RESUMO
Aim of the study: to evaluate the incidence of IgA deficiency among children suffering from recurrent infections that were suspected to have a primary immunodeficiency disorder from the Assiut Pediatrics University Hospitals, in a hope that this would be the first step to establish a wider epidemiological study including the whole suspected primary immunodeficiency [PID] patients in the country
Methods: This study included 80 cases suffering from recurrent infections that were suspected to have a PID disorder from the Assiut Pediatrics University Hospitals. And eight apparently normal infants and children, age and sex matched that were enrolled as control in the period from July 2011 to May 2012. All children included in the study were subjected to the initial evaluation of immunocompetence which includes a clinical history and physical examination, and investigated for complete blood picture, ESR, Plain chest X-ray when indicated, Screening for serum level of IgA by Enzyme Linked Immuno-sorbent Assay [ELISA] and Radial Immuno-Diffusion [RID]. Patients with proved IgA deficiency were assayed for IgG and IgM
Results: Out of the 80 patients studied, 4 [5%] patients [group I] had laboratory evidence of IgA deficiency; three of them had IgG and IgM within normal level so they were diagnosed as selective IgA deficiency and one [25%] patient had low IgG and normal IgM level so that was diagnosed as common variable immunodeficiency disorder [CVID]. The mean values of age at onset of symptoms, age at diagnosis and the diagnosis lag were 10, 37.25 and 27.25 respectively
The diagnosis delay ranges from 2 months to 7 years. Parents' consanguinity was evident in our study, since 3/4 [75%] patients of group I were the products of consanguineous parents while the ratio was lesser in group II18/76 [23.7%] patients. The current study showed that patients with severe infections had significantly lower IgA levels, younger age and had earlier onset of recurrent infections than patients with less severe infections. There was no specific sex predilection in patients with IgA deficiency. There were no significant differences in both the total leucocytic, absolute lymphocyte and neutrophil counts or hemoglobin level in patients with selective IgA deficiency when compared to patients with normal IgA levels. The duration of infections was significantly longer in patients with selective IgA deficiency [mean: 11.5 days] when compared to patients with normal IgA levels [mean: 9.18 days]