Otologic Manifestations of Ectodermal Dysplasia
نویسندگان
چکیده
منابع مشابه
Otologic manifestations of ectodermal dysplasia.
OBJECTIVES To determine the range and prevalence of otologic disorders in patients with ectodermal dysplasia (ED) and provide a general review of its multiple otolaryngological manifestations. DESIGN Case series. SETTING Ectodermal dysplasia family conference. PATIENTS Sixty-nine individuals with ED were evaluated. The average age was 11 years (range, 1-56 years). Most were male patients ...
متن کاملHeterozygote manifestations in X-chromosomal ectodermal dysplasia
About 60-70 % of the heterozygotes of X-linked hypohidrotic ectodermal dysplasia (XLHED) show some clinical manifestations of the disease. Dental abnormalities are key diagnostic features and can be best evaluated at a young age. Compared to controls, carriers have a significantly higher frequency of agenesis of permanent teeth with persistence of deciduous teeth, small teeth resulting in gaps ...
متن کاملOphthalmic manifestations in patients with ectodermal dysplasia syndromes.
BACKGROUND Ectodermal dysplasia (ED) is a disorder that results from abnormal formation of at least two of the four major ectodermal derivatives in the developing embryo. The ectoderm of the embryo forms the skin, teeth, hair and nails, sweat glands and part of the eyes. OBJECTIVES The aim of this article is to reveal ophthalmologic symptoms and signs as multidisciplinary, reliable criteria f...
متن کاملPathogenesis of ectodermal dysplasia
Ectodermal dysplasias (ED) are characterized by impaired development of organs forming from the embryonic surface ectoderm. Thus, in ED organs like teeth, hair, nails and exocrine glands are hypoplastic or totally missing. The pathogenesis of the defects is starting to be understood thanks to the identification of the responsible gene mutations, and to the advances in developmental biology. Rap...
متن کاملCongenital Ectodermal Dysplasia
Two cases of phenotypically similar ectodermal dysplasia characterised by reduction of hair, diminished sweat pores, missing teeth and somewhat cracked and abnormal skin were studied to understand the genetic basis of this disorder with the help of pedigrees. The mode of inheritance in one case was suggested to be x-linked where as in the other case it was autosomal recessive. (JPMA 35 225, 1985).
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ژورنال
عنوان ژورنال: Archives of Otolaryngology–Head & Neck Surgery
سال: 2004
ISSN: 0886-4470
DOI: 10.1001/archotol.130.9.1104