immunology allergic disorders
Chronic Granulomatous Disease (CGD)
Treatment of CGD
Prophylactic antibiotics and usually antifungals Usually interferon gamma For severe infections, granulocyte transfusions Hematopoietic stem cell transplantation Treatment of chronic granulomatous disease is continuous prophylactic antibiotics, particularly sulfamethoxazole/trimethoprim800/160 mg orally twice a day. Oral antifungals are given as primary prophylaxis or are added if fungal infections occur even once; most useful are Itraconazoleorally once a day (100 mg capsule for patients < 13 years or weighing < 50 kg; 200 mg tablet for those ≥ 13 years or weighing > 50 kg) Voriconazoleorally every 12 hours (9 mg/kg oral suspension for patients 2 to 12 years or weighing < 40 kg; 200 mg tablet for those weighing ≥ 40 kg) Posaconazole300 mg delayed-release tablet orally twice a day on day 1, then 300 mg once daily; weight-based dosing for patients weighing < 40 kg Interferon gamma may reduce severity and frequency of infections and is usually included in the treatment regimen. Usual dose is 50 mcg/m2subcutaneously 3 times a week. Granulocyte transfusions can be lifesaving when infections are severe. When preceded by pretransplantation chemotherapy, Hematopoietic Stem Cell Transplantation from an HLA (human leukocyte antigen)-identical sibling is usually successful. Gene therapy is under study. 