期刊导航

论文摘要

无汗型外胚叶发育不全1 例

A case report of ectodermal dysplasia anhidrotic

作者:黄玉辉 赖文莉 王璟

Author:Huang Yuhui, Lai Wenli, Wang Jing.

收稿日期:2010-06-21          年卷(期)页码:2011,38(6):659-661

期刊名称:国际口腔医学杂志

Journal Name:International Journal of Stomatology

关键字:先天无牙症,无汗型外胚叶发育不全,口腔表现,

Key words:congenitally total anodontia,ectodermal dysplasia anhidrotic,oral manifestation,

基金项目:

中文摘要

先天性无牙症是先天完全无牙或大多数牙齿先天缺失,常是外胚叶发育不全综合征的一种表现。外胚叶发育不全综合征是一类遗传性疾病,表现为牙齿先天缺失、毛发稀疏和皮肤异常等多种症状。本病分为2 类:一类为无汗型外胚叶发育不全(EDA)或称少汗型外胚叶发育不全,另一类为有汗型外胚叶发育不全。除了牙齿、毛发、皮肤等结构异常外,EDA 患者皮肤无汗腺或少汗腺,故其体温调节有障碍;有汗型患者则汗腺正常。

英文摘要

Congenitally total anodontia is congenital absence of all or most teeth, and also is a performance of ectodermal dysplasia syndrome. It is a kind of genetic disease, which represents complex group of diseases characterized by various defects in teeth, hair, skin, and so on, which is divided into two categories as ectodermal dysplasia anhidrotic(EDA) or called hypohidrotic ectodermal dysplasia and hidrotic ectodermal dysplasia. In addition to teeth, hair, skin and other structural abnormalities, patients of EDA are lack of sweat glands, who can not regulate body temperature, hidrotic type have normal sweat glands.

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