Author/Authors :
Farhondi, A Department of Pediatric infectious disease - Children Medical Centre - Tehran University of Medical Sciences, Tehran , Siadati, A Department of Pediatric infectious disease - Children Medical Centre - Tehran University of Medical Sciences, Tehran , Atarod, Lida Department of Pediatric infectious disease - Children Medical Centre - Tehran University of Medical Sciences, Tehran , Tabatabae, P Department of Pediatric infectious disease - Children Medical Centre - Tehran University of Medical Sciences, Tehran , Mamishi, Setareh Department of Pediatric infectious disease - Children Medical Centre - Tehran University of Medical Sciences, Tehran , Khotaii, Gh Department of Pediatric infectious disease - Children Medical Centre - Tehran University of Medical Sciences, Tehran , Armin, Sh Department of Pediatric infectious disease - Children Medical Centre - Tehran University of Medical Sciences, Tehran , Shirvani, F Department of Pediatric infectious disease - Children Medical Centre - Tehran University of Medical Sciences, Tehran
Abstract :
Chronic granulomatous disease is an infrequent primary immunodeficiency characterized by defective intracellular killing of ingested microorganisms thereby making patients highly susceptible to recurrent life threatening bacterial and fungal infections. In this study, we review the medical course of an 8 yr old girl with AR-CGD. She suffered from recurrent dermal and deep abscesses, retractable salmonellosis, disseminated BCGosis, recurrent aspergillus infection presenting as mandibular osteomyelitis and pulmonary involvement with invasion to rib and vertebral bodies. Despite of longterm IV amphotricin B, itraconazole and IFN-y administration, and surgical interventions (drainage and resection), she died in spite of long term antibiotic anti fungal prophylaxis and interferon-gamma administrations, invasive aspergillosis resistant to current conventional therapies is the cause of 1/2 to 1/3 of CGD deaths.
