Bani Mehmet, Tezkirecioglu Ali Melih, Akal Nese, Tuzuner Tamer
DDS, Research Assistant, Gazi University, Faculty of Dentistry, Department of Paediatric Dentistry, Ankara, Turkey.
Eur J Dent. 2010 Apr;4(2):215-22.
Ectodermal dysplasia is a hereditary disorder that occurs as a consequence of disturbances in the ectoderm of the developing embryo. The triad of nail dystrophy, alopecia or hypotrichosis and palmoplantar hyperkeratosis is usually accompanied by a lack of sweat glands and a partial or complete absence of primary and/or permanent dentition. Two case reports illustrating the prosthetic rehabilitation of 2 young boys with anhidrotic ectodermal dysplasia associated with severe anodontia are presented. Since the oral rehabilitation of these cases is often difficult; particularly in pediatric patients, treatment should be administered by a multidisciplinary team involving pediatric dentistry, orthodontics, prosthodontics and oral-maxillofacial surgery.
外胚层发育不全是一种遗传性疾病，是发育中胚胎的外胚层受到干扰的结果。指甲营养不良、脱发或毛发稀少以及掌跖角化过度三联征通常伴有汗腺缺乏以及乳牙和/或恒牙部分或完全缺失。本文介绍了两例病例报告，阐述了两名患有与严重无牙症相关的无汗性外胚层发育不全的年轻男孩的修复治疗。由于这些病例的口腔修复通常很困难，尤其是对于儿科患者，治疗应由包括儿童牙科、正畸、修复和口腔颌面外科的多学科团队进行。
