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Clinical and Radiographic Findings of Pyknodysostosis: Report of a Case | ||
| Journal of Dentistry | ||
| مقاله 8، دوره 4، شماره 1، شهریور 2003، صفحه 69-75 اصل مقاله (236.81 K) | ||
| نوع مقاله: Case Report | ||
| نویسنده | ||
| HR. Pakshir* | ||
| Assistant Professor of Department Orthodontics, School of Dentistry, Shiraz University of Medical Sciences | ||
| چکیده | ||
| Pyknodysostosis is a rare sclerosing bone disorder with autosomal recessive inheritance. It is characterized by short stature, brachycephaly, short and stubby fingers, open cranial sutures and fontanelle and diffuse osteosclerosis. Here, we report a case of pyknodysostosis with characteristic physical and radiographical findings. A large skull and generalized bonehyperdensification are typical features. This malformation shows a loss of the normal mandibular angle. The patient had a small face, with a big nose and a large head.Irregularity of the teeth and a wide arched furrowed palate are other intra-oral findings.Although the profile showed to be convex, the patient had a skeletal and dental class III relation.Key words: Pyknodysostosis, Dwarfism, Hyperdensification | ||
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