Early dental intervention in patients with X-linked hypohidrotic ectodermal dysplasia
نویسنده
چکیده
Introduction Familiar or syndrome-associated hypo-, oligo or anodontia in children and adolescents demands for professional dental intervention, because poor dental function and aesthetics often result in psychosocial deprivation. Treatment challenges in these patients are to develop functionally and aesthetically acceptable intermediate solutions that strive for tooth and bone preservation and allow for dental growth.
منابع مشابه
Autosomal Recessive Hypohidrotic Ectodermal Dysplasia Caused by a Novel Mutation in EDAR Gene
Backgrounds: Hypohidrotic ectodermal dysplasia (HED) is a rare genetic disorder, distinguished by hypotrichosis, hypohidrosis, and hypodontia. HDE can be inherited in X-linked recessive manner as a result of mutations in the ectodysplasin A (EDA) gene as well as autosomal dominant and autosomal recessive manners both of them caused by mutations in EDA receptor (EDAR) and EDAR-associated death d...
متن کاملHypohidrotic ectodermal dysplasia: a clinical case with a longitudinal approach.
AIM This paper describes a clinical case of a male with hypohidrotic ectodermal dysplasia submitted to rehabilitation and occlusal dental interventions with follow-up from 3 to 14 years of age. BACKGROUND Due to the severe effects on function and esthetics, the clinical manifestations of ectodermal dysplasia exert a negative impact on quality of life. However, oral rehabilitation in childhood...
متن کاملDental Management of Ectodermal Dysplasia Syndrome at an Early Age: A Case Report
Objectives: Ectodermal dysplasia (ED) is a relatively common sex-linked dermatitis characterized by congenital dysplasia of one or more ectodermal structures and their accessory appendages. Common manifestations include fragile skin and nails, defective teeth and salivary glands, frontal bossing with prominent supra orbital ridges, nasal bridge depression and protuberant lips. Teeth are often f...
متن کاملLung and eye involvement in X-linked hypohidrotic ectodermal dysplasia
Objective X-linked hypohidrotic ectodermal dysplasia (XLHED; ectodysplasin deficiency) has been classically described as affecting hair, sweat glands and dentition. What may be underappreciated is the effect ectodysplasin deficiency has on glands surrounding the airways and eyes and the resulting chronic health issues. In this study, we evaluated respiratory and ocular symptoms in XLHED patients.
متن کاملClinical aspects of X-linked hypohidrotic ectodermal dysplasia.
Boys with X-linked hypohidrotic ectodermal dysplasia and their families were studied. Many suffered severe illness in early childhood and nearly 30% died; many had feeding problems, severe fever, atopic disease, and recurrent respiratory infections. Some infants failed to thrive. We found no consistent common endocrine or immunological abnormality, although, most had abnormal immunoglobulin pro...
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