Langer-Giedion Syndrome: a Rare Case Report

Langer-Giedion syndrome is a very uncommon autosomal dominant genetic disorder caused by the deletion of chromosomal material. It is characterized by multiple bony exostosis, short stature, mental retardation, and typical facial features. The characteristic appearance of individuals includes sparse...

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Autores principales: Katge, Farhin Ali, Rusawat, Bhavesh Dahyabhai, Shivasharan, Pooja Ravindra, Patil, Devendra Pandurang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Shiraz University of Medical Sciences 2016
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5006835/
https://www.ncbi.nlm.nih.gov/pubmed/27602401
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author Katge, Farhin Ali
Rusawat, Bhavesh Dahyabhai
Shivasharan, Pooja Ravindra
Patil, Devendra Pandurang
author_facet Katge, Farhin Ali
Rusawat, Bhavesh Dahyabhai
Shivasharan, Pooja Ravindra
Patil, Devendra Pandurang
author_sort Katge, Farhin Ali
collection PubMed
description Langer-Giedion syndrome is a very uncommon autosomal dominant genetic disorder caused by the deletion of chromosomal material. It is characterized by multiple bony exostosis, short stature, mental retardation, and typical facial features. The characteristic appearance of individuals includes sparse scalp hair, rounded nose, prominent philtral area and thin upper lip. Some cases with this condition have loose skin in childhood which typically resolves with age. Oral and dental manifestations include micrognathia, retrognathia, hypodontia, and malocclusion based on cephalometric analysis. This report presents a case of Langer-Giedion syndrome in a 10-year-old child.
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spelling pubmed-50068352016-09-07 Langer-Giedion Syndrome: a Rare Case Report Katge, Farhin Ali Rusawat, Bhavesh Dahyabhai Shivasharan, Pooja Ravindra Patil, Devendra Pandurang J Dent (Shiraz) Case Report Langer-Giedion syndrome is a very uncommon autosomal dominant genetic disorder caused by the deletion of chromosomal material. It is characterized by multiple bony exostosis, short stature, mental retardation, and typical facial features. The characteristic appearance of individuals includes sparse scalp hair, rounded nose, prominent philtral area and thin upper lip. Some cases with this condition have loose skin in childhood which typically resolves with age. Oral and dental manifestations include micrognathia, retrognathia, hypodontia, and malocclusion based on cephalometric analysis. This report presents a case of Langer-Giedion syndrome in a 10-year-old child. Shiraz University of Medical Sciences 2016-09 /pmc/articles/PMC5006835/ /pubmed/27602401 Text en © 2016: Journal of dentistry (Shiraz) This is an Open Access article distributed under the terms of the Creative Commons Attribution License, (http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Katge, Farhin Ali
Rusawat, Bhavesh Dahyabhai
Shivasharan, Pooja Ravindra
Patil, Devendra Pandurang
Langer-Giedion Syndrome: a Rare Case Report
title Langer-Giedion Syndrome: a Rare Case Report
title_full Langer-Giedion Syndrome: a Rare Case Report
title_fullStr Langer-Giedion Syndrome: a Rare Case Report
title_full_unstemmed Langer-Giedion Syndrome: a Rare Case Report
title_short Langer-Giedion Syndrome: a Rare Case Report
title_sort langer-giedion syndrome: a rare case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5006835/
https://www.ncbi.nlm.nih.gov/pubmed/27602401
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AT patildevendrapandurang langergiedionsyndromeararecasereport

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