Abstract
We evaluated a 14 month old white male, for recurrent bacterial otitis media and sinusitis. The patient's father had similar infections as a child. The mother was normal. Father and son had normal IgG, IgA, IgM, IgE, IgG1, IgG2 and IgG3. The child had undctoctable IgG4 (normal 0.9-107 mg/dl) and the father had IgG4<5 mg/dl (5-362) as measured by radial immunodiffusion. The family was screened for defects in PMN function measuring chemotaxis activity in response to E.Coli-endotoxin, bacteria killing against S.aureus and superoxide product ion with Cyto B/ FMLP. PMN chemotaxis and bacterial killing of S.aureus were markedly impaired in father and son compared to controls.
Control serum did not correct the chemotaxis and bacterial killing defects. Both O2 was normal. Father is HLA (2,)(15, 14)and son is(2, 31)(15, 7). High resolution chromosomal banding is pending. We conclude that familial IgG4 subclass deficiency with NL IgA can be present in association with defects in PMN chemotaxis and bacterial killing.
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Etlis, M., Dumars, K., Gillman, S. et al. FAMILIAL IgG4 SUBCLASS DEFICIENCY WITH NL IgA ASSOCIATED RECURRENT INFECTIONS WITH DECREASED NEUTROPHIL CHEMOTAXIS AND BACTERIAL KILLING. Pediatr Res 21 (Suppl 4), 311 (1987). https://doi.org/10.1203/00006450-198704010-00863
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DOI: https://doi.org/10.1203/00006450-198704010-00863