Chronic Granulomatous Disease, Case Report and Review of Literature

Chronic granulomatous disease (CGD) is a rare inherited disorder characterized by inability of phagocytes to generate oxygen radicals needed for intracellular killing of phagocytic microorganisms. We report a 2.5-year-old Iranian female with multiple liver abscesses. She was admitted in surgical ward because of abdominal pain and fever for one month duration that had no response to conventional antibiotic treatment. The results of laboratory tests were:WBC=17000, Neutrophils=82%, lymph=17%, ESR=100. Other tests except nitroblue tetrazolium (NBT) and dihydrorhodamine (DHR) tests were negative and according to these two tests the final diagnosis was CGD. Supportive care along with recombinant interferon γ and oral trimethoprimsulfamethoxazolefor prophylaxis of infections started. Now after 18 months she is alive and healthy.Any patient with recurrent or unusual lymphadenitis, hepatic abscesses, osteomyelitis at multiple sites, a family history of recurrent infections, or unusual infections with catalase positive organisms, like S. aureus, requires evaluation for CGD

Keywords:

Chronic Granulomatous Disease , Hepatic Abscess , Nitrotetrazolium Blue , Iran