Ocular and non-ocular manifestations of hypohidrotic ectodermal dysplasia

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Involvement of the ocular system in hypohidrotic ectodermal dysplasia

X-linked hypohidrotic ectodermal dyplasia (XLHED) is the most common form of ectodermal dysplasia. It often presents with ocular symptoms, already in early childhood. Multiple ophthalmological tests are available, but in early childhood only tests of lower invasiveness can be applied. We have evaluated tear film tests and ocular surface staining as screening methods in pediatric patients with s...

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Hypohidrotic ectodermal dysplasia.

X linked hypohidrotic ectodermal dysplasia was studied in the dentition of both affected males and carrier females. Hypodontia was more severe in males than females and there were differences in the pattern of tooth absence between the sexes. Abnormal crown form, with the maximum diameter of the teeth being apically displaced, was noted particulady in the anterior teeth. Taurodontism was common...

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Hypohidrotic Ectodermal Dysplasia: Prosthetic and Endodontic Management

Individuals affected by ectodermal dysplasia syndromes have abnormalities of the glands, tooth buds, hair follicles, and nail development. Oral finding in ectodermal dysplasia patient are significant and can include multiple abnormalities of the dentition such as anodontia, hy-podontia or malformed and widely spaced peg like teeth, loss of occlusal vertical dimension, protuberant lips and lack ...

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Early recognition of hypohidrotic ectodermal dysplasia

Hypohidrotic ectodermal dysplasia (HED) is characterized by severe hypohidrosis, hypoplasia of sweat, sebaceous, submucous, meibomian and mammary glands, hypotrichosis, and oligodontia. In early childhood, HED is a life-threatening disorder based on the risks for hyperthermia and pneumonia. Awareness of the hazards related to this rare genetic disease is most helpful in preventing avoidable cal...

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Hypohidrotic ectodermal dysplasia: clinical and radiographic characteristics

www.medigraphic.org.mx * Specialist in Endodontics and Dental Radiology and Imaging, Hospital for the Rehabilitation of Craniofacial Anomalies of the University of Sao Paulo. Professor in Applied Dental Sciences, Bauru School of Dentistry, University of Sao Paulo, Brazil. § PhD in Oral Pathology. Bauru School of Dentistry, University of Sao Paulo, Brazil. Teacher at the Pediatric Dentistry Depa...

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ژورنال

عنوان ژورنال: Case Reports

سال: 2011

ISSN: 1757-790X

DOI: 10.1136/bcr.01.2011.3731