ABSTRACT
Chronic granulomatous disease [CGD] is an uncommon primary immuno deficiency, resulting in bacterial infections produced by catalase- positive microorganism and fungal diseases. The present study describes the clinical characteristics, the infections detected in a group of pediatric patients diagnosed with CGD. A review is made of the clinical records of 20 pediatric patients diagnosed with CGD between first January 1999 and 31th December 2008. The mean age at diagnosis was 10.6 years. The diagnosis was confirmed using the nitroblue tetrazolium test [NBT]. All patients received antibiotic prophylaxis with cotrimoxazole and itraconzole. The median age at the time of diagnosis was 23.8 months. There were 14 boys and 6 girls. In the course of these disease processes there were 97 infections: abscesses [n = 32], lymphadenitis [n=9], pneumoniae [n=8], invasive aspergillus pneumonitis [n=4], osteomyelitis [n=5], gastroenteritis [n=6], sepsis [n=2] and others [n=31].The principal microorganisms isolated were: Staphylococcus sp. [n=32], Aspergillus sp. [n=5]. Two patients had died due to Salmonella infections. CGD isn't rare in Tunisia. The most common clinical manifestations were abscesses and pneumonia