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少汗型外胚层发育不良

Hypohidrotic ectodermal dysplasia (HED).

作者信息

Ahmed Bilal, Yazdanie Nazia

机构信息

Department of Prosthodontics, de'Montmorency Institute of Dental Sciences, Lahore, Pakistan.

出版信息

J Coll Physicians Surg Pak. 2006 Jan;16(1):61-3.

Abstract

Hypohidrotic Ectodermal Dysplasia (HED) is a hereditary congenital disorder of ectodermal origin. It is characterized by lack of sweat glands (hypohidrosis), nail dystrophy(onychodysplasia), alopecia (hypotrichosis), defective palms and soles (palmoplantar hyperkeratosis) and the oral presentations of partial absence of teeth (hypodontia) or complete absence of teeth (anodontia). Hypodontia of primary and permanent dentition is one of the most frequently occurring oral symptoms in HED patients. These features of poor aesthetic affect the social and the psychological well-being of the patient. This case report describes the prosthetic rehabilitation of a HED patient.

摘要

少汗型外胚层发育不良(HED)是一种源于外胚层的遗传性先天性疾病。其特征为汗腺缺乏(少汗症)、指甲发育不良(甲发育异常)、脱发(毛发稀少)、手掌和足底有缺陷(掌跖角化过度)以及口腔表现为部分缺牙(少牙症)或完全无牙(无牙症)。乳牙列和恒牙列的少牙症是HED患者最常见的口腔症状之一。这些美观性差的特征会影响患者的社交和心理健康。本病例报告描述了一名HED患者的修复治疗。

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