A young woman with a severe life-threatening staphylococcal infection was studied to determine if abnormalities of host defense were present. Immunoglobulin levels, serum complement, and numbers of polymorphonuclear neutrophils (PMN) were normal. Assays of PMN function by a sensitive technique demonstrated a consistent impairment of bactericidal activity.
The association of enhanced susceptibility to infection and defective bactericidal activity of phagocytic cells has been recognized in several clinical syndromes. The best studied of these is chronic granulomatous disease of childhood, a disease of young males who suffer from severe staphylococcal and gram-negative infections.1,2 The PMN from these children are probably deficient in an oxidase of nicotinamide adenine nucleotide dehydrogenase (NADH).3 A clinically similar syndrome has been described in females, but their leukocytes appear to be deficient in glutathione peroxidase.4 A patient with disseminated candidiasis was found to have absent neutrophil myeloperoxidase activity,5 and a patient with a