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  • Title: Familial odontogenic keratocysts. Report of 3 cases and review of Japanese dental literature.
    Author: Kamiya Y, Narita H, Yamamoto T, Kameyama Y, Maeda H, Nakane S.
    Journal: Int J Oral Surg; 1985 Feb; 14(1):73-80. PubMed ID: 2580807.
    Abstract:
    Familial odontogenic keratocysts are described in this report. The Case 1 patient, who has 3 sisters, developed odontogenic keratocysts. The 2 younger sisters (Cases 2 and 3) also had odontogenic keratocysts, although the elder sister did not have any odontogenic cysts. The father of the patients had a history of removal of a jaw cyst, and the mother was found later to have malignant ameloblastoma. Besides the odontogenic keratocysts, the Case 1 patient had basal cell nevus, prominent frontal process, and ocular hypertelorism; the Case 2 patient had prominent frontal process; the Case 3 patient had prominent frontal process, ocular hypertelorism, and squint. All 3 sisters are suspected of being patients with the basal cell nevus syndrome. The Japanese dental literature concerning the basal cell nevus syndrome is reviewed.
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