Cases reported "Ectodermal Dysplasia"

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1/3. Anhidrotic ectodermal dysplasia (Christ-Seimens-Touraine syndrome)--case report with a review.

    Anhidrotic ectodermal dysplasia commonly transmitted as an x-linked recessive disorder is very rare. The complete syndrome occurs in males and females are carriers. Our patients comprised two sisters who had the complete syndrome. This is much rarer and is reported to be inherited as autosomal recessive disorder, there are only a few case reports of females presenting with a complete syndrome in the literature.
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keywords = x-linked
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2/3. Hidrotic ectodermal dysplasia: study of a large Chinese pedigree.

    Hidrotic ectodermaldysplasia was found, to our knowledge, for the first time in a Chinese family in malaysia, and it affected 15 members in five generations. The disease, which is transmitted as a non-sex-linked autosomal dominant trait, presumably originated from southern china. All 15 members had the typical nail, hair, and skin lesions, and we observed three different types of nail defects. scalp alopeica was more extensive in the female members while keratoderma of the palms and soles was more notable in the male members. The nail and skin lesions also became severer with age. Except for the infectious eczematoid dermatitis present in the propositus, none had other skin or systemic disorders. All were relatively healthy and had normal life expectancies;
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keywords = x-linked
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3/3. Anhidrotic ectodermal dysplasia with transient hypogammaglobulinemia.

    An 8-month-old white boy with anhidrotic ectodermal dysplasia (AED) who was referred to the north carolina Baptist Hospital because of recurrent respiratory infections and hypogammaglobulinemia is presented. His mother had partial expression of AED suggesting x-linked recessive inheritance in this family. She was incidentally given oral glucocorticoids during pregnancy for the treatment of chronic urticaria. The patient's serum immunoglobulins G, A and M were low at 8 months but normal by 15 months of age, and immunologic evaluation failed to show a defect in antibody production or cell-mediated immunity. Although rare, the diagnosis of AED must be considered in infant boys with recurrent fever and respiratory infections. The diagnostic features of the disease may be subtle in young child prior to the eruption of the characteristic peg-shaped teeth.
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keywords = x-linked
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