| CLINICAL MANAGEMENT OF ECTODERMAL DYSPLASIA : A CASE REPORT |
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| 외배엽 이형성증 환자의 임상적 치험례 |
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오소희1, 권순원1, 김종수1, 김용기1, 임헌송2 |
1단국대학교 치과대학 소아치과학교실
2단국대학교 치과대학 보철학교실 |
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| Abstract |
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Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structures and their accessory appendages. At least 120 subtypes of ectodermal dysplasia have been reported. The disease is usually transmitted as an X-linked recessive trait in which the gene is carried by the female and manifested in the male. Hypohidrotic ectodermal dysplasia is manifested as a triad of defects that include hypohidrosis, hypotrichosis and hypodontia. The characteristic facial features consist of asteatosis, onchodysplasia, sparse and fine blond hair, prominent forehead, a depressed nasal bridge, thick everted lips. The patient may suffer from dry skin, hyperthermia and unexplained high fever as a result of the deficiency of sweat glands. This case report presents detailed procedures of rehabilitating functional and esthetic defect of a 6-year-old boy with hypohidrotic ectodermal dysplasia along with the review of relevant literatures. |
| Key Words:
Ectodermal dysplasia, Hypodontia, Complete denture |
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