• Title/Summary/Keyword: Hypohidrotic Ectodermal Dysplasia(HED)

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Treatment including orthognathic surgery of a patient with Hypohidrotic ectodermal dysplasia with cleft palate: A Clinical report (구개열을동반한저한성외배엽형성이상환자의 악교정수술을포함한치료: A Clinical report)

  • Kim, Jwa-Young;Park, In-Young;Song, Yun-Jung
    • The Journal of the Korean dental association
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    • v.57 no.2
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    • pp.93-99
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    • 2019
  • Ectodermal dysplasia is a genetic disorder in which various clinical manifestations involve two or more of the differentiated tissues of the ectoderm. Facial deformity, which is frequently associated with ectodermal dysplasia, appears in the form of cleft lip or cleft palate, especially in the middle facial area.Cleft and tooth defects result in decreased alveolar bone development.This leads to severe skeletal incongruity. Facial features include frontal protrusion, malar bone hypoplasia, flat nose, mandibular prominence and long lower facial height. This clinical report presents treatment including orthognathic surgery of a patient with Hypohidrotic Ectodermal dysplasia with cleft palate.

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DENTAL MANAGEMENT OF ECTODERMAL DYSPLASIA : A CASE REPORT (외배엽 이형성증 환자의 치험례)

  • Jang, Hyang-Gil;Lee, Sang-Ho;Lee, Nan-Young
    • Journal of the korean academy of Pediatric Dentistry
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    • v.36 no.4
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    • pp.631-639
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    • 2009
  • Ectodermal dysplasia is a genetic disease which shows various congenital dysplasias in tissues differentiated from the ectoderm. As the most common type of the ectodermal dysplasia, hypohidrotic ectodermal dysplasia(HED) shows dysplasia mainly in the hair, fingernails, teeth and the skin. Symptoms are more severe in males than in females and heterozygous females are usually normal showing no symptom. The treatment for these patients differ according to individuals, but since patients can easily become depressed socially and emotionally due to a decrease in mastication and speech function caused by multiple loss of teeth as well as some aesthetic problems, an early treatment is required. In a case, with a 10 years-old boy diagnosed with HED which shows partial edentia of the maxilla, and the edentia of the mandible in the pediatric dentistry department of the Chosun University Dental Hospital, a fabrication of denture resulted in the recovery of mastication and speech function and aesthetic improvement due to an increase of the face height.

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Prosthetic Treatment Strategies for Improving Denture Retention in Pediatric Patients with Hypohidrotic Ectodermal Dysplasia: a Report of Two Cases (저한성 외배엽 이형성증을 가진 소아 환자에서 의치 유지력 향상을 위한 보철 치료 전략: 두 가지 증례 보고)

  • Hongju Jeon;Seonmi Kim;Namki Choi
    • Journal of the korean academy of Pediatric Dentistry
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    • v.50 no.4
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    • pp.483-494
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    • 2023
  • This report presents two cases concerning strategies for improving denture retention in pediatric patients with oligodontia caused by hypohidrotic ectodermal dysplasia (HED). Both patients presented with multiple missing teeth, conical canines, alveolar bone atrophy, and a skeletal Class III tendency. In the first case, a modified form of conical-crown-retained denture was used to cover the canines. This approach was carried out without tooth extraction or coping. In the second case of severe alveolar bone resorption and a distally tilted lower left canine, the tooth was restored with a hybrid ceramic crown. It was subsequently converted into a clasp-retained removable partial denture, utilizing a suction mechanism. Both patients are currently receiving regular check-ups for the maintenance of their prosthetic appliances and the evaluation of their growth patterns. This study presents innovative prosthetic treatment methods for pediatric patients with HED who have inadequate denture retention.