دراسة متلازمة عوز الغلوبولين المناعي A الانتقائي عند الاطفال في مستشفى الاطفال الجامعي

Background& Objective: Selective IgA deficiency is the most common primary immunodeficiency in humans. Its prevalence ranges from 1 in 100 to 1 in 1000 in among Arab populations. The majority of patients with selective IgA deficiency are asymptomatic. The remaining present with recurrent upper and lower respiratory tract infections, recurrent gastrointestinal infections, otitis media, sinusitis, celiac disease, giardiasis, allergic disorders and some autoimmune diseases. to highlight the clinical manifestations and laboratory findings of Selective IgA deficiency in our study, and to compare it with medical literature. To study the bacterial complications and their prevalence. Materials & Methods: A retrospective study was carried out in Damascus University Children’s Hospital on sixteen child diagnosed as Selective IgA deficiency, in a period of the last ten years (from 2000 till September 2010). Results: most cases in our study were among males, failure to thrive was found in 75%, hepatosplenomegaly in one third, the most prominent infectious complications were: recurrent pneumonia (in 87.5% of cases), recurrent GI infections (in 75% of cases) and UTI’s in (50%)of cases. On the other hand, the celiac disease was found in (25%) of our patients, which is much higher than described in medical literature. In general, the infectious complications had a good response to antibacterial therapy, and the IgA levels became normal in one patient, when he reached the age of eleven years. Conclusion: there is no information in medical literature about the association of selective IgA deficiency with hepatomegaly, splenomegaly, failure to thrive and an increased prevalence of urinary tract infections, therefore, it is necessary to conduct researches about this association.