IADR Abstract Archives

Root Microstructure of Retained Primary Teeth in Hyperimmunoglobulinemia E Syndrome

Background: HyperimmunoglobulinemiaE syndrome (HIES) is a rare autosomal dominant immunodeficiency characterized by elevated IgE levels, dermatitis, cyst-forming pneumonias, and retained primary teeth due to STAT3 mutation. The persistence of Hertwig's epithelial root sheath, influencing cementum development, and cytokine imbalances have been proposed as possible etiologies of primary teeth retention.

Objective: To analyze the microstructure of retained HIES primary teeth and identify possible irregularities in root resorption patterns.

Methods: Scanning electron microscopy was performed on 4 extracted retained primary incisors from 2 HIES patients (2 females, age 7-8 years) with confirmed STAT3 mutations. Root surface and cross-sectional morphologies of HIES teeth were compared to 4 non-retained primary teeth (exfoliated normally or extracted for orthodontics) from 3 unaffected controls (1 male, 2 females, age 6-8 years).

Results: The root surfaces of HIES teeth showed some normal accellular extrinsic fiber cementum but mostly hyperplastic appearing cementum, with minimized resorption lacunae at resorption areas and/or retention of dentinal peritubular matrix, indicative of an altered resorption pattern. This contrasted with the polycyclic resorption lacunae and demineralization patterns seen through the normal cementum of control teeth. Cross-sectionally, intact HIES roots manifested thicker, more irregular cemental layers compared to controls. A macroscopically non-resorbed control root demonstrated microscopic evidence of resorption around its apex, compared with a pristine HIES root apex. Cementicles- cemental developmental anomalies of calcified spherical bodies attached to or embedded within cementum- were a common finding along HIES root surfaces but were not seen in controls.

Conclusion: Microstructural morphological variations were observed on HIES root surfaces. Altered resorption morphology, rougher cementum layer, and cementicles on HIES roots indicate either a more regenerative cementum or less active resorption, and may be directly associated with the delayed or absent deciduation of primary teeth in HIES. These findings provide novel insight into the pathogenesis of this complex syndrome.


Division: IADR/AADR/CADR General Session
Meeting: 2009 IADR/AADR/CADR General Session (Miami, Florida)
Location: Miami, Florida
Year: 2009
Final Presentation ID: 2106
Abstract Category|Abstract Category(s): Mineralized Tissue
Authors
  • Bassim, Carol Walker  ( National Institute of Dental and Craniofacial Research / National Institutes of Health, Bethesda, MD, USA )
  • Hart, Thomas C.  ( National Institute of Dental and Craniofacial Research / National Institutes of Health, Bethesda, MD, USA )
  • Freeman, Alexandra F.  ( National Institute of Allergy and Infectious Diseases / National Institutes of Health, Bethesda, MD, USA )
  • Davis, Joie  ( National Human Genome Research Institute / National Institutes of Health, Bethesda, MD, USA )
  • Holland, Steven M.  ( National Institute of Allergy and Infectious Diseases / National Institutes of Health, Bethesda, MD, USA )
  • Domingo, Demetrio L.  ( National Institute of Dental and Craniofacial Research / National Institutes of Health, Bethesda, MD, USA )
  • SESSION INFORMATION
    Poster Session
    Properties of Enamel and Dentin
    04/03/2009