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Meixner, I.* ; Hagl, B. ; Kröner, C.I.* ; Spielberger, B.D.* ; Paschos, E.* ; Dückers, G.* ; Niehues, T.* ; Hesse, R.* ; Renner, E.D.

Retained primary teeth in STAT3 hyper-IgE syndrome: Early intervention in childhood is essential.

Orphanet J. Rare Dis. 15:244 (2020)
Verlagsversion Forschungsdaten DOI
Open Access Gold
Creative Commons Lizenzvertrag
Background STAT3 hyper-IgE syndrome (STAT3-HIES) is a rare primary immunodeficiency that clinically overlaps with atopic dermatitis. In addition to eczema, elevated serum-IgE, and recurrent infections, STAT3-HIES patients suffer from characteristic facies, midline defects, and retained primary teeth. To optimize dental management we assessed the development of dentition and the long-term outcomes of dental treatment in 13 molecularly defined STAT3-HIES patients using questionnaires, radiographs, and dental investigations. Results Primary tooth eruption was unremarkable in all STAT3-HIES patients evaluated. Primary tooth exfoliation and permanent tooth eruption was delayed in 83% of patients due to unresorbed tooth roots. A complex orthodontic treatment was needed for one patient receiving delayed extraction of primary molars and canines. Permanent teeth erupted spontaneously in all patients receiving primary teeth extraction of retained primary teeth during average physiologic exfoliation time. Conclusions The association of STAT3-HIES with retained primary teeth is important knowledge for dentists and physicians as timely extraction of retained primary teeth prevents dental complications. To enable spontaneous eruption of permanent teeth in children with STAT3-HIES, we recommend extracting retained primary incisors when the patient is not older than 9 years of age and retained primary canines and molars when the patient is not older than 13 years of age, after having confirmed the presence of the permanent successor teeth by radiograph.
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Publikationstyp Artikel: Journalartikel
Dokumenttyp Wissenschaftlicher Artikel
Korrespondenzautor
Schlagwörter Retained Primary Teeth ; Delayed Eruption Of Permanent Teeth ; Root Resorption ; Hyper-ige Syndrome ; Hies ; Stat3 ; Stat3-hies ; Primary Immunodeficiency ; Pid; Signal Transducer; Key Findings; Mutations; Deficiency; Eruption; Resorption; Activator
ISSN (print) / ISBN 1750-1172
e-ISSN 1750-1172
Zeitschrift Orphanet Journal of Rare Diseases : OJRD
Quellenangaben Band: 15, Heft: 1, Seiten: , Artikelnummer: 244 Supplement: ,
Verlag BioMed Central
Verlagsort London
Nichtpatentliteratur Publikationen
Begutachtungsstatus Peer reviewed
Institut(e) Institute of Environmental Medicine (IEM)
Förderungen FritzBender foundation
Wilhelm-Sander foundation
German Research Foundation