Clinical aspects of X-linked hypohidrotic ectodermal dysplasia.
نویسندگان
چکیده
منابع مشابه
Clinical 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...
متن کاملPossible genetic heterogeneity in X linked hypohidrotic ectodermal dysplasia.
Hypohidrotic ectodermal dysplasia has been mapped to Xq11-q13 by linkage studies and by a translocation in a manifesting female. We report a family with hypohidrotic ectodermal dysplasia in which the disease did not segregate with this region of the X chromosome as expected. Ten DNA probes which are localised between Xp11 and Xq22 were used in the investigation. The difficulties in diagnosing t...
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 کاملHypohidrotic 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...
متن کاملHypohidrotic 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: Archives of Disease in Childhood
سال: 1987
ISSN: 0003-9888,1468-2044
DOI: 10.1136/adc.62.10.989