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Chronic Granulomatous Disease, Pediatric

Benjamin T. Prince, MD, MSci and Ramsay L. Fuleihan, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

Chronic granulomatous disease (CGD) is a rare, primary immunodeficiency caused by a genetic defect that results in an inability of phagocytes to generate superoxide, which is importa...

DIAGNOSIS

The diagnosis of CGD should be considered in patients with 
  • Recurrent lymphadenitis

  • Staphylococcal hepatic abscess

  • Infections with Burkholderia cepacia, Serratia marcescens, Nocardia, or Asperg...

TREATMENT

MEDICATION

  • Prophylaxis

    • Trimethoprim-sulfamethoxazole (TMP-SMX)

      • First line for antibacterial prophylaxis; also can be used for the treatment of acute bacterial infections

      • Prophylaxis dosing: 5 mg...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Regular screening blood tests

    • CBC with differential and LFTs should be performed every 6 months to monitor for any adverse effects of prophylacti...

ADDITIONAL READING

  • Cole T, Pearce MS, Cant AJ, et al. Clinical outcome in children with chronic granulomatous disease managed conservatively or with hematopoietic stem cell transplantation. J Allergy ...

CODES

ICD9

288.1 Functional disorders of polymorphonuclear neutrophils 

ICD10

D71 Functional disorders of polymorphonuclear neutrophils 

SNOMED

387759001 Chronic granulomatous disease (disorder) 

FAQ

  • Q: Should patients with CGD receive dental prophylaxis?

  • A: Yes. Antibiotic prophylaxis should be started before and continued for 24 hours after any dental treatment likely to cause bleeding.

  • Q: Can...

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