Inborn Errors of Immunity among Egyptian Children with Recurrent Acute Otitis Media

Objective Our objective was to investigate the relative frequency and pattern of inborn errors of immunity (IEIs) among Egyptian children with recurrent acute otitis media (rAOM). Methods This was a cross-sectional study that included children from the age of 6 months to 16 years with rAOM. Those with structural, functional, and environmental risk factors were excluded. Enrolled children underwent thorough clinical, otorhinolaryngological, and immunological evaluation, including hematological counting, quantitative immunoglobulins assay, lymphocytic flow cytometric immunophenotyping, CH50, and phagocytic function tests. Results The study included 69 children with rAOM (44 boys and 25 girls; median age 30 months). IEIs were identified in 14 children, including transient hypogammaglobulinemia (three cases), selective IgA deficiency (three cases), agammaglobulinemia (two cases), common variable immunodeficiency (two cases), and one case for each of congenital neutropenia, Chediak-Higashi syndrome, hyper IgM syndrome, and Griscelli syndrome. Parental consanguinity and history of unexplained/infection-related siblings' deaths were significantly associated with IEIs (p = 0.018 and 0.003, respectively). AOM and related complications were more frequent among IEI cases (p = 0.018 and 0.032, respectively). IEI cases had lower levels of hemoglobin (10.7 +/- 2.80 vs. 12.3 +/- 1.64 gicIL; p = 0.002), IgG (203 [78-1,370J vs. 708 [42.3-1,509] mg/dl; p = 0.000), and IgA (24.3 [3-310] vs. 80 [15.6-305] mg/dl; p = 0.009) compared with non-IEI cases. Conclusion The current study identified IEIs in one-fifth of children with rAOM, most of which were predominately antibody deficiencies. An immunological workup for rAOM is particularly important in the presence of certain indicators for IEIs, provided that other more common risk factors are excluded.