Cargando…
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 (...
Autores principales: | , , , |
---|---|
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 |
_version_ | 1782463665844256768 |
---|---|
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. |
format | Online Article Text |
id | pubmed-5086016 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Jaypee Brothers Medical Publishers |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT tandonsandeep gorlingoltzsyndromeararecasereportofa11yearoldchild AT chauhanyashwant gorlingoltzsyndromeararecasereportofa11yearoldchild AT sharmameenakshi gorlingoltzsyndromeararecasereportofa11yearoldchild AT jainmanish gorlingoltzsyndromeararecasereportofa11yearoldchild |