Gorlin-goltz syndrome

Gorlin-Goltz syndrome also known as nevoid basal cell carcinoma syndrome (NBCCS) is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT) in the jaw,...

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Main Authors: B V Shobha, S Mosby, N Barkha, Biswajeet, M Ajit, T Deepak
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2011-01-01
Series:Journal of Indian Academy of Oral Medicine and Radiology
Subjects:
Online Access:http://www.jiaomr.in/article.asp?issn=0972-1363;year=2011;volume=23;issue=5;spage=487;epage=490;aulast=Shobha
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author B V Shobha
S Mosby
N Barkha
Biswajeet
M Ajit
T Deepak
author_facet B V Shobha
S Mosby
N Barkha
Biswajeet
M Ajit
T Deepak
author_sort B V Shobha
collection DOAJ
description Gorlin-Goltz syndrome also known as nevoid basal cell carcinoma syndrome (NBCCS) is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT) in the jaw, multiple basal cell carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic examination in the first decade of life, as KCOTs are usually one of the first manifestations of the NBCCS syndrome. This article reports the case of a 12-year-old girl with Gorlin-Goltz syndrome, emphasizing its clinical and radiographic manifestation. This study highlights the importance of health professionals in the early diagnosis of this syndrome and a multidisciplinary approach to provide a better diagnosis and prognosis.
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spelling doaj.art-09bca9424a0b421ca93f41c2b2442e852022-12-22T02:05:10ZengWolters Kluwer Medknow PublicationsJournal of Indian Academy of Oral Medicine and Radiology0972-13630975-15722011-01-0123548749010.5005/jp-journals-10011-1206Gorlin-goltz syndromeB V ShobhaS MosbyN BarkhaBiswajeetM AjitT DeepakGorlin-Goltz syndrome also known as nevoid basal cell carcinoma syndrome (NBCCS) is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT) in the jaw, multiple basal cell carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic examination in the first decade of life, as KCOTs are usually one of the first manifestations of the NBCCS syndrome. This article reports the case of a 12-year-old girl with Gorlin-Goltz syndrome, emphasizing its clinical and radiographic manifestation. This study highlights the importance of health professionals in the early diagnosis of this syndrome and a multidisciplinary approach to provide a better diagnosis and prognosis.http://www.jiaomr.in/article.asp?issn=0972-1363;year=2011;volume=23;issue=5;spage=487;epage=490;aulast=ShobhaGorlin-Goltz syndromeKeratocvstic odontogenic tumorBasal cell nevus syndrome
spellingShingle B V Shobha
S Mosby
N Barkha
Biswajeet
M Ajit
T Deepak
Gorlin-goltz syndrome
Journal of Indian Academy of Oral Medicine and Radiology
Gorlin-Goltz syndrome
Keratocvstic odontogenic tumor
Basal cell nevus syndrome
title Gorlin-goltz syndrome
title_full Gorlin-goltz syndrome
title_fullStr Gorlin-goltz syndrome
title_full_unstemmed Gorlin-goltz syndrome
title_short Gorlin-goltz syndrome
title_sort gorlin goltz syndrome
topic Gorlin-Goltz syndrome
Keratocvstic odontogenic tumor
Basal cell nevus syndrome
url http://www.jiaomr.in/article.asp?issn=0972-1363;year=2011;volume=23;issue=5;spage=487;epage=490;aulast=Shobha
work_keys_str_mv AT bvshobha gorlingoltzsyndrome
AT smosby gorlingoltzsyndrome
AT nbarkha gorlingoltzsyndrome
AT biswajeet gorlingoltzsyndrome
AT majit gorlingoltzsyndrome
AT tdeepak gorlingoltzsyndrome