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Table 10 Treatment of Chronic Granulomatous Disease

From: Chronic granulomatous disease: a review of the infectious and inflammatory complications

Prophylaxis of Infection  
Antibacterial therapy Trimethoprim-sulfamethoxazole (TMP-SMX) 5 mg/kg/day (based upon the TMP component, maximum dose 320 mg P.O in two divided daily doses) [187]
Antifungal therapy Itraconazole 5 mg/kg [85] (maximum dose 200 mg orally daily)
Immunomodulatory therapy Interferon-gamma (IFN-γ) [85, 137] 50 μg/m2 (subcutaneous) three times a week 1.5 μg/Kg (subcutaneous) three times a week for children <0.5 m2
Management of Infection  
Empirical treatment TMP-SMX/Fluoroquinolone/Antifungal (Voriconazole)
     • Burkholderia, Serratia species: TMP-SMX
     • Nocardia species: TMP-SMX and/or Cabapenem
     • Staphylococcus aureus:TMP-SMX or Vancomycin
     • Fungal infection: Antifungal agent ±Steroid
Liver abscess Surgical excision [111]; IFN γ [108, 120]
Granulocyte Transfusion Unirradiated white blood cells [183, 184]
Definitive treatment  
Stem cell transplant HLA identical sibling umbilical cord stem cell transplantation (UCSCT) after myeloablative conditioning (Stem cell transplantation from a HLA-identical donor may, at present, be the only proven curative approach to CGD) [185187]
Gene therapy Still experimental [188192]