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Table 3 Strategies for the treatment and management of PIDs.

From: Primary immunodeficiency

  Supportive Definitive
CIDs/SCID Ig replacement therapy (IV or SC)
Antibiotic prophylaxis
Antifungal prophylaxis
Aggressive management of established infections
Infectious precautions when hospitalized
Withhold all live vaccines
BMT
HSCT
Gene therapy a possibility for some SCIDs
B-cell disorders Ig replacement therapy (IV or SC)
Antibiotic prophylaxis
Antifungal prophylaxis depending upon etiology
Hearing assessment
Assessment of pulmonary status and function
Close monitoring for co-morbidities
Gene therapy is a potential future treatment in some patients
Innate disorders Antibiotic prophylaxis
Antifungal prophylaxis
Cytokine replacement (IFNγ) for CGD
Vaccinations (e.g., meningococcal)
Ig replacement is sometimes indicated
BMT, e.g., for CGD
Gene therapy is a potential future treatment
  1. Ig, immunoglobulin; IV: intravenous; SC, subcutaneous; CID, combined immunodeficiency; SCID, severe combined immunodeficiency; IFNγ, interferon-gamma; BMT, bone marrow transplantation; CGD, chronic granulomatous disease; HSCT, hematopoietic stem cell transplantation