Ear nose throat manifestations in hypoidrotic ectodermal dysplasia

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dc.contributor.authorCallea, Michele
dc.contributor.authorTeggi, Roberto
dc.contributor.authorYavuz, Izzet
dc.contributor.authorTadini, Gianluca
dc.contributor.authorPriolo, Manuela
dc.contributor.authorCrovella, Sergio
dc.contributor.authorClarich, Gabriella
dc.date.accessioned2024-04-24T16:14:55Z
dc.date.available2024-04-24T16:14:55Z
dc.date.issued2013
dc.departmentDicle Üniversitesien_US
dc.description.abstractThe ectodermal dysplasias (EDs) are a large and complex group of inherited disorders. In various combinations, they all share anomalies in ectodermal derived structures: hair, teeth, nails and sweat gland function. Clinical overlap is present among EDs. Few causative genes have been identified, to date. Altered gene expression is not limited to the ectoderm but a concomitant effect on developing mesenchymal structures, with modification of ectodermal-mesenchymal signaling, takes place. The two major categories of ED include the hidrotic and hypohidrotic form, the latter more frequent; they differentiate each other for the presence or absence of sweat glands. We report Ear Nose Throat manifestations of ED, linked to the reduction of mucous glands in the nasal fossae with reduced ciliar function, and decrease salivary glands function. Often patients report an increased rate of infections of the upper respiratory tract and of the ear. Nasal obstruction due to the presence of nasal crusting, hearing loss and throat hoarseness are the most represented symptoms. Environmental measures, including a correct air temperature and humidification, is mandatory above all in subjects affected by hypohidrotic form. (C) 2013 Elsevier Ireland Ltd. All rights reserved.en_US
dc.identifier.doi10.1016/j.ijporl.2013.09.004
dc.identifier.endpage1804en_US
dc.identifier.issn0165-5876
dc.identifier.issn1872-8464
dc.identifier.issue11en_US
dc.identifier.pmid24080322
dc.identifier.scopus2-s2.0-84885425197
dc.identifier.scopusqualityQ2
dc.identifier.startpage1801en_US
dc.identifier.urihttps://doi.org/10.1016/j.ijporl.2013.09.004
dc.identifier.urihttps://hdl.handle.net/11468/15510
dc.identifier.volume77en_US
dc.identifier.wosWOS:000326561000002
dc.identifier.wosqualityQ2
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoenen_US
dc.publisherElsevier Ireland Ltden_US
dc.relation.ispartofInternational Journal of Pediatric Otorhinolaryngology
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectEctodermal Dysplasiaen_US
dc.subjectHypohidrosisen_US
dc.subjectChronic Rhinitisen_US
dc.subjectChronic Otitis Mediaen_US
dc.subjectHearing Lossen_US
dc.subjectHypodontiaen_US
dc.titleEar nose throat manifestations in hypoidrotic ectodermal dysplasiaen_US
dc.titleEar nose throat manifestations in hypoidrotic ectodermal dysplasia
dc.typeReview Articleen_US

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