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001-es BibID:	BIBFORM116713
035-os BibID:	(Scopus)85175040518 (WOS)001091026700006
Első szerző:	Tar Ildikó (fogszakorvos)
Cím:	Intraoral and maxillofacial abnormalities in patients with autosomal dominant hyper-IgE syndrome / Ildikó Tar, Márta Szegedi, Ewa Krasuska-Sławińska, Edyta Heropolitańska-Pliszka, Ewa A. Bernatowska, Elif Öncü, Sevgi Keles, Sukru N. Guner, Ismail Reisli, Nevena Gesheva, Elissaveta Naumova, Lydie Izakovicova-Holla, Jiri Litzman, Igor Savchak, Larysa Kostyuchenko, Melinda Erdős
Dátum:	2023
ISSN:	1426-3912
Megjegyzések:	Autosomal dominant hyper-IgE syndrome (AD-HIES) is an inborn error of immunity (IEI) caused by a dominant-negative mutation in the signal transducer and activator of transcription 3 (STAT 3). This disease is characterized by chronic eczematoid dermatitis, recurrent staphylococcal skin abscesses, pneumonia, pneumatoceles, and extremely high serum IgE levels. Loss-of-function STAT3 mutations may also result in distinct non-immunologic features such as dental, facial, skeletal, and vascular abnormalities, central nervous system malformations and an increased risk for bone fractures. Prophylactic treatment of Candida infections and prophylactic antimicrobial therapy for staphylococcal skin infections and sinopulmonary infections are essential. An awareness of the oral and maxillofacial features of HIES may facilitate early diagnosis with genetic counselling and may improve future patient care. This study describes oral, dental, and maxillofacial manifestations in 14 patients with genetically defined AD-HIES. We also review the literature and propose recommendations for the complex care of patients with this rare primary immunodeficiency.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk dentist hyper-IgE syndrome intraoral maxillofacial
Megjelenés:	Central European Journal of Immunology. - 48 : 3 (2023), p. 228-236. -
További szerzők:	Szegedi Márta Krasuska-Sławińska, Ewa Heropolitanska-Pliszka, Edyta Bernatowska, Ewa Öncü, Elif Keles, Sevgi Guner, Sukru Nail Reisli, Ismail Gesheva, Nevena Naumova, Elissaveta Izakovicova-Holla, Lydie Litzman, Jiri Savchak, Igor Kostyuchenko, Larysa Erdős Melinda (1975-) (infektológus, gyermekimmunológus)
Internet cím:	Szerző által megadott URL DOI Intézményi repozitóriumban (DEA) tárolt változat
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