Title
Author
DOI
Article Type
Special Issue
Volume
Issue
Hypohidrotic ectodermal dysplasia: dental, clinical, genetic and dermatoglyphic findings of three cases
1Department of Pediatric Dentistry, Dental School, Marmara University, İstanbul, Turkey
2Department of Orthodontics, Dental School, Marmara University, İstanbul, Turkey
3Department of Genetics, Dental School, Marmara University, İstanbul, Turkey
DOI: 10.17796/jcpd.26.1.g156r02u66w30729 Vol.26,Issue 1,January 2002 pp.5-12
Published: 01 January 2002
*Corresponding Author(s): B. Kargül E-mail: b.kargul.ped@marun.edu.tr
Patients with hypohidrotic ectodermal dysplasia (HED) are characterized by the clinical manifestations of hypodontia, hypohidrosis, hypotrichosis and a highly characteristic facial physiognomy.This disorder is inherited as an X- linked trait. This report presents three cases with HED in which the clinical evalua-tion (intraoral and radiological), genetic findings and SEM examination of hair. Boys 6 to 14 year old and a 11 year old girl were referred to the Marmara University, Faculty of Dentistry, complaining of oligodontia in the maxillary and mandibular arches and delay in eruption of other teeth. Peg-shaped teeth have been observed. The dermatoglyphs of the patients were striking. SEM exmimation of hair demostrated a distinctly abnormal longitudinal groving along the entire length of each hair and a desqua-mation of the surface cuticles. The treatment was planned in a multidisciplinary odontological group involving pediatric dentistry, orthodontics, prosthodontics and oral surgery and maxillofacial radiology of future dental habilitation. A specially designed overdenture, a removable prosthesis and osseointe-grated implants were constructed. Periodic recall visits were advised, to monitor the dentures and implants during periods of growth and development, and eruption of the permanent teeth.
B. Kargül,T. Alcan,U. Kabalay,M. Atasu. Hypohidrotic ectodermal dysplasia: dental, clinical, genetic and dermatoglyphic findings of three cases. Journal of Clinical Pediatric Dentistry. 2002. 26(1);5-12.
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