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Osteogenesis Imperfecta (Type IV) with Dental Findings in Siblings

Osteogenesis imperfecta (OI) is a hereditary disorder characterized by increased tendency for bone fractures due to high fragility. The clinical and radiological features of OI manifest in different age groups, although the disease is congenital in nature. Besides bone fragility, features like laxit...

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Autores principales: Shetty, Shishir Ram, Dsouza, Deepa, Babu, Subhas, Balan, Preethi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3335498/
https://www.ncbi.nlm.nih.gov/pubmed/22567450
http://dx.doi.org/10.1155/2011/970904
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author Shetty, Shishir Ram
Dsouza, Deepa
Babu, Subhas
Balan, Preethi
author_facet Shetty, Shishir Ram
Dsouza, Deepa
Babu, Subhas
Balan, Preethi
author_sort Shetty, Shishir Ram
collection PubMed
description Osteogenesis imperfecta (OI) is a hereditary disorder characterized by increased tendency for bone fractures due to high fragility. The clinical and radiological features of OI manifest in different age groups, although the disease is congenital in nature. Besides bone fragility, features like laxity of the ligaments, blue sclera, growth retardation, and scoliosis are also observed. In severe cases, respiratory distress and death have been reported. The most important oral finding in OI is the presence of yellowish-brown-coloured brittle teeth characteristic of dentinogenesis imperfecta. Genetic factors play a very important role in the pathogenesis of OI either as a dominant or recessive factor. When a child has OI, there is a 25% chance of the sibling to have the same disorder. We report two cases of OI in siblings born to parents with a history of consanguineous marriage. The clinical and radiological features of the two cases are described in detail.
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spelling pubmed-33354982012-05-07 Osteogenesis Imperfecta (Type IV) with Dental Findings in Siblings Shetty, Shishir Ram Dsouza, Deepa Babu, Subhas Balan, Preethi Case Rep Dent Case Report Osteogenesis imperfecta (OI) is a hereditary disorder characterized by increased tendency for bone fractures due to high fragility. The clinical and radiological features of OI manifest in different age groups, although the disease is congenital in nature. Besides bone fragility, features like laxity of the ligaments, blue sclera, growth retardation, and scoliosis are also observed. In severe cases, respiratory distress and death have been reported. The most important oral finding in OI is the presence of yellowish-brown-coloured brittle teeth characteristic of dentinogenesis imperfecta. Genetic factors play a very important role in the pathogenesis of OI either as a dominant or recessive factor. When a child has OI, there is a 25% chance of the sibling to have the same disorder. We report two cases of OI in siblings born to parents with a history of consanguineous marriage. The clinical and radiological features of the two cases are described in detail. Hindawi Publishing Corporation 2011 2011-09-06 /pmc/articles/PMC3335498/ /pubmed/22567450 http://dx.doi.org/10.1155/2011/970904 Text en Copyright © 2011 Shishir Ram Shetty et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Shetty, Shishir Ram
Dsouza, Deepa
Babu, Subhas
Balan, Preethi
Osteogenesis Imperfecta (Type IV) with Dental Findings in Siblings
title Osteogenesis Imperfecta (Type IV) with Dental Findings in Siblings
title_full Osteogenesis Imperfecta (Type IV) with Dental Findings in Siblings
title_fullStr Osteogenesis Imperfecta (Type IV) with Dental Findings in Siblings
title_full_unstemmed Osteogenesis Imperfecta (Type IV) with Dental Findings in Siblings
title_short Osteogenesis Imperfecta (Type IV) with Dental Findings in Siblings
title_sort osteogenesis imperfecta (type iv) with dental findings in siblings
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3335498/
https://www.ncbi.nlm.nih.gov/pubmed/22567450
http://dx.doi.org/10.1155/2011/970904
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