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Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child

Gorlin-Goltz Syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an autosomal dominant trait caused due to mutations in the patched tumor suppressor gene (PTCH) gene found on the long arm of chromosome 9. The syndrome is characterized by the presence of odontogenic keratocysts (...

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Autores principales: Tandon, Sandeep, Chauhan, Yashwant, Sharma, Meenakshi, Jain, Manish
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Jaypee Brothers Medical Publishers 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5086016/
https://www.ncbi.nlm.nih.gov/pubmed/27843260
http://dx.doi.org/10.5005/jp-journals-10005-1374
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author Tandon, Sandeep
Chauhan, Yashwant
Sharma, Meenakshi
Jain, Manish
author_facet Tandon, Sandeep
Chauhan, Yashwant
Sharma, Meenakshi
Jain, Manish
author_sort Tandon, Sandeep
collection PubMed
description Gorlin-Goltz Syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an autosomal dominant trait caused due to mutations in the patched tumor suppressor gene (PTCH) gene found on the long arm of chromosome 9. The syndrome is characterized by the presence of odontogenic keratocysts (OKCs), basal cell carcinomas, and skeletal malformations. Early diagnosis of the syndrome can be done by pedodontist as OKC is one of the early manifestations of the syndrome. Early diagnosis and treatment is important for long-term prognosis of the syndrome by reducing the severity of cutaneous carcinomas and deformities due to jaw cyst. The present case describes an 11-year-old patient with some typical features of NBCCS, which were diagnosed through its oral and maxillofacial manifestations. This case emphasizes the importance of pedodontist in early recognition of the syndrome. HOW TO CITE THIS ARTICLE: Tandon S, Chauhan Y, Sharma M, Jain M. Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child. Int J Clin Pediatr Dent 2016;9(3):264-268.
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spelling pubmed-50860162016-11-14 Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child Tandon, Sandeep Chauhan, Yashwant Sharma, Meenakshi Jain, Manish Int J Clin Pediatr Dent Case Report Gorlin-Goltz Syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an autosomal dominant trait caused due to mutations in the patched tumor suppressor gene (PTCH) gene found on the long arm of chromosome 9. The syndrome is characterized by the presence of odontogenic keratocysts (OKCs), basal cell carcinomas, and skeletal malformations. Early diagnosis of the syndrome can be done by pedodontist as OKC is one of the early manifestations of the syndrome. Early diagnosis and treatment is important for long-term prognosis of the syndrome by reducing the severity of cutaneous carcinomas and deformities due to jaw cyst. The present case describes an 11-year-old patient with some typical features of NBCCS, which were diagnosed through its oral and maxillofacial manifestations. This case emphasizes the importance of pedodontist in early recognition of the syndrome. HOW TO CITE THIS ARTICLE: Tandon S, Chauhan Y, Sharma M, Jain M. Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child. Int J Clin Pediatr Dent 2016;9(3):264-268. Jaypee Brothers Medical Publishers 2016 2016-09-27 /pmc/articles/PMC5086016/ /pubmed/27843260 http://dx.doi.org/10.5005/jp-journals-10005-1374 Text en Copyright © 2016; Jaypee Brothers Medical Publishers (P) Ltd. This work is licensed under a Creative Commons Attribution 3.0 Unported License. To view a copy of this license, visit http://creativecommons.org/licenses/by/3.0/
spellingShingle Case Report
Tandon, Sandeep
Chauhan, Yashwant
Sharma, Meenakshi
Jain, Manish
Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child
title Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child
title_full Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child
title_fullStr Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child
title_full_unstemmed Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child
title_short Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child
title_sort gorlin-goltz syndrome: a rare case report of a 11-year-old child
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5086016/
https://www.ncbi.nlm.nih.gov/pubmed/27843260
http://dx.doi.org/10.5005/jp-journals-10005-1374
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