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Year : 2015  |  Volume : 3  |  Issue : 3  |  Page : 204-207

Three cases with pycnodysostosis in a family

Department of Orthodontics, Faculty of Dentistry, Kirikkale University, Kirikkale, Turkey

Correspondence Address:
Hasan Kamak
Department of Orthodontics, Faculty of Dentistry, Kirikkale University, 71100 Kirikkale
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2321-3825.159543

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Pycnodysostosis (the Toulouse - Lautrec syndrome), a rare autosomal-recessive disorder is caused by cathepsin K mutation. The main features of the disorder are osteosclerosis, bone fragility, short stature, acro-osteolysis, and delayed closure of the cranial sutures. Other common features are prominent eyes with blue sclera, beaked nose, clavicular dysplasia, obtused mandibular gonial angle, and in some cases visceromegaly. The aim of this case report is to emphasize the main aspects of interest to the orthodontists to give the right treatment to this population.

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