How are active infections treated in 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

Patients with superficial or deep infections (vs those with obstructing granulomas) should receive aggressive antibiotics; the initial route is parenteral. Treatment usually requires antibiotic coverage for several weeks and should be associated with clear physical signs of resolution and systemic improvement (eg, decreased WBC count and decreased erythrocyte sedimentation rate if elevated at presentation).

Ciprofloxacin with or without additional drug for staphylococcal infection is a common first choice in patients with chronic granulomatous disease. If no response is noted within the first 24-48 hours, coverage should be broadened to include additional antistaphylococcal agents (including coverage for methicillin-resistant Staphylococcus aureus), gram-negative possibilities, and Nocardia species.

The newer antifungals (eg, voriconazole, posaconazole) should be considered for expected fungal infection instead of amphotericin because of decreased toxicity (although they require special consideration with renal dysfunction) and proven efficacy. In established fungal infection, treatment doses of antifungal agents should continue for as long as 6 months.

For poorly responsive infections leading to prolonged consolidation in the lung or large abscesses in the liver, surgical debulking or drainage should be considered. This is especially true of suspected fungal infections and even more so if the chest wall or vertebrae are affected. Prolonged postoperative antibiotics are necessary to deal with slow wound healing and the propensity for wound infection that follows major surgery in these patients.

When an infection breaks through prophylaxis and when it is life-threatening or poorly responsive to antibiotics, growth factor or dexamethasone-induced granulocyte transfusions from healthy donors may improve the outcome.

High-dose interferon-gamma during severe infectious episodes has been advocated.

Patients who present with granulomatous manifestations may have some response to intravenous antimicrobial therapy. However, low-dose corticosteroids are the treatment of choice and are cautiously used in patients who do not appear to have obvious infection, even in the absence of biopsy, especially in patients with GI or genitourinary (GU) obstruction to decrease the time of obstruction without increasing the risk of infection. Prednisone (1 mg/kg) is administered and continues for at least a week until symptoms begin to resolve. A slow taper over 4-6 weeks should be used to avoid recurrence of obstructive symptoms. Chronic treatment with low-dose prednisone or every-other-day treatment may be necessary for resistant obstructive symptoms. Treatment with corticosteroids always increases the risk of infection; thus, increased vigilance in patients throughout steroid treatment is required.


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