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Most patients are asymptomatic
Patients are most susceptible to infections with encapsulated bacteria (eg, Haemophilus influenzae, Streptococcus pneumoniae, Neisseria meningitides)
A minority of patients have frequent and recurrent sinusitis, otitis, bronchitis, or gastrointestinal infections
Can be associated with atopic diseases and autoimmune disorders, including Graves disease, systemic lupus erythematosus, juvenile rheumatoid arthritis, type 1 diabetes mellitus, and celiac disease
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Characterized by undetectable serum IgA levels (< 7 mg/dL) with normal levels of IgG and IgM
Some patients with undetectable levels of serum IgA may have high titers of anti-IgA, IgG, or IgE antibodies and are at risk for anaphylactic reactions to IgA following exposure to it through infusions of plasma (or blood transfusions)
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Treatment with commercial immune globulin replacement therapy is not indicated and may result in anaphylaxis from preparations that contain detectable amounts of IgA
Antibiotics as needed for infections