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Retained primary teeth in STAT3 hyper-IgE syndrome: Early intervention in childhood is essential.
Orphanet J. Rare Dis. 15:244 (2020)
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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Publication type
Article: Journal article
Document type
Scientific Article
Keywords
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
Quellenangaben
Volume: 15,
Issue: 1,
Article Number: 244
Publisher
BioMed Central
Publishing Place
London
Non-patent literature
Publications
Reviewing status
Peer reviewed
Institute(s)
Institute of Environmental Medicine (IEM)
Grants
FritzBender foundation
Wilhelm-Sander foundation
German Research Foundation
Wilhelm-Sander foundation
German Research Foundation