Familial selective IgA deficiency with circulating anti-IgA antibodies: a distinct group of patients?
Two families were investigated in which the mothers had selective IgA deficiency and circulating class-specific anti-IgA antibodies. Both gave birth to two children who were found to be IgA deficient. Three of these children developed anti-IgA antibodies before puberty. In vitro immunoglobulin production studies performed in the children of both families revealed an IgA B cell defect combined with IgA-specific excessive T suppressor function in all four. The mechanisms by which transplacental passage of maternal anti-IgA antibodies could have interfered with the developing IgA system in the offspring are discussed.
de Laat
P C
PC
Department of Pediatrics, University Hospital Nijmegen, The Netherlands.
Weemaes
C M
CM
Bakkeren
J A
JA
van den Brandt
F C
FC
van Lith
T G
TG
de Graaf
R
R
van Munster
P J
PJ
Stoelinga
G B
GB
D000328:Adult; D000888:Antibodies, Anti-Idiotypic / Q000032:analysis*; D000917:Antibody Formation; D002648:Child; D004406:Dysgammaglobulinemia / Q000097:blood / Q000235:genetics* / Q000276:immunology; D005192:Family Health; D005260:Female; D006801:Humans; D017098:IgA Deficiency; D007070:Immunoglobulin A / Q000032:analysis / Q000276:immunology*
D000888:Antibodies, Anti-Idiotypic; D007070:Immunoglobulin A; C049937:anti-IgA