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.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Abbreviations
- HIE:
-
hyperimmunoglobulin-E-syndrome
References
Brestel EP, Klingberg WG, Veltri RW, Dorn JS (1982) Osteogenesis imperfecta tarda in a child with hyper-IgE-syndrome. Am J Dis Child 136:774–776
Buckley RH, Sampson HA (1981) The hyperimmunoglobulinemia E syndrome. In: Franklin ED (ed) Clinical immunology update. Churchill Livingstone, Edinburg, pp 147–167
Buckley RH, Wray BB, Belmaker EZ (1972) Extreme hyperimmunoglobulinemia E and undue susceptibility to infection. Pediatrics 49:59–70
Cohen MM (1979) Craniosynostosis and syndromes with craniosynostosis. Birth Defects 15 (5 B):13–63
David JD, Poswillo D, Simpson D (1982) The craniosynostoses. Causes, natural history, and management. Springer Verlag, Berlin Heidelberg New York
Donabedian H, Gallin JI (1983) The hyperimmunoglobulin E recurrent infection (Job's) syndrome. A review of the NIH experience and the literature. Medicine 62:195–208
Lallemand D, Kalifa G, Buriot D, Sauvegrain J, Griscelli C (1979) Anomalies osseuses constitutionelles dans les déficits immunitaires congénitaux. Ann Radiol 22:108–118
McCallum JE, Brand E, Selker RG (1974) Osteomyelitis of the skull in chronic granulomatous disease of childhood. J Neurosurg 40:764–766
Nelson RD, Quie PG, Simmons RL (1975) Chemotaxis under agarose: A new and simle method for measuring chemotaxis and spontaneous migration of human polymorphonuclear leukocytes and monocytes. J Immunology 115:1650–1656
Prader A, Budliger H (1977) Body measurements, growth velocity and bone age of healthy children up to 12 years of age (longitudinal growth study Zürich). Helv Paediatr Acta [Suppl] 37:1–44
Smithwick EM, Finelt M, Pahwa S, Good RA, Naspitz CK, Mendes NF, Kopersztyck S, Spira TJ, Nahmias AJ (1978) Cranial synostosis in Job's syndrome (Letter). Lancet I:826
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Höger, P.H., Boltshauser, E. & Hitzig, W.H. Craniosynostosis in hyper-IgE-syndrome. Eur J Pediatr 144, 414–417 (1985). https://doi.org/10.1007/BF00441793
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00441793