What is the role of infection prophylaxis in the treatment of pediatric chronic granulomatous disease (CGD)?

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

Daily prophylaxis of bacterial infections with trimethoprim-sulfamethoxazole (TMP-SMZ; Bactrim) is indicated in chronic granulomatous disease.

TMP-SMZ prophylaxis reduces the incidence of bacterial infections in chronic granulomatous disease without increasing the incidence of fungal infections.

Although numerous other antibiotics have been used, the selective concentration of TMP-SMZ in phagocytes, its broad spectrum of microbicidal activity, and its lack of activity against anaerobic GI flora make this the antimicrobial of choice for prophylaxis in chronic granulomatous disease.

In patients with sulfa allergies, TMP alone or a cephalosporin can be used as daily prophylaxis; however, the effectiveness of this treatment has not been proven.

Ketoconazole is ineffective in reducing fungal infections in patients with chronic granulomatous disease.

Itraconazole prophylaxis against fungal infections is somewhat problematic. A prospective open-label study of long-term itraconazole prophylaxis demonstrated excellent tolerance and a significantly lowered rate of Aspergillus infections versus historical controls. [14] If Aspergillus infection occurs, consult the treatment guidelines from the Infectious Diseases Society of America. [15, 16] A randomized double-blind placebo-controlled study showed that itraconazole prophylaxis in chronic granulomatous disease prevented serious and superficial fungal infections. [17] Adverse effects included rash, increased liver-function values, and headache; these resolved after itraconazole was discontinued. Newer formulations of itraconazole may allow more reliable blood levels and more consistent prophylaxis.


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