Infectious Disease Compendium

Chronic Granulomatous Disease

Diagnosis

X-Linked defect in nicotinamide adenine dinucleotide phosphate oxidase activity.  Diagnose with the nitroblue-tetrazolium (NBT) test.

Syndromes

Lung infection occurrs in 87% of patients, and liver abscess in 32% (PubMed).

Microbiology

Aspergillus incidence is 2.6 cases per 100 patient-years; Burkholderia, 1.06 per 100 patient-years; Nocardia, 0.81 per 100 patient-years; Serratia, 0.98 per 100 patient-years, and severe Staphylococcus infection, 1.44 per 100 patient-years.

They aslo get infections with Methylotrophs:  Granulibacter bethesdensis (9 cases), Acidomonas methanolica (2 cases), and Methylobacterium lusitanum (1 case) (PubMed).  To quote the EID article "Methylotroph infections in CGD patients typically result in elevated inflammatory markers and lymphadenopathy, which may progress to necrotizing lymphadenitis with or without abscess formation. The clinical course may be protracted because of infection persistence, antimicrobial drug resistance, and relapse. Culturing these bacteria is difficult, requiring atypical media and prolonged incubation."

Treatment

Interferon gamma 1b decreases infections.