DENTAL TREATMENT APPROACH IN PEDIATRIC PATIENT WITH ECTODERMAL DYSPLASIA : A CASE REPORT

Abstract

Ectodermal dyplasia is a hereditary disease associated with abnormal development of two or more embryonic ectodermal structures such as teeth, nail, hair, nerve cells and sweat glands. According to the state of sweat glands involvement ectodermal dysplasia is divided into two major grups: hydrotic and hypohydrotic/ anhydrotic. As hypohydrotic type is inherited in X- linked mode, hydrotic type is inherited in autosomally dominant. The most common oral manifestation of ectodermal dysplasia is teeth deficiency in primary and/ or permenant dentition. Therefore, during the development of the child, protetic rehabilitations could be necessary. Hydrotic type of ectodermal dysplasia diagnosed case with the typical features of the diseas such as hypodontia and onycodysplasia is presented in this study in term of oral manifestation and dental treatment approach

Keywords

• Hydrotic ectodermal dysplasia, hypodontia, autosomal dominant

EKTODERMAL DİSPLAZİLİ ÇOCUK HASTADA DENTAL TEDAVİ YAKLAŞIMI: BİR OLGU RAPORU

Abstract

Ektodermal displazi diş, tırnak, saç, sinir hücreleri ve ter bezleri gibi embriyonik ektodermal kaynaklı yapıların iki veya daha fazlasında anormal gelişim ile ilişkili kalıtsal bir hastalıktır. Ektodermal displazi ter bezlerinin durumuna göre hidrotik ve anhidrotik/ hipohidrotik olarak ikiye ayrılırlar. Hipohidrotik tip X-bağlı resesif kalıtım gösterirken, hidrotik tip ise otozomal dominant kalıtım gösterir. Ektodermal displazinin en sık rastlanılan ağız bulgusu süt ve daimi dişlerdeki eksikliktir. Bu nedenle, etkilenen hastalara çoğunlukla gelişim yıllarında diş protez tedavileri yapılması önerilmektedir. Bu çalışmada hidrotik tip ektodermal displazi teşhisi konmuş,  hipodonti ve onikodisplazi (displastik tırnak) gibi hastalığın tipik özelliklerini taşıyan bir olgunun ağız içi bulguları ve dental tedavi yaklaşımı sunulmuştur.

Keywords

• Hidrotik Ektodermal displazi, hipodonti, otozomal dominant

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