JOURNAL OF CLINICAL AND ANALYTICAL MEDICINE, cilt.6, ss.695-697, 2015 (ESCI İndekslerine Giren Dergi)
Herein we report a surprising diagnosis of a patient admitting with prolonged high fever and septic shock to our pediatric intensive care unit. She was looked for immunodeficiency because of history of recurrent infections from neonatal period and Salmonella enteritis bacteremia. She was investigated at a local hospital for fever lasting for 45 days, leucocytosis, blood and urine cultures, viral serology, autoimmune markers, bone marrow analysis were all normal. Nitroblue tetrazolium test demonstrated the diagnosis of autosomal recessive chronic granulomatous disease with mutation at p22phox. Chronic granulomatous disease is an immuno deficiency caused by defect in nicotinamid adenine dinucleotide phosphate oxidase enzyme primarily affecting the phagocytes. Salmonella infections are generally mild in children presenting as acute gastroenteritis but can cause bacteremia in patients with altered host defences especially neutrophil dysfunction.