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- Title
Novel intraoral phenotypes in hyperimmunoglobulin-E syndrome.
- Authors
Domingo, D L; Freeman, A F; Davis, J; Puck, J M; Tianxia, W; Holland, S M; Hart, T C
- Abstract
Hyperimmunoglobulin-E syndrome (HIES) is a primary immunodeficiency characterized by eczema, recurrent skin and lung infections with pneumatocoele formation, and extremely elevated serum immunoglobulin-E. The precise immunologic defect and genetic etiology remain unknown. Non-immunologic findings include characteristic facial features (prominent forehead, fleshy nasal tip, and increased interalar distance); skeletal involvement (pathological fractures, scoliosis, and craniosynostosis); and retention of primary teeth. This study aims to characterize intraoral soft tissue findings in HIES patients.
- Publication
Oral diseases, 2008, Vol 14, Issue 1, p73
- ISSN
1354-523X
- Publication type
Journal Article
- DOI
10.1111/j.1601-0825.2007.01363.x