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,...
Main Authors: | , , , , , |
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Format: | Article |
Language: | English |
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Wolters Kluwer Medknow Publications
2011-01-01
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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. |
first_indexed | 2024-04-14T07:51:25Z |
format | Article |
id | doaj.art-09bca9424a0b421ca93f41c2b2442e85 |
institution | Directory Open Access Journal |
issn | 0972-1363 0975-1572 |
language | English |
last_indexed | 2024-04-14T07:51:25Z |
publishDate | 2011-01-01 |
publisher | Wolters Kluwer Medknow Publications |
record_format | Article |
series | Journal of Indian Academy of Oral Medicine and Radiology |
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 |