Pycnodysostosis with Special Emphasis on Dentofacial Characteristics

Pycnodysostosis is an autosomal recessive disorder that manifests as osteosclerosis of the skeleton due to the defective osteoclasts mediated bone turnover. The diagnosis of this disorder is established on the basis of its characteristic features and must be differentially diagnosed with other bone...

Descripción completa

Detalles Bibliográficos
Autores principales: Khoja, Aisha, Fida, Mubassar, Shaikh, Attiya
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4663328/
https://www.ncbi.nlm.nih.gov/pubmed/26649209
http://dx.doi.org/10.1155/2015/817989
_version_ 1782403277861683200
author Khoja, Aisha
Fida, Mubassar
Shaikh, Attiya
author_facet Khoja, Aisha
Fida, Mubassar
Shaikh, Attiya
author_sort Khoja, Aisha
collection PubMed
description Pycnodysostosis is an autosomal recessive disorder that manifests as osteosclerosis of the skeleton due to the defective osteoclasts mediated bone turnover. The diagnosis of this disorder is established on the basis of its characteristic features and must be differentially diagnosed with other bone disorders. Dental surgeons should be aware of the limitations and possible adverse oral complications such as osteomyelitis of bone in these patients. This will guide them in planning realistic treatment goals. This paper reports the clinical and radiographic features of pycnodysostosis with the great emphasis on its dentofacial characteristics. The aim of this case report is to give an insight into the etiology, pathogenesis, and differential diagnosis of this disorder and to prepare the dentists and maxillofacial surgeons to overcome the challenges in treating these patients.
format Online
Article
Text
id pubmed-4663328
institution National Center for Biotechnology Information
language English
publishDate 2015
publisher Hindawi Publishing Corporation
record_format MEDLINE/PubMed
spelling pubmed-46633282015-12-08 Pycnodysostosis with Special Emphasis on Dentofacial Characteristics Khoja, Aisha Fida, Mubassar Shaikh, Attiya Case Rep Dent Case Report Pycnodysostosis is an autosomal recessive disorder that manifests as osteosclerosis of the skeleton due to the defective osteoclasts mediated bone turnover. The diagnosis of this disorder is established on the basis of its characteristic features and must be differentially diagnosed with other bone disorders. Dental surgeons should be aware of the limitations and possible adverse oral complications such as osteomyelitis of bone in these patients. This will guide them in planning realistic treatment goals. This paper reports the clinical and radiographic features of pycnodysostosis with the great emphasis on its dentofacial characteristics. The aim of this case report is to give an insight into the etiology, pathogenesis, and differential diagnosis of this disorder and to prepare the dentists and maxillofacial surgeons to overcome the challenges in treating these patients. Hindawi Publishing Corporation 2015 2015-11-16 /pmc/articles/PMC4663328/ /pubmed/26649209 http://dx.doi.org/10.1155/2015/817989 Text en Copyright © 2015 Aisha Khoja 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
Khoja, Aisha
Fida, Mubassar
Shaikh, Attiya
Pycnodysostosis with Special Emphasis on Dentofacial Characteristics
title Pycnodysostosis with Special Emphasis on Dentofacial Characteristics
title_full Pycnodysostosis with Special Emphasis on Dentofacial Characteristics
title_fullStr Pycnodysostosis with Special Emphasis on Dentofacial Characteristics
title_full_unstemmed Pycnodysostosis with Special Emphasis on Dentofacial Characteristics
title_short Pycnodysostosis with Special Emphasis on Dentofacial Characteristics
title_sort pycnodysostosis with special emphasis on dentofacial characteristics
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4663328/
https://www.ncbi.nlm.nih.gov/pubmed/26649209
http://dx.doi.org/10.1155/2015/817989
work_keys_str_mv AT khojaaisha pycnodysostosiswithspecialemphasisondentofacialcharacteristics
AT fidamubassar pycnodysostosiswithspecialemphasisondentofacialcharacteristics
AT shaikhattiya pycnodysostosiswithspecialemphasisondentofacialcharacteristics