Ectodermal dysplasia (ED) syndrome

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Ectodermal dysplasia (ED) syndrome

Ectodermal dysplasia (ED) syndrome comprises a large, heterogeneous group of inherited disorders that are defined by primary defects in the development of 2 or more tissues derived from the embryonic ectoderm. The tissues primarily involved are the skin and its appendages (including hair follicles, eccrine glands, sebaceous glands, nails) and teeth. The clinical features include sparse hair, ab...

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Rapp-Hodgkin ectodermal dysplasia syndrome

Gross R E, Neuhauser E B D. Compression of the trachea by an anomalous innominate artery: an operation for its relief. Am J Dis Child 1948; 75: 570-4. 2 Fearon B, Shortreed R. Tracheobronchial compression by congenital cardiovascular anomalies in children. Syndrome of apnea. Ann Otol Rhinol Laryngol 1963; 72: 949-69. 3 Mustard W T, Bayliss C E, Fearon B, Pelton D, Trusler G A. Tracheal compress...

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Why do experts on ectodermal dysplasia (ED) meet again?

Hypohidrotic ectodermal dysplasia was first described in the mid 19 century by John Thurnam. In 1875, Charles Darwin described the signs and symptoms in a fourgeneration family, “the ten men from Scinde”, explaining X-linked inheritance at the same time when Gregor Mendel mapped the laws of inheritance. The typical clinical expression in boys with X-linked hypohidrotic ED makes this syndrome ea...

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Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome.

The Ectrodactyly-Ectodermal Dysplasia-Clefting syndrome is also known as the EEC syndrome. The word ectrodactyly is derived from the Greek, and means congenital absence of all or part of a finger or toe. While both hands and both feet are usually involved, exceptions have been noted in that one hand may be normal and, rarely, all extremities are normal. Tear ducts are abnormal in most affected ...

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Dental 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...

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ژورنال

عنوان ژورنال: BioMedicine

سال: 2014

ISSN: 2211-8039

DOI: 10.7603/s40681-014-0027-9