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An enigma of Gorlin–Goltz syndrome: Two cases reported in mother and daughter

Gorlin–Goltz syndrome (GGS) also known as the nevoid basal cell carcinoma syndrome or the nevus–Bifid rib syndrome is an inherited autosomal dominant syndrome. It is caused by genetic alteration produced by a mutation in the “patched” tumor suppressor gene. This rare syndrome is characterized by bas...

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Autores principales: Sahu, Sudipto, Sahoo, Sushil, Banerjee, Rajarshi, Ghosh, Sucharu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6421933/
https://www.ncbi.nlm.nih.gov/pubmed/30967739
http://dx.doi.org/10.4103/jomfp.JOMFP_160_18
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author Sahu, Sudipto
Sahoo, Sushil
Banerjee, Rajarshi
Ghosh, Sucharu
author_facet Sahu, Sudipto
Sahoo, Sushil
Banerjee, Rajarshi
Ghosh, Sucharu
author_sort Sahu, Sudipto
collection PubMed
description Gorlin–Goltz syndrome (GGS) also known as the nevoid basal cell carcinoma syndrome or the nevus–Bifid rib syndrome is an inherited autosomal dominant syndrome. It is caused by genetic alteration produced by a mutation in the “patched” tumor suppressor gene. This rare syndrome is characterized by basal cell carcinoma of skin, multiple odontogenic keratocyst and bifid ribs along with other features such as hypertelorism, sex organ abnormalities, palmar and/or plantar pits and calcification of falx cerebri. Early detection and treatment are essential for patients suffering from this syndrome. Only a few cases of this syndrome with familial background have been reported from India. In this study, we present a rare case of GGS in a mother and her daughter. The purpose of this study is to discuss the role of a dentist in early detection and the need for a multidisciplinary approach for the treatment of this syndrome.
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spelling pubmed-64219332019-04-09 An enigma of Gorlin–Goltz syndrome: Two cases reported in mother and daughter Sahu, Sudipto Sahoo, Sushil Banerjee, Rajarshi Ghosh, Sucharu J Oral Maxillofac Pathol Case Report Gorlin–Goltz syndrome (GGS) also known as the nevoid basal cell carcinoma syndrome or the nevus–Bifid rib syndrome is an inherited autosomal dominant syndrome. It is caused by genetic alteration produced by a mutation in the “patched” tumor suppressor gene. This rare syndrome is characterized by basal cell carcinoma of skin, multiple odontogenic keratocyst and bifid ribs along with other features such as hypertelorism, sex organ abnormalities, palmar and/or plantar pits and calcification of falx cerebri. Early detection and treatment are essential for patients suffering from this syndrome. Only a few cases of this syndrome with familial background have been reported from India. In this study, we present a rare case of GGS in a mother and her daughter. The purpose of this study is to discuss the role of a dentist in early detection and the need for a multidisciplinary approach for the treatment of this syndrome. Medknow Publications & Media Pvt Ltd 2019-02 /pmc/articles/PMC6421933/ /pubmed/30967739 http://dx.doi.org/10.4103/jomfp.JOMFP_160_18 Text en Copyright: © 2019 Journal of Oral and Maxillofacial Pathology http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Sahu, Sudipto
Sahoo, Sushil
Banerjee, Rajarshi
Ghosh, Sucharu
An enigma of Gorlin–Goltz syndrome: Two cases reported in mother and daughter
title An enigma of Gorlin–Goltz syndrome: Two cases reported in mother and daughter
title_full An enigma of Gorlin–Goltz syndrome: Two cases reported in mother and daughter
title_fullStr An enigma of Gorlin–Goltz syndrome: Two cases reported in mother and daughter
title_full_unstemmed An enigma of Gorlin–Goltz syndrome: Two cases reported in mother and daughter
title_short An enigma of Gorlin–Goltz syndrome: Two cases reported in mother and daughter
title_sort enigma of gorlin–goltz syndrome: two cases reported in mother and daughter
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6421933/
https://www.ncbi.nlm.nih.gov/pubmed/30967739
http://dx.doi.org/10.4103/jomfp.JOMFP_160_18
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