Bala Madhu, Pathak Anuradha
Department of Pedodontics and Preventive Dentistry, JCD Dental College, Sirsa, Haryana, India.
J Oral Maxillofac Pathol. 2011 May;15(2):244-6. doi: 10.4103/0973-029X.84515.
The hereditary condition known as ectodermal dysplasia is characterized by the absence or defect of two or more ectodermally derived structures. The most commonly observed forms of ectodermal dysplasia are the hidrotic and hypohidrotic types; discrimination is based on the absence or presence of sweat glands. A case of 8-year-old male child with hypohidrotic ectodermal dysplasia with complete anodontia of primary as well as secondary dentitions is presented. The child had a short stature, low intelligent quotient (I.Q.,), and was underweight. The patient experienced episodes of high fever, was intolerant to heat, and did not sweat. He exhibited smooth and dry skin, sparse light-colored eyebrows. Dental clinicians can be the first to diagnose ectodermal dysplasia due to the absence of teeth.
遗传性疾病外胚层发育不良的特征是两个或更多外胚层衍生结构的缺失或缺陷。最常见的外胚层发育不良形式是多汗型和少汗型;区分基于汗腺的有无。本文介绍了一例8岁男性儿童,患有少汗型外胚层发育不良,乳牙和恒牙均完全无牙。该儿童身材矮小,智商低,体重不足。患者曾有高热发作,不耐热,不出汗。他表现出皮肤光滑干燥,浅色眉毛稀疏。由于牙齿缺失,牙科临床医生可能是最早诊断出外胚层发育不良的。