Retained primary teeth in STAT3 hyper-IgE syndrome: early intervention in childhood is essential
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(2020) 15:244
RESEARCH
Open Access
Retained primary teeth in STAT3 hyper-IgE syndrome: early intervention in childhood is essential Iris Meixner1,2†, Beate Hagl1,3†, Carolin I. Kröner1, Benedikt D. Spielberger1, Ekaterini Paschos4, Gregor Dückers5, Tim Niehues5, Ronny Hesse2† and Ellen D. Renner3*†
Abstract 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. Keywords: Retained primary teeth, Delayed eruption of permanent teeth, Root resorption, Hyper-IgE syndrome, HIES, STAT3, STAT3-HIES, Primary immunodeficiency, PID
Background STAT3 hyper-IgE syndrome is a rare primary immunodeficiency associated with eczema, recurrent infections, and high serum IgE levels [1, 2]. Due to the clinical overlap with atopic dermatitis, diagnosis is often delayed * Correspondence: [email protected] † Iris Meixner and Beate Hagl are first co-authors and Ronny Hesse and Ellen D. Renner are senior authors. They contributed equally to this work. 3 Chair and Institute of Environmental Medicine, UNIKA-T, Technical University of Munich and HelmholtzZentrum München, Munich/Augsburg, Germany Full list of author information is available at the end of the article
until irreversible complications, such as chronic lung disease, already have occurred [3]. Early diagnosis has been improved by identifying heterozygous dominantnegative mutations in the signal transducer and activator of transcription 3 (STAT3) gene in 2007 and corresponding molecular testing [4–6]. The impaired STAT3 signaling in STAT3-HIES leads to defective B cell development and antibody
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