Craniosynostosis in hyper-IgE-syndrome |
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| Authors: | P. H. Höger E. Boltshauser W. H. Hitzig |
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| Affiliation: | (1) Department of Pediatrics, Divisions of Immunology/Hematology and Neuropediatrics, University of Zürich, Zürich, Switzerland;(2) Present address: Universitäts-Kinderklinik, Josef-Schneider-Str. 2, D-8700 Würzburg, Federal Republic of Germany |
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| Abstract: | A 9-year-old boy with hyperimmunoglobulin-E-syndrome (HIE) and craniosynostosis is reported. Premature fusion of the sagittal and lambdoid suture led to scaphocephaly. A partial optic atrophy without clinical signs of raised intracranial pressure was observed. This is the fourth reported case of craniosynostosis in HIE. Bone anomalies like osteoporosis are frequent findings in HIE. Apart from their clinical impact they could be related to factors involved in the pathogenesis of HIE, such as impairment of chemotaxis in tissues or monocyte differentiation.Abbreviation HIE hyperimmunoglobulin-E-syndrome |
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| Keywords: | Hyperimmunoglobulin-E-syndrome Craniosynostosis Scaphocephaly Partial optic atrophy Bone anomalies |
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