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Answer 3
  1. SCID. Patients with SCID are unable to produce functional antibodies. Although the basic defect in SCID is T-cell dysfunction, B cells are unable to produce functional antibody due to lack of T-cell help. Therefore, routine IVIg replacement therapy is indicated until more definitive therapy (eg, bone marrow transplantation) can be performed. Isolated IgG2 subclass deficiency without evidence of functional antibody production deficiency is not an indication for IVIg therapy. The benefit of IVIg in reduction of serious bacterial infection in HIV-infected persons is apparent only in children who do not receive trimethoprim-sulfamethoxazole (TMP-SMX)as prophylaxis for opportunistic infections. IVIg therapy in these patients should be restricted to those who develop recurrent infections despite combination antiviral therapy and prophylactic TMP-SMX.

     Bonilla FA, Geha RS. 12. Primary immunodeficiency diseases [published erratum appears in J Allergy Clin Immunol 2003;112:267]. J Allergy Clin Immunol 2003;111(2 Suppl):S571-81.

    2. Mouthon L, Lortholary O. Intravenous immunoglobulins in infectious diseases: where do we stand? Clin Microbiol Infect 2003;9:333-8.

    3. Tangsinmankong N, Bahna SL, Good RA. The immunologic workup of the child suspected of immunodeficiency. Ann Allergy Asthma Immunol 2001;87:362-9.

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