Prophylactic therapies for CGD
| Category . | Medication . | Suggested dosing . | Potential adverse events . | Notes . |
|---|---|---|---|---|
| Antibiotics | Trimethoprim/sulfamethoxazole | 5 mg/kg/day TMP, Max 160 mg TMP daily | Hematologic: agranulocytosis, hemolysis, and thrombocytopenia Renal: interstitial nephritis and hyperkalemia GI: abdominal pain, diarrhea, and pancreatitis Dermatologic: photosensitivity and Stevens-Johnson syndrome | Monitor CBC, Cr, and K at baseline and 1 mo Assess G6PD status given potential risk |
| Second line: | | | | |
| Levofloxacin | 10 mg/kg/dose BID, max 750 mg/dose | MSK: Risk of tendinopathy CV: QTc prolongation Neuro: Peripheral neuropathy and lowering of seizure threshold | Monitor CBC, hepatic panel, and Cr at baseline and 1 mo | |
| Antifungals | Itraconazole | 5 mg/kg/day | GI: transaminitis; abdominal pain and diarrhea (in oral suspension) CV: hepatotoxicity (black box warning for ventricular dysfunction) Neuro: peripheral neuropathy Drug–drug interactions due to CYP3A4 inhibitors | Goal drug trough level: ≥1 mcg/ml (incl. metabolites); monitor trough after 7–14 days (if no loading dose given) of dose changes or initiation of any interacting medications, and at 1–3 mo intervals Monitor hepatic panel at baseline and q6 mo Oral formulation and pills are not interchangeable |
| Posaconazole | DR tablet: | GI: Transaminitis, potent CYP3A4 inhibitor CV: QTc prolongation | Tablets and suspension formulations are not interchangeable (see Data S1). Goal drug trough level: ≥0.7 mcg/ml | |
| | 10 to <20 kg: 100 mg BID × 2 doses, then 100 mg daily | |||
| | 20 to <40 kg: 200 mg BID × 2 doses, then 200 mg daily | |||
| | ≥40 kg: 300 mg BID × 2 doses, then 300 mg daily | |||
| Voriconazole | 9 mg/kg BID, max 200 mg/dose | GI: transaminitis Neuro: visual disturbance, optic neuritis, vision color changes, hallucination, and peripheral neuropathy CV: QTc prolongation Derm: photosensitivity/dermatologic cancer | Risk of photosensitivity and skin cancers Goal drug trough level: 0.5–5 mcg/ml | |
| Immunomodulators | IFN-γ-1b | 50 mcg/m2 BSA, or 1.5 mcg/kg/dose (<0.5 m2) three times weekly | Fevers, cytopenias, and transaminitis | May induce low-grade fevers with initial medication start, which may be treated or prevented with NSAIDs |
| Category . | Medication . | Suggested dosing . | Potential adverse events . | Notes . |
|---|---|---|---|---|
| Antibiotics | Trimethoprim/sulfamethoxazole | 5 mg/kg/day TMP, Max 160 mg TMP daily | Hematologic: agranulocytosis, hemolysis, and thrombocytopenia Renal: interstitial nephritis and hyperkalemia GI: abdominal pain, diarrhea, and pancreatitis Dermatologic: photosensitivity and Stevens-Johnson syndrome | Monitor CBC, Cr, and K at baseline and 1 mo Assess G6PD status given potential risk |
| Second line: | | | | |
| Levofloxacin | 10 mg/kg/dose BID, max 750 mg/dose | MSK: Risk of tendinopathy CV: QTc prolongation Neuro: Peripheral neuropathy and lowering of seizure threshold | Monitor CBC, hepatic panel, and Cr at baseline and 1 mo | |
| Antifungals | Itraconazole | 5 mg/kg/day | GI: transaminitis; abdominal pain and diarrhea (in oral suspension) CV: hepatotoxicity (black box warning for ventricular dysfunction) Neuro: peripheral neuropathy Drug–drug interactions due to CYP3A4 inhibitors | Goal drug trough level: ≥1 mcg/ml (incl. metabolites); monitor trough after 7–14 days (if no loading dose given) of dose changes or initiation of any interacting medications, and at 1–3 mo intervals Monitor hepatic panel at baseline and q6 mo Oral formulation and pills are not interchangeable |
| Posaconazole | DR tablet: | GI: Transaminitis, potent CYP3A4 inhibitor CV: QTc prolongation | Tablets and suspension formulations are not interchangeable (see Data S1). Goal drug trough level: ≥0.7 mcg/ml | |
| | 10 to <20 kg: 100 mg BID × 2 doses, then 100 mg daily | |||
| | 20 to <40 kg: 200 mg BID × 2 doses, then 200 mg daily | |||
| | ≥40 kg: 300 mg BID × 2 doses, then 300 mg daily | |||
| Voriconazole | 9 mg/kg BID, max 200 mg/dose | GI: transaminitis Neuro: visual disturbance, optic neuritis, vision color changes, hallucination, and peripheral neuropathy CV: QTc prolongation Derm: photosensitivity/dermatologic cancer | Risk of photosensitivity and skin cancers Goal drug trough level: 0.5–5 mcg/ml | |
| Immunomodulators | IFN-γ-1b | 50 mcg/m2 BSA, or 1.5 mcg/kg/dose (<0.5 m2) three times weekly | Fevers, cytopenias, and transaminitis | May induce low-grade fevers with initial medication start, which may be treated or prevented with NSAIDs |
G6PD, glucose-6-phosphate dehydrogenase. Cr: creatinine, K: potassium; Gl: glucose; BID: twice daily; q6: every 6 hours; CV: cardiovascular; QTc: corrected QT interval; DR: delayed release