Montanari Marco, Callea Michele, Battelli Filippo, Piana Gabriela
Unit of Dentistry for Disables Department of Oral Science, Alma Mater Studiorum, University of Bologna, Bologna, Italy.
BMJ Case Rep. 2012 Jun 21;2012:bcr0120125652. doi: 10.1136/bcr.01.2012.5652.
The aim of this study was to describe the clinical treatment of young patients, affected by ectodermal dysplasia (ED), and to possibly establish clinical guidelines. The study design was case series. ED syndromes (EDs) are a heterogeneous group of inherited diseases characterised by abnormal development of tissues of ectodermal origin. The most common form of EDs is X linked hypohidrotic ED (HED). Characteristic triad of HED is oligo-anodontia, hypotricosis, hypo-anhydrosis. Oligo-anodontia is one of the most severe impairment, since it affects chewing, swallowing, speech, esthetics and social relation. Early prosthetic rehabilitation (at 2-3 years of age), with partial or complete dentures, is essential to improve oral function and reduce the social impairment.
本研究的目的是描述受外胚层发育不全(ED)影响的年轻患者的临床治疗情况,并可能制定临床指南。研究设计为病例系列。ED综合征(EDs)是一组遗传性疾病,其特征是外胚层起源组织的异常发育。EDs最常见的形式是X连锁少汗型ED(HED)。HED的特征三联征是少牙或无牙、毛发稀少、少汗。少牙或无牙是最严重的损害之一,因为它会影响咀嚼、吞咽、言语、美观和社会关系。早期(2至3岁)使用局部或全口假牙进行修复康复对于改善口腔功能和减少社会功能障碍至关重要。