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Tooth eruption, epithelial root sheath and craniofacial profile in hyper-IgE syndrome: report of two cases
Pubblication date: /4/2001
Authors: K.B. Becktor, I. Kjær, C. Koch
Institution: Department of Orthodontics, School of Dentistry, University of Copenhagen, Denmark
and Department of Paediatrics GGK, Clinic for Cystic Fibrosis, The Juliane Marie Centre, National University Hospital ("Rigshospitalet"), Denmark.
Publication: European Journal of Paediatric Dentistry
Title: Tooth eruption, epithelial root sheath and craniofacial profile in hyper-IgE syndrome: report of two cases
Abstract: Patients with hyper-IgE syndrome (Job Syndrome) have elevated serum IgE values and a characteristic face. Problems with retention of primary teeth have been reported, and these may be associated with the persistence of Hertwig’s epithelial root sheath. Aim The purpose of the present study was to analyse the dentition and the craniofacial morphology of two adolescent patients (one female, one male) with hyper-IgE syndrome. Methods Radiographs of the dentition and the craniofacial profile were analysed. Histochemical analysis using cytokeratin KWS was conducted on sections from primary molars extracted from both patients. Results The case reports demonstrated a persistence of an epithelial root sheath on the primary molars and retention of the primary dentition. Furthermore, the craniofacial profile was shown to have normal values in both patients.