Polymorphonuclear leukocytes (PMNs) from children with chronic granulomatous disease of childhood have been shown to readily phagocytize staphylococci and certain gram-negative bacteria, but they demonstrate an impaired intracellular bactericidal capacity for these organisms. The in vitro phagocytic and bactericidal capacities of PMNs from three patients with this disease for four species of streptococci (Streptococcus faecalis, Streptococcus viridans, microaerophlic streptococci, and Streptococcus pyogenes) were tested by the modified method of Maaloe.
The PMNs obtained from the patients phagocytized and killed the four species of streptococci in a normal manner while still showing the defect for Staph. aureus and S. marcescens. Morphologic examination of coverslip preparations of PMNs revealed minimal post-phagocytic degranulation and vacuole formation when either staphylococci and serratia or the streptococcal species were tested. This suggests that different intracellular mechanisms may be responsible for the streptococcal killing.
These observations are in accord with the clinical courses of these patients, who rarely have serious streptococcal infections in contrast to the frequent and life-threatening infections caused by staphylococci and some gram-negative bacteria.
332. Chronic Granulomatous Disease Gene Therapy Functionally Corrects the Phenotype of Polymorphonuclear Leukocytes (PMN)
