What is included in the management 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. Moreover, interferon-gamma is now recommended as life-long therapy for infection prophylaxis in CGD.

Patients with superficial or deep infections (vs those with obstructing granulomas) should receive aggressive antibiotics; the initial route is parenteral.

Hematopoietic stem cell transplantation (HSCT) is the only curative therapeutic modality currently available for CGD.

Despite the increased risk of wound healing associated with surgical intervention, surgery is still an important tool for patients with this disease. Operative treatment may be required to relieve obstruction of ureters from large granulomas, drainage of abscesses, and aggressive removal of established infection, especially in the lung and liver.


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