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Inborn Errors of Immunity

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26 Management Section 8. Immunoglobulin Replacement Recommendation 8.1 ➤ We recommend immunoglobulin replacement therapy (IgRT) for patients with IEI with IgG antibody deficiency. (S-H) Recommendation 8.2 ➤ We recommend that initial dosing of immunoglobulin for replacement therapy be at 400 mg/kg to 600 mg/kg per month, followed by dose adjustment, if necessary. (S-M) Recommendation 8.3 ➤ We recommend the monitoring of serum IgG levels, complete blood cell counts with differential, and serum chemistry panel for patients on immunoglobulin replacement therapy. (S-M) Recommendation 8.4 ➤ We recommend maintaining serum IgG levels at more than 800 mg/dL to improve outcomes. (S-H) Recommendation 8.5 ➤ We recommend that immunoglobulin replacement therapy is indicated as a continuous therapy for IEI. (S-L) Recommendation 8.6 ➤ We recommend that a low or absent IgA level, in the setting of low IgG levels, is not a contraindication for immunoglobulin replacement therapy. (S-M) Recommendation 8.7 ➤ We suggest that the route of immunoglobulin replacement therapy be determined based on patient tolerance or preference. (C-M)

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