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Inborn Errors of Immunity

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28 Management Table 9A. Antibiotic Prophylaxis for Selected IEI a IEI Indication for prophylaxis Drug regimen CGD Severe bacterial infection: pneumonia, skin abscess TMP/SMX (5 mg/kg max dose 160 mg TMP component twice daily) Alternatives: cephalosporins Invasive aspergillosis (lung, bone) Itraconazole 5 mg/kg daily, max daily dose 100 mg (<50 kg ), 200 mg (>50 kg ) All infections IFN-γ 50 μg/m 2 (min 1.5 μg ) subcutaneous injection 3 d/wk SCID, congenital athymia Pneumocystis pneumonia TMP/SMX (5 mg/kg max dose 160 mg TMP component 2–3 times/week Alternatives: pentamidine, atovaquone, dapsone Candidiasis, fungal infection Fluconazole 3 mg/kg Prevention/ treatment of CMV viremia, pneumonia, hepatitis, central nervous system (CNS) infection Acyclovir 12.5 mg/kg/day Ganciclovir Valganciclovir 16 mg/kg/day Treat until infant is CMV PCR negative weekly × 4 Antibody deficiencies Respiratory infections Azithromycin (5 mg/kg/d max of 250 mg daily or 500 mg 3d/wk) Alternatives: Amoxicillin TMP/SMX Terminal complement defects or asplenia Sepsis, meningitis with encapsulated bacteria Amoxicillin or penicillin daily or through 5y of age STAT3/IL-6– related HIES Bacterial infection TMP/SMX dose 5–6 mg/kg/d of TMP component (divided BID) Chronic mucocutaneous infection Fluconazole (if not on itraconazole or other triazoles) 5 mg/kg Prophylaxis against aspergilloma Itraconazole 5 mg/kg daily, (max 100 mg/d <50 kg, 200 mg/d >50 kg ) Treat if pneumatocele present

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