Abstract
Chronic rhinosinusitis and other sinopulmonary infections can be a hallmark of impaired immunity in children. Rhinosinusitis in the setting of immunodeficiency can often be chronic and refractory, and involve opportunistic pathogens. We review the evaluation and management of pediatric chronic rhinosinusitis in the context of primary immunodeficiency diseases. Immunodeficiencies found to be associated with chronic rhinosinusitis include common variable immune deficiency, X-linked agammaglobulinemia, antibody deficiencies, hyper-IgM syndrome, hyper-IgE syndrome, Wiskott–Aldrich syndrome, activated PI3K disease, DiGeorge syndrome, and chronic granulomatous disease. Chronic rhinosinusitis in children may be a clinical manifestation of immunodeficiency. The evaluation and diagnosis of immunodeficiencies are essential for initiating appropriate therapeutic strategies, including antibiotics, prophylaxis, topical therapy, surgery, and immunoglobulin replacement.
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