Pediatric Chronic Granulomatous Disease Medication

Updated: Aug 07, 2019
  • Author: Lawrence C Wolfe, MD; Chief Editor: Cameron K Tebbi, MD  more...
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Medication

Antimicrobials

Class Summary

These agents are used prophylactically to prevent infections in susceptible individuals or to treat active infections.

Trimethoprim-sulfamethoxazole (TMP-SMZ, Cotrimoxazole, Septra, Bactrim)

Antimicrobial drug of choice administered prophylactically to prevent infections in patients with CGD.

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Biologic response modifiers

Class Summary

These agents regulate the immune system by various mechanisms, including enhancing activity of macrophages and cytotoxic actions of T lymphocytes.

Interferon gamma 1b (Actimmune)

Indicated in CGD to reduce frequency and severity of bacterial infections (50 mcg = 1 million IU).

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Antifungal agents

Class Summary

The mechanism of action may involve increasing the permeability of the cell membrane, which, in turn, causes intracellular components to leak.

Itraconazole (Sporanox)

Continuous antifungal therapy effective in preventing infection due to Aspergillus species. Synthetic triazole antifungal agent that slows fungal cell growth by inhibiting CYP–dependent synthesis of ergosterol, vital component of fungal cell membranes.

Voriconazole (VFEND)

Used for primary treatment of invasive aspergillosis and salvage treatment of Fusarium species or Scedosporium apiospermum infections. A triazole antifungal agent that inhibits fungal CYP450-mediated 14 alpha-lanosterol demethylation, which is essential in fungal ergosterol biosynthesis.

Posaconazole (Noxafil)

Triazole antifungal agent. Blocks ergosterol synthesis by inhibiting the enzyme lanosterol 14-alpha-demethylase and sterol precursor accumulation. This action results in cell membrane disruption. Available as oral susp (200 mg/5 mL). Indicated for prophylaxis of invasive Aspergillus and Candida infections in patients at high risk because of severe immunosuppression.

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Immunomodulators

Class Summary

These agents suppress an overactive immune system that leads to formation of granulomas.

Prednisone (Deltasone, Orasone)

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear (PMN) activity.

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