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Hypohidrotic Ectodermal Dysplasia (ED): A Case Series

机译:低渗性皮下发育不良(ED):一个病例系列

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摘要

Ectodermal Dysplasia (ED) is a rare disorder with defects in two or more of the following structures: the teeth and the skin and its appendages including hair, nails, eccrine, and sebaceous glands. Dental manifestations include hypodontia, complete anodontia or malformed teeth. The most common form of the ED syndrome is hypohidrotic ED and is usually inherited as an X-linked recessive trait. Female carriers may have a variable degree of clinical manifestations. The condition is thought to occur in approximately 1 in every 100,000 live births. Dental treatment for these patients varies on an individual basis. Children with ED are often treated dentally with conventional adult appearing prosthesis which are focused only on the oral manifestations of the syndrome. We are here reporting two classical cases of hypohidrotic ED with a review of the literature.
机译:皮肤外胚层发育不良(ED)是一种罕见的疾病,在以下两个或多个结构中存在缺陷:牙齿和皮肤及其附属物,包括头发,指甲,内分泌和皮脂腺。牙齿表现包括牙髓不足,牙齿完全缺损或牙齿畸形。 ED综合征最常见的形式是低汗性ED,通常以X连锁隐性遗传。女性携带者可能具有不同程度的临床表现。据认为,这种情况大约每10万例活产中就有1例发生。这些患者的牙科治疗因人而异。患有ED的儿童通常用常规的成人假体进行牙科治疗,这些假体仅侧重于该综合征的口腔表现。我们在这里报道了两例典型的低渗性ED,并回顾了文献。

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