RT Journal Article
SR Electronic
T1 Clinical manifestations of selective IgA immunodeficiency in childhood
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P1220
VO 42
IS Suppl 57
A1 Borislav Kamenov
A1 Svetlana Kamenov
A1 Aleksanar Kamenov
A1 Hristina Stamenkovic
A1 Tatjana Stankovic
YR 2013
UL http://erj.ersjournals.com/content/42/Suppl_57/P1220.abstract
AB Introduction: Selective IgA deficiency is the most common primary immunodeficiency, with a prevalence of approximately 1/600.Methods: The clinical manifestations of pediatric patients diagnosed as selective IgA immunodeficiency were analyzed, because of clinical heterogeneity in this category of patients.Results: 84 patients with selective IgA immunodeficiency were analyzed with the following clinical manifestations: asymptomatic (13); patients who suffer from frequent respiratory and gastrointestinal infections (28), patients with vitiligo (9), autoimmune hypothyroidism (5), cow milk intolerance (10), allergy (7) rheumatoid arthritis (5), coeliac disease (7). Children with recurrent respiratory infections usually had a defect in antibody responses toward polysaccharides, associated with IgG2 (3) or IgG4 (5) deficiency. It seams that infection susceptibility was not related to the degree of IgA deficiency, but to deficiency of IgG4 and IgG2, as well as to low basal serum concentrations of anti-polysaccharide antibodies. One IgA-deficient patients develop more severe common variable immunodeficiency, which is associated with decreased IgG and IgM production as well as partial T-cell defect. Some children developed serous infections during the follow up. Hyper IgG was observed in the most of the patients with selective IgA immunodeficiency (69) as a compensatory phenomenon or because of disregulation of the oral tolerance in those patients. Increased IgE did not differ from the control group.Conclusion: Children with selective IgA immunodeficiency have high risk of serious infections, inflammatory, autoimmune or other chronic diseases.