A boy with recurrent infections, impaired PMN-chemotaxis, increased IgE concentrations and cranial synostosis--a variant of the hyper-IgE syndrome?
A patient with coarse facies, craniosynostosis, recurrent staphylococcal infections with pneumatocele formation is described. Laboratory features included moderately elevated serum IgE, cutaneous anergy, decreased numbers of T-suppressor cells and variable inhibition of neutrophil chemotaxis. The combination of clinical findings suggests the diagnosis hyper-IgE syndrome, though the total IgE serum concentration (800 U/ml) and the level of IgE-specific antibodies to staphylococci (9.4%, normal less than 5%) were only slightly elevated.
Universitäts-Kinderklinik Göttingen, FRG.
M
M
Gahr
W
W
Müller
B
B
Allgeier
C P
CP
Speer
D002634:Chemotaxis, Leukocyte; D002648:Child; D003398:Craniosynostoses / Q000150:complications* / Q000000981:diagnostic imaging; D005149:Facial Expression; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007073:Immunoglobulin E; D008297:Male; D009504:Neutrophils; D011023:Pneumonia, Staphylococcal / Q000150:complications* / Q000000981:diagnostic imaging; D011859:Radiography; D012008:Recurrence; D013211:Staphylococcus aureus; D013577:Syndrome
D007073:Immunoglobulin E