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Inborn Errors of Immunity

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11 Recommendation 4.4 ➤ We recommend the diagnosis of selective IgA deficiency (SIGAD) for patients older than 4 years of age with serum IgA level below the limit of detection and normal serum IgG and IgM levels. (S-H) Recommendation 4.5 ➤ We suggest the diagnosis of IgG subclass deficiency for patients with recurrent infections and low levels of 1 or more serum IgG subclass levels (IgG1, IgG2, or IgG3 excluding IgG4) and normal serum total IgG levels. (C-M) Recommendation 4.6 ➤ We suggest the diagnosis of specific antibody deficiency (SAD) to polysaccharides for patients with recurrent respiratory infections and impaired antibody responses to polysaccharides and normal serum total IgG levels. (C-M) Recommendation 4.7 ➤ We suggest the diagnosis of SAD to protein antigen for patients with recurrent infections and impaired antibody responses to protein antigen immunizations and normal serum total IgG levels. (C-L) Recommendation 4.8 ➤ We recommend that patients with low serum IgG and IgA levels and normal or elevated serum IgM level be given the diagnosis of immunoglobulin class-switch defects after ruling out combined immunodeficiencies that present with similar laboratory findings. (S-H) Recommendation 4.9 ➤ We recommend the diagnosis of transient hypogammaglobulinemia of infancy (THI) for infants and children with low serum IgG level and normal antibody response to immunizations and absent evidence of secondary causes. (S-H) Recommendation 4.10 ➤ We suggest the diagnosis of unspecified primary hypogammaglobulinemia for patients with recurrent infections and low serum IgG level and normal cellular immunity and absent evidence of secondary causes of low IgG levels and not fulfilling diagnostic criteria for the above-mentioned antibody deficiency disorders. (C-M)

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