]
  • Microphthalmia Long philtrum Short nose Depressed nasal ridge Abnormality of the parathyroid gland Widely spaced teeth Relative macrocephaly Peritonitis Wide nasal base Multiple mucosal neuromas Ganglioneuromatosis Prominent corneal nerve fibers Inguinal freckling[mendelian.co]
Neglect
  • Though this is not a serious condition clinically, psychosocial trauma cannot be neglected owing to the cosmetic disfigurement it produces. [ FULL TEXT ] [ PDF ]*[ijdr.in]
  • Though this is not a serious condition clinically, psychosocial trauma cannot be neglected owing to the cosmetic disfigurement it produces. [Indexed for MEDLINE] Free full text[ncbi.nlm.nih.gov]
  • Though this is not a serious condition clinically, psychosocial trauma cannot be neglected...[medworm.com]
  • Idiopathic gingival fibromatosis : A neglected case. Indian J Radiol Imaging 2002;12:335-8 How to cite this URL: Pappachan B, Narayan J, Nayak A. Idiopathic gingival fibromatosis : A neglected case.[ijri.org]
Learning Difficulties
  • difficulties, and a characteristic facies.[lessonsingenetics.blogspot.com]
  • The phenotype in this case includes skeletal malformations (lower limb bowing, progressive scoliosis and dental abnormalities), feeding problems, mild learning difficulties, and a characteristic facial appearance.[pesquisa.bvsalud.org]
Cognitive Deficit
  • Further, social cognitive deficits must be considered as important target for intervention among the at risk siblings to improve their outcome.[medworm.com]
  • deficits Intellectual impairment Mental impairment [ more ] 0100543 Downslanted palpebral fissures Downward slanting of the opening between the eyelids 0000494 Epicanthus Eye folds Prominent eye folds [ more ] 0000286 Seizures Seizure 0001250 Synophrys[rarediseases.info.nih.gov]

Treatment

  • After treatment, regular recalls are necessary to evaluate oral hygiene and stability of surgical treatment. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms.[ijdr.in]
  • Stay at the forefront of your field with updated treatment methods throughout, as well as an increased focus on patients with skin of color.[books.google.com]
  • After treatment, regular recall visits are necessary in order to evaluate oral hygiene, and the stability of the periodontal treatment.[jisponline.com]

Prognosis

  • Diagnosis - Zimmerman-Laband syndrome Prognosis - Zimmerman-Laband syndrome Prognosis is dominated by the risk of recurrence, which is high. Physical systemic evaluation is essential. The syndrome is not life-threatening.[checkorphan.org]
  • Oral hygiene and the superimposition of plaque accumulation have a crucial effect on the prognosis of HGF.[njms.in]
  • Prognosis is precarious and the risk of recurrence exists.[ojrd.biomedcentral.com]

Etiology

  • ., who described the etiology as specific genetic alteration, that is, pericentric inversion (8) (p11.2; q22,) and can also be due to fetal alcohol syndrome and fetal hydentoin syndrome. [3], [4] AS is characterized by increase in vellous type of hair[jisppd.com]
  • […] by etiological heterogeneity [ 4, 23, 29, 41, 77 ].[ojrd.biomedcentral.com]
  • Conclusion GF is a rare and slowly progressive condition that is also characterized by etiological heterogeneity.[omicsonline.org]
  • […] is reported to have a phenotype frequency of 1:175,000 and a gene frequency of 1:350,000 assuming that the condition to be inherited is the result of an autosomal dominant gene. [3] Mutation in son-of-seven less gene (SOS-1) has been identified as an etiologic[saudijhealthsci.org]
  • The hyperplastic response does not involve the periodontal ligament and occurs peripheral to the alveolar bone within attached and marginal gingival. [8] There is no consensus of the cellular and molecular mechanisms involved in the etiology of gingival[contempclindent.org]

Epidemiology

  • Mutat 2009;30:583 ) "Hyaline fibromatosis syndrome" proposed as an umbrella term for juvenile hyaline fibromatosis and infantile systemic hyalinosis since the two syndromes have significant clinicopathologic overlap ( J Am Acad Dermatol 2009;61:695 ) Epidemiology[pathologyoutlines.com]
  • The longer wavelength Nd: YAG laser is considered safest in treating darker skin phototypes. [6] Reporting such rare syndromes not only adds to the database, but pooled data analysis may give us a better insight into patterns of inheritance, epidemiology[ijdvl.com]
  • Epidemiology, diagnosis and management of hirsutism: a consensus statement by the Androgen Excess and Polycystic Ovary Syndrome Society. Hum Reprod Update. 2011 Nov 6. [Medline]. Liu K, Motan T, Claman P. No. 350-Hirsutism: Evaluation and Treatment.[emedicine.medscape.com]
  • 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]
  • 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

  • URI: Appears in Collections: Journal of the Indian Society of Pedodontics and Preventive Dentistry Files in This Item: There are no files associated with this item.[imsear.li.mahidol.ac.th]
  • Prevention - Zimmerman-Laband syndrome Not supplied. Diagnosis - Zimmerman-Laband syndrome Prognosis - Zimmerman-Laband syndrome Prognosis is dominated by the risk of recurrence, which is high. Physical systemic evaluation is essential.[checkorphan.org]
  • Tea tree oil : Tea tree oil is purported to have antiseptic properties, and has been used traditionally to prevent and treat infections.[livingnaturally.com]
  • Severe overgrowth can result in crowding of the tongue, speech impediment, and difficulty with mastication and can prevent normal closure of lips. [5] The onset of gingival overgrowth usually coincides with the eruption of the permanent incisors, or,[saudijhealthsci.org]

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